tag:blogger.com,1999:blog-377894752024-03-12T21:07:57.525-05:00CREUTZFELDT JAKOB DISEASESEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comBlogger449125tag:blogger.com,1999:blog-37789475.post-48479601821438965882024-02-08T16:55:00.001-06:002024-02-08T16:55:07.614-06:00A systemic analysis of Creutzfeldt Jakob disease cases in Asia<p><span style="background-color: white; font-family: arial; font-size: 16px;">A systemic analysis of Creutzfeldt Jakob disease cases in Asia</span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Urwah Rasheed, Sana Khan,Minahil Khalid, Aneeqa Noor ORCID Icon & Saima Zafar</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Pages 11-27 | Received 20 Oct 2023, Accepted 25 Jan 2024, Published online: 07 Feb 2024</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Creutzfeldt Jakob Disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder, also known as a subacute spongiform encephalopathy. There are three major subtypes of CJD i.e. Sporadic CJD, which occurs for reasons unbeknown to science (85% of known cases), Genetic or Familial CJD which is characterized by the presence of mutations in the human prion protein (PRNP) gene (10–15% cases) and Iatrogenic CJD that occurs via accidental transmission through medical and surgical procedures (1–2% cases). CJD cases occur globally with 1 case per one million population/year. Considerable data is available related to the incidence and prevalence of CJD in Europe and America. However, the global surveillance database is yet to include Asia even though several Asian countries have their own CJD monitoring units. sCJD is the highest among all CJD cases in Asia. China (1957) and Japan (1705) have reported more cases of sCJD than any Asian country and Hong Kong (1) has reported the least. On the other hand, gCJD is highest in Japan (370) and least in India (2). Our analysis establishes the presence of all variants of CJD across Asia. However, in most Asian countries in general and Southeast Asian countries in particular, CJD cases are misdiagnosed and often underreported. Since Asia is the most populated continent in the world, the actual global prevalence of CJD cannot be estimated until and unless these countries are accounted for. Concrete and reliable surveillance networks are needed across Asia to evaluate the prevalence and incidence of CJD in the region.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4. Incidence of sporadic CJD in Asia</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.1. China</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">China recorded the highest number of sCJD cases since the early cases emerged in 1982. Guo et al., published two case reports, out of which one was sporadic, whereas the other was genetic. While the source of infection for the sporadic case could not be identified, the EEG showed progressing changes aiding in the clinical diagnosis [Citation24]. Following the initial reports, the Department of Neurology, General Hospital of the People’s Liberation Army diagnosed 57 cases of sCJD, between the years 1992 and 2011. Out of these 57, 11 patients were diagnosed with sCJD, 39 were probable and 7 were possible diagnoses. Of these cases, 24 were females and 33 were males, with a gender ratio of 0.727:1. The onset age range is 36 to 75 years with 8.8% of the patients in the age group 30 to 39, 14% of the patients were in 40–49 years, 35.1% were in 50–59, 36.8% were in 60–69 and 5.3% were in above 70 age group. A follow-up study was conducted, but most patients were deceased. The time between the onset of disease and death ranged from 5 to 22 months. Among the deceased, 28 died 7 to 12 months following the onset of the disease, 17 died after a year of onset, and only 9 lived up to 6 months of onset [Citation25]. In another report published by Shi et al., 192 suspected cases of CJD were identified between the years 2006 and 2007 through surveillance systems. Fifty-one patients out of these were diagnosed as probable sCJD cases, while 30 of them were considered as possible sCJD cases, on the basis of diagnosis criteria [Citation26].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">With an increase in the elderly population, more cases have emerged recently. In 2016, Shi et al., reported the surveillance data were retrieved from 15 hospitals and 12 provincial CDCs which covered Shanghai, Beijing, Chongqing, Tianjin, Hubei, Jilin, Shaanxi, Guizhou, Guangdong, Henan, Anhui, and Xinjiang. A total of 1585 cases were reported, out of which 700 were categorized as sCJD. Two out of these were definite, 560 were probable and 138 were possible [Citation27]. Overall, 5078 CJD cases have been reported between the years 2006 to 2021, out of which 1900 cases were attributed to sCJD cases. All PLADs located on the Chinese mainland experienced cases of sCJD, with the sole exception being the Xizang Autonomous Region (Tibet). It should be noted that there was no discernible correlation between the occurrence of sCJD and factors such as geographical location, season, or occupation.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The initial indications and manifestations of sCJD cases exhibited a wide range of diversity. The prevailing symptom that was frequently cited was the gradual onset of dementia, accounting for approximately 41% of the reported cases. This was followed by impairments in cerebellar and visual functions, which constituted 18% of the cases, mental health issues at 13%, and pyramidal and extrapyramidal symptoms at 10%. As the condition progressed, a greater number of neurological abnormalities became evident. It is worth noting that all sCJD cases were accompanied by dementia, as indicated by the reports. Furthermore, the other four symptoms associated with sCJD, namely visual or cerebellar dysfunction (67%), myoclonus (76%), pyramidal or extrapyramidal symptoms (80%), and mutism (39%), were also frequently observed. A total of 19%, 40%, and 41% of the patients displayed dementia along with 4, 3, and 2 additional neurological symptoms, respectively. Small granules and the type-1 PrPSc molecule exhibited extensive dispersion throughout the cerebral tissues of a limited cohort of individuals affected by sCJD subsequent to neuropathological and molecular evaluation, whether conducted posthumously or through cerebral biopsy [Citation28].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.2. Hong Kong</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">A case report of a 67-year-old was published by Chan et al., in 1987. The patient was suffering from the clinical onset of a disease for the past 6 months and was admitted to the hospital with a worsening mental state. Apart from the physical symptoms, EEG depicted irregular activities in all areas of the brain. Although the patient was reactive to painful stimuli, slight droopiness in the left central face was observed, with myoclonic jerks occurring. He was then diagnosed with CJD. The patient then slipped into coma for the next 2 months with myoclonic jerks slowly disappearing. The patient was declared deceased 4 months following his admission. His survival period from onset to death was 10 months [Citation29].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.3. India</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">India is a developing country, and not many cases were reported. One of the reasons could be the lack of applicable diagnostic techniques. In the period between 1971 and 1990, 30 patients were documented which included 20 confirmed and 10 probable cases of CJD. Among them, 18 were men and 12 were women. The age of onset ranged between 34 and 76 years. The survival time ranged from 1 month to 36 months. The patients had clinical onset with psychiatric symptoms such as visual complaints, and confusion state before they were diagnosed with CJD [Citation30,Citation31].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In another case reported by Biswas et al., 10 people were categorized as probable sCJD in the year range 2011–2013 with a gender ratio of M: F = 4:6. Their ages ranged from 39 to 70 years and averaged 56.1 years. Six of them presented clinical characteristics such as behavioural abnormalities, four of them had ataxia, five presented extrapyramidal features, four complained of visual hallucination and one had cortical blindness. Within 4 months, all of them were rendered bedridden and eight died within a mean duration of 4.56 months following onset. The remaining two patients were not available for follow-up [Citation32].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.4. Japan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">After China, Japan recorded the highest number of CJD cases. From 1999 to 2009, the CJD Surveillance Committee of Japan registered 1,241 cases of prion diseases. Among them, 76.8% were sCJD cases, found mainly of MM2 genotype in thalamic and cortical forms [Citation33]. Furthermore, Iwasaki et al., reported 29 autopsied sCJD patients recorded by the Institute for Medical Science of Aging, Aichi Medical University from the year 1997 to 2009. The patients were found to be carrying the MM1 genotype of PRNP codon 129. The mean age at disease onset was 67.1 years and the mean survival time was 11.9 months. In order to diagnose, EEG was done after the onset of symptoms. Once the akinetic mutism state was reached, the period of progression to death was 9 months [Citation34].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">A 2022 study by Kosami et al., reported 2440 CJD cases with sCJD attributed for 1676 (69%) cases. Patients with homozygosity for methionine may potentially face an elevated susceptibility to the development of prion disorders. The odds ratio (95% confidence interval) adjusted for sCJD was calculated to be 2.21 (1.46 to 3.34) [Citation35]. In 2023, a 72-year-old female patient arrived at the medical facility displaying visual impairment in both eyes and a duration of light sensitivity spanning two to 3 months. Her visual acuity in both eyes was recorded as 20/2000 seven days prior. Upon examination, her pupillary light reflex remained intact and fundoscopy revealed no abnormalities. However, an observation of left homonymous hemianopia and limited downward movement of the left eye was possible. Upon admission, her visual acuity was reduced to light perception. Both electroencephalography and cranial magnetic resonance imaging results exhibited no irregularities or periodic synchronous discharges. On the sixth day of hospitalization, an examination of the patient’s cerebral spinal fluid exposed the presence of tau and 14-3-3 protein, with a successful real-time quaking-induced conversion outcome. Subsequently, the patient experienced myoclonus, became mute, and eventually succumbed. Postmortem analysis revealed spongiform alteration and thinning of the right occipital lobe cerebral cortex. Deposits of irregular PrP and enlarged astrocytes were observed with immunostaining. Through the examination of brain tissue via western blot and the analysis of the PrP gene’s codon 129 polymorphism, it was subsequently established that she possessed the Heidenhain variant of sCJD characterized by the presence of both methionine/methionine type 1 and type 2 cortical form [Citation36].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.5. Oman</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Two sporadic cases were reported during the period between 1991 and 1995, in Oman [Citation37]. Aged 50 and 75, both presented clinical characteristics such as rapidly progressive dementia and myoclonic jerks. The patients were diagnosed based on the symptoms and EEG results depicting abnormalities.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.6. Pakistan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Pakistan reported 2 probable sporadic cases in the year 2012 and 2013. The 62- and 72-years old females were diagnosed with sCJD after both presented psychiatric and behavioural symptoms along with positive EEG and MRI results. Both patients had no history of hormone therapy or grafting [Citation38]. Furthermore, one case of CJD was confirmed through biopsy [Citation39]. This 69-year-old female patient was brought into Agha Khan University Hospital, Karachi, Pakistan, with behavioural symptoms which began 5 months earlier. She was also suffering from falls and gait unsteadiness for 2 months before admission. Weeks following admission, the patient started facing myoclonus jerks, eventually leading to unresponsiveness, and was rendered bed bound. To confirm a diagnosis, multiple tests were performed. A biopsy of the frontal lobe of the brain was done which depicted spongiform changes. The biopsy sample was immune stained with 3F4 monoclonal antibody, revealing granular deposits that are specifically found in prion diseases [Citation40].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.7. Singapore</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Two sCJD cases, a 50-year-old male, and a 61-year-old female, have been reported in the years 1993 and 2002, respectively. The former was admitted to the hospital due to a deteriorating mental state. He presented behavioural changes, poor coordination, and jerky movements. An EEG was performed after several other tests, which demonstrated superimposed periodic high-voltage, sharp, and slow wave complexes in both hemispheres. A biopsy was also done from the frontal right lobe which showed degeneration of the grey matter in the cortical area, along with astrocytosis. The patient died a year later. The second patient’s EEG showed periodic lateralized epileptiform discharges (PLEDs) in the brain’s left frontal and central regions. After the confirmed diagnosis, the patient was bedridden for 10 months after onset [Citation41].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.8. Taiwan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Taiwan CJD Surveillance Unit (CJDSU) was established in 1997 and registered CJD cases dating back to 1975. Prior to 1997, 74 cases of sCJD were reported, with 3 biopsy-proved cases only. A total of 809 cases were reported to the CJD surveillance unit from 1996 to 2020 for the purpose of confirmation. Among these cases, 441 (with 230 women) were determined to be sporadic CJD. The average age of onset was 67 years with a standard deviation of 9.9 years. The median survival period was found to be 13.3 to 14.2 months on average. The two primary clinical signs observed were myoclonus (73% of cases) and akinetic mutism (54% of cases). In terms of PRNP polymorphism, 99% of patients (195 out of 197) exhibited the methionine homozygous genotype at codon 129 (M129M). EEG demonstrated sensitivity to periodic sharp wave complexes (PSWCs) in 59.7% of cases. The sensitivity of total tau protein (>1200 pg/mL) and 14-3-3 protein (>1200 pg/mL) in cerebral fluid was determined to be 75.6% and 69.7%, respectively. It was observed that patients who were younger tended to have longer survival compared to those who were over 65 years old (hazard ratio of 0.466, P .001). In the initial triennial period, the likelihood of survival for women surpassed that of men (hazard ratio [HR] 0.712, p = .005). The presence of periodic sharp wave complexes (PSWCs) continued to exert a deleterious influence on survival (HR 0.788, p < .05). Among the various factors, only epileptic seizures emerged as a clinically significant predictor for survival duration, despite their infrequency (HR 0.768, p < .05). PSWCs represent a prognostic EEG biomarker that can be effectively capitalized upon. Although rare, epileptic seizures serve as the sole clinical prognostic marker for a brief period of survival [Citation42].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">4.9. Thailand</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">While there are no official surveillance or statistics on CJd in Thailand, 2 probable and 1 possible case between the year 2012 and 2014 were reported from Thammasat University Hospital. A 42-year-old Thai woman exhibited an irregular walking pattern and subacute dizziness. After 2 weeks, she began experiencing cognitive decline, including memory loss and difficulty concentrating. Her level of enjoyment and interest in activities significantly diminished. Eventually, she became uncommunicative and developed rigid muscles as her overall health worsened. Subsequently, her limbs exhibited spontaneous myoclonus movements. T2-weighted and diffusion-weighted imaging revealed heightened signal intensity in the bilateral caudate nuclei, putamen, and left frontotemporal cortex. Electroencephalography detected generalized and synchronous periodic sharp wave complexes occurring at intervals of 0.6–0.8 seconds. Further analysis with back-averaging EEG confirmed multifocal myoclonus originating from the brain. Unfortunately, 32 weeks after the initial onset of symptoms, she passed away. The patient’s clinical history, physical manifestations, EEG findings, and brain MRI results all align with a probable diagnosis of sCJD.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In another case, a 76-year-old Thai male patient sought medical attention due to a three-week history of mental confusion, hallucinations, difficulties in speech articulation, and slight weakness on his left side. He denied ever experiencing a headache or a fever. The analysis of his CSF and the general blood chemistry were both consistent with acceptable values. On fluid-attenuated inversion recovery (FLAIR) MRI sequences, his bilateral caudate nuclei, putamen, and bilateral fronto-temporo-parietal cortices demonstrated symmetrical hypersignal intensity, with restricted diffusion on diffusion-weighted imaging (DWI). His electroencephalogram (EEG) detected periodic generalized sharp wave complexes. As time progressed, his clinical condition deteriorated, and he began to experience involuntary muscular contractions throughout his body. Sadly, he developed aspiration pneumonia, and after a duration of 8 months from the initial onset of symptoms, he tragically passed away. The patient’s condition was identified as being consistent with sCJD, with a high probability.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In the same period, a female Thai teacher, aged 53, who had previously suffered from exhaustion, insomnia, and vertigo, presented with symptoms. These symptoms included memory impairment, reduced motor coordination, and withdrawal from social interactions 1 month later. Upon physical examination, the patient displayed alertness but a significant decline in her ability to produce speech. We observed general stiffness and occasional spontaneous muscle contractions. Following an initial diagnosis of akinetic-rigid syndrome, comprehensive blood tests and cerebrospinal fluid investigations were conducted. A CT scan of the brain revealed no abnormalities. EEG detected intermittent delta slow waves superimposed on a characteristic alpha background. The patient’s condition rapidly deteriorated, resulting in muteness and confinement to bed. Spontaneous muscle contractions in the limbs were observed. Four months after the onset of symptoms, the patient passed away. Based on the clinical history, disease progression, and physical examination findings, a potential diagnosis of sCJD was indicated [Citation43].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5. Incidence of genetic CJD in Asia</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5.1. China</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">CJD was hardly diagnosed in China till the end of the 1980s. According to the data provided by Chinese National Surveillance for CJD, a total of 5,078 cases of CJD were reported of which 243 cases accounted for different types of Genetic Prion Diseases (gPRDs) including gCJD. Of all the prion disease (PrD) cases diagnosed between 2006 and 2021, 11.1% were gPrD cases of which 167 were confirmed as gCJD [Citation44]. Overall, 19 different subtypes of PRNP mutations were identified in Chinese patients [Citation45]. CJD cases were diagnosed according to the criteria provided by the Chinese National Health Commission and World Health Organization. Clinical examinations including MRI, EEG, CSF 14-3-3, CSF tau, and CSF biochemistry analysis was done followed by PRNP PCR and sequencing. The results were clinically correlated by the expert neurologists and epidemiologists.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The most common mutation in China is T188K with 65 cases i.e., 29.8% of all the gPRD cases. T188K cases have been identified in 20 provinces across China, with largest number of cases in Shandong. First case of T188K mutation in China was identified in 2009. Median age at the onset of gCJD with T188K mutation is 61 years and the average survival time of the patient is 4 months. MRI of 78% of the T188K patients showed special abnormalities [Citation46,Citation47]. Only a single instance of the eight CJD T188K cases documented in one study exhibited a positive familial background of the ailment. The affected individuals ranged in age from 39 to 76 years old. The initial symptoms are reportedly inconsistent. The predominant clinical presentation in most patients (63%) was rapidly progressive dementia, followed by walking instability (50%), in that particular sequence. Each individual experienced dementia, as well as pyramidal or extrapyramidal complications, during the later stages of the condition. Moreover, cerebellar dysfunctions and myoclonus were also frequently observed, akinetic mutism was also recorded [Citation47]. Another study that analysed 30 patients with the T188K mutation concluded that progressive dementia observed among these patients was quite prevalent, with a prevalence rate of 66%. Additional early markers included extrapyramidal symptoms, mental dysfunction, and cerebellar problems at a rate of 30%, 33%, and 40%, respectively. Visual abnormalities and akinetic mutism were also observed in the later stages of the illness among the patients. Approximately 27.9% of T188K mutation cases showed periodic sharp wave complexes (PSWC) on EEG [Citation48].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The second most common mutation to have been diagnosed in China is E200K with 41 cases in total and constituting 19.8% of the total gPRD cases [Citation44,Citation49]. Only 6 out of 41 E200K patients showed family history. Increased proportion of CSF tau protein was observed in these patients. The first Chinese case of E200K mutation was diagnosed in 2010 which displayed missense mutation in codon 200 (E200K) and methionine homozygous genotype at codon 129 of PRNP gene [Citation50]. In a study, 30 patients with E200K mutation were analysed, showed that 14-3-3 marker was detected in a majority of the patients. The EEG pattern exhibited an anomalous nature characterized by a decrease in the rate of background activity and the presence of periodicity, accompanied by triphasic sharp waves. In nearly half (48%) of the gCJD cases pertaining to the E200K mutation, the presence of PSWC on EEG recordings were identified [Citation48]E200K cases are more prevalent in Northern China and are identified in 16 provinces. The average age at the onset of E200K mutation gCJD is 57 years and survival time is 6 months [Citation44].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The third most common mutation in gCJD patients is E196A with a total of 16 cases till date and 7.3% of the entire gPRDs [Citation48,Citation51]. None of the patients with E196A reported any family history of CJD. A total of 73% of the patients with E196A mutation displayed special abnormalities on MRI. The average age at the onset of gCJD in patients with E196A mutation is 61 years and the survival time is six and half months [Citation44]. E196A patients show similarities with sCJD phenotypically with slight differences in a few clinical features [Citation52]. In a study, it was reported that an elderly Chinese male, aged 76, who was diagnosed with CJD, exhibited a unique mutation in the PRNP gene. The presence of the 14-3-3 protein was detected in the cerebrospinal fluid, while diffusion-weighted MRI scans revealed the existence of a ribbon-like high signal intensity in both cortices. Furthermore, electroencephalography demonstrated a typical periodic synchronous discharge. The diagnosis of CJD was established based on the manifestation of distinct clinical symptoms. Remarkably, the identification of a point mutation at codon 196 (E196A: GAG→GCG) within the PRNP gene was made [Citation53].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Other mutations with less than 5 cases throughout China are E196K, V2031, R208H, V210I, G114I, R148H, V180I, T183A and E200G with 5, 3, 3, 3, 2, 2, 1, 1, and 1 case identified respectively [Citation54–57].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5.2. India</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Due to the lack of genetic testing facilities, reports regarding gCJD from India are limited [Citation58]. Only two studies identifying genetic or familial CJD have been carried out so far. First familial CJD case with D178N from South-East Asian region was reported in India. A 42-year-old male patient exhibited a history of dementia, behavioural problems, and difficulty in walking. Over the course of 3 months his symptoms exacerbated rapidly, and he developed myoclonus. He also had a family history on his maternal side of the family where his family members developed similar symptoms. Genetic testing revealed D178N mutation in the PRNP gene. CSF 14-3-3 turned out to be negative which is in accordance with the previous studies which discovered that CSF 14-3-3 is negative in patients with D178N mutation. Neuroimaging and clinical evaluation also indicated CJD. All the symptomatic members of his family died within 3 to 15 months of the onset of the disease. Two more family members were also tested positive for D178N mutation and V/V polymorphism was identified at 129th amino acid but they were asymptomatic [Citation59].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In another case report in India, a man presented a history of short-term memory loss, behavioural changes, mood swings, deterioration in communication, hand tremor, lost control of urination. Neuropathological analysis showed major neuronal loss along with the shrinkage of cortex. Rare small plaques were seen in PrP immunostaining. Genetic analysis showed D178N mutation in PRNP. Genetic testing on 27 members of his was performed, the results showed that two of his family members were positive for D178N mutation [Citation60].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5.3. Japan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In 1996, three gCJD patients were identified with M232R mutation in Japan. The patients exhibited rapidly progressive dementia, abnormal EEG results and myoclonus. All three patients suffered akinetic mutism within 2 to 6 months of developing the disease and died within 4 to 24 months. Diffused grey matter was seen in immune PrP staining. Loss of neurons could be seen in histopathological analysis. Plaque formation in the brain wasn’t observed. Healthy controls did not have the M232R mutation indicating that the disease in those three patients was indeed caused by M232R [Citation61].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In another report, two brothers were reported to have familial CJD, both patients presented rapidly progressive dementia, abnormal EEG, and myoclonus. One brother was 62 years old and died within 6 months of developing the disease. The second brother was 58-year-old when he developed the disease and died within 13 months. Genetic analysis identified point mutation in PrP gene at codon 200 of one of the first brother. Heterogenous phenotype of CJD200 was identified in Europe and America whereas in Japan the phenotype was homogenous [Citation62]. In 1997, another family was reported to have familial CJD with codon 200 and codon 219 mutation [Citation63]. In 1999, Japan established its national surveillance system to identify prion diseases in humans. This program collected clinical, neurological, and genetic information of patients suffering from CJD from 1999 to 2014. Of 2,394 cases identified, 365 were of gCJD. The overall prevalence was 1.2 cases/million per year. According to the genetic testing, the most prevalent mutation was V180I with a total of 211 cases followed by M232R and E200K with 63 and 61 cases respectively. Currently, there are numerous reports indicating that the V180I mutation in PRNP is responsible for distinct clinical and pathological findings. Due to the rarity of a familial history of the disease among patients with V180I, the question of whether this mutation is the cause of prion disease persists. Conversely, patients with V180I exhibit various specific clinical features that differentiate them from those diagnosed with Scjd or other genetic prion diseases (gPrDs). Patients with V180I can be easily distinguished from those with other forms of dementia due to the presence of specific hyperintensity observed in the cerebral cortex during diffusion-weighted MRI [Citation64] Five cases of D179N were also identified [Citation59]. Five cases of D179N were also identified [Citation65].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5.4. South Korea</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Due to the close proximity of the Republic of Korea and Japan, similar patterns of mutations were identified, however data related to mutations and polymorphisms associated with gCJD is scarce in Korea. A surveillance study was carried out from 2001 to 2019 in Korea, during this period 33 were familial or genetic. The mutations that were prevalent in South Korea include E200K, V1801, M232R, D178N, and V203I [Citation66].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In a study, in addition to MRI and EEG, genetic analysis was carried out which revealed gCJD patients had three mutations at codon D178N, E200K, and M232R. The D178N mutation (with homozygous M/M at codon 129) was initially identified in 2009 in a male patient of age 67 who suffered from atypical CJD, without any familial background. The observed symptoms consisted of gradual disruption of gait and speech difficulties accompanied by extrapyramidal indicators, but not insomnia. Additionally, the patient displayed rigidity and bradykinesia, yet there was an absence of myoclonus, visual impairment, cognitive decline, or pyramidal symptoms. Over the course of a few months, the patient’s condition rapidly deteriorated into akinetic mutism, although the duration of the disease was relatively extended, exceeding 2 years. EEG results were within the normal range, with positive findings for the 14-3-3 CSF marker. MRI scans exhibited elevated signal intensities in both the parietal and occipital gyri [Citation67].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The 58-year-old patient with E200K mutation displayed swift disease progression, impaired gait, cognitive disarray, and myoclonus. Subsequently, dysarthria, diminished gait, decreased attention, and restlessness manifested. During the neuropsychological assessment, the patient exhibited signs of confusion and disorientation. The patient succumbed to the illness 3 months after the initial onset of symptoms. MRI unveiled heightened signal intensity in various regions of the brain, encompassing both the bilateral frontal temporoparietal area and the caudate nucleus. EEG revealed the presence of sharp spikes and slow waves, and CSF tested positive for the 14-3-3 signal [Citation67]. Another case of gCJD with E200k mutation was documented in Korea, the patient, displayed the onset of progressive dysarthria and visual hallucinations at the age of 63. Subsequently, within a brief period, the patient also encountered impairments in gait, myoclonus, and behavioural abnormalities. The DWI examination revealed significant elevation in signal intensity within the basal ganglia and occipitoparietal cortex regions. A patient who was 65-year-old with M232R mutation exhibited rapidly progressive dementia and walking disability, MRI displayed heightened signal intensities in the parieto-occipital cortex and temporal lobes and CSF exhibited positivity for 14-3-3 protein. The patient died within 16 months of developing the disease [Citation67].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In another case, a 75-year-old woman was reported to have fCJD with V180I mutation. This is the first case of a point mutation at codon 180 in South Korea. The EEG examination of the patient revealed the presence of delayed oscillations in the right cerebral hemisphere, whereas the CSF analysis showed a positive outcome for the 14-3-3 protein. In addition, MRI showed elevated intensities of signal in the frontal, parietal, temporal, and occipital regions, bilaterally. The woman did not have any family history of dementia [Citation68]. One study documented the admission of an elderly patient that had V180I mutation with serious neurological symptoms to the hospital. The patient had intense lesions in the thalamus, right frontal cortex, and temporal cortex. Analysis of brain tissue showed changes in the tissue, empty spaces, scarring, and loss of neurons in most parts of the brain [Citation69]. Another study documented the case of five patients with V180I mutation, The onset of the disease occurred between the ages of 72 and 77 in four of the subjects, while one patient experienced the onset at the age of 57. All five patients exhibiting the V180I mutation displayed either positive or slightly positive results for the presence of 14-3-3 protein in their CSF. The symptoms consisted of cognitive impairment, coordination difficulties, mood disorder, or brain dysfunction [Citation70].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">5.5 Taiwan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">According to the surveillance study conducted by CJDSU from 1998 to 2017, eight cases of gCJD were identified in Taiwan. Of the eight cases identified, two cases consisted of E196A-129 M and one case of R148H-129 M mutation [Citation71]. The age range of the onset of disease was 29–67 years and the mean survival time was 2.5 to 5.5 years [Citation72]. Table 1 summarizes the PRNP mutations that are prevalent in Asian countries.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">6. Incidence of iatrogenic CJD in Asia</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">6.1 Japan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Iatrogenic cases increased significantly during the 1990s in Asia, mostly due to the transplantation of dura mater grafts which corresponds to 95% of the cases [Citation73]. The CJD surveillance committee of Japan performed a comparative analysis among Japan and other counties and revealed that the prevalence of iCJD was significantly higher than in other counties, the reason being the frequent use of lyodura in 2012 [Citation74]. Most iCJD cases were those of dura mater graft-associated CJD (dCJD) i.e., 0.05% to 0.02% [Citation67,Citation75]. In Japan, 1,241 patients have been identified as definite and probable cases of CJD from 1975 to 2009 among which 77 cases were iCJD [Citation74]. According to Japan’s monitoring system, 2003 showed a significant decline in iCJD cases but in 2017, one case was recorded which was attributed to the long incubation period of this disease [Citation76,Citation77].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Other than dCJD, iCJD can also be caused due to corneal transplant. CJD cases recorded from 1990 to 2006 also included patients who got corneal transplants 1.5 years earlier. But unfortunately, the origin couldn’t be detected as eye banks denied that any donors were infected with prion disease. No patients of iCJD have been reported in Asia who previously received gonadotrophin and human growth hormone (hGH), and this trend can correlate with the careful preparation of human growth hormone through chromatographic purification in Japan [Citation78]. Similarly, since the 1980s no cases of iCJD have been reported to occur via EEG needles and surgical instruments.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">6.2. Taiwan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">A hospital-based nationwide study performed under the Taiwan CJD surveillance unit from 1996 onwards identified 123 patients of CJD but no patients of iCJD [Citation42]. Epidemiological studies in Taiwan indicated that the annual incidence of CJD is lesser compared to other countries. Specifically, iCJD cases are non-existent in Taiwan despite their full-scale tracking system [Citation71,Citation79].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">6.3. India</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Unfortunately, India, like many other countries have not developed any nationwide surveillance unit, hence we are unable to detect the actual number of CJD in general and specifically iCJD patients. Contrary to these induvial studies have shown that there are probable cases of iatrogenic origin in India. For example, a case study conducted from 1971 to 1990 which depicted 3 out of 20 CJD patients had undergone an invasive medical procedure in past [Citation31].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">7. Comparison to US and Europe</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Different countries in Asia exhibit varying rates of sCJD, with certain regions reporting lower incidences compared to others. Among the Asian nations, Japan stands out with the highest reported rates of sCJD. In comparison to numerous Asian nations, the US displays a relatively lower prevalence of sCJD. Approximately 1 out of every 1 million individuals are annually affected by this condition. Significant regional disparities in sCJD surveillance and reporting can be observed in Asia, potentially leading to underreporting in certain areas. The US employs a more centralized and standardized approach to monitor and report rare diseases like sCJD, resulting in more accurate statistical data. The prevalence rates in Europe exhibit substantial variation, with specific Western European countries documenting elevated frequencies of sCJD compared to other Eastern European countries. sCJD has emerged as the central area of interest for multiple extensively financed research networks and institutions throughout Europe, demonstrating noteworthy advancements in the examination and management of this condition [Citation80].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The mutation pattern observed in Japan differed from that observed in the European countries. Among the mutations associated with CJD, the occurrence of V180I (0.2% in the EuroCJD population) and M232R (not detected in the EuroCJD population) was found to be significantly higher in Japan compared to Europe. Among the mutations associated with gCJD, the V180I mutation was found to occur at a rate of 0.2% in the EuroCJD population, while the M232R mutation was not detected in EuroCJD. Interestingly, these mutations were observed to be significantly more common in Japan compared to Europe. On the other hand, the E200K mutation was found to be present in 17.1% of Japanese CJD cases and 41.2% of European cases. Similarly, the V210I mutation was absent in Japanese patients but had a prevalence of 16.2% in European patients. Additionally, the occurrence of the octapeptide insertion was found to be 1.4% in Japan and 9.9% in Europe, while the D178N mutation with either the 129 M/M or V/V genotype was less frequent in Japan compared to European patients [Citation81].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">T188K has been documented in two legal instances within the European region, specifically in Austria and Germany. Furthermore, there is a lack of available data regarding the presence of T188K within Asian populations, namely Korean or Japanese, with the exception of Chinese individuals [Citation45]. On the other hand, E196K appears to be more prevalent among European patients, as it has solely been observed in a solitary Chinese case [Citation82]. The occurrence of M129V and E219K showed dissimilarities in East Asia and Europe as well. In the overall population of Koreans and Japanese, a majority of individuals possess the MM allele for M129, whereas the MV allele (approximately 6–7%) and the VV allele (less than 1%) may be infrequent. Conversely, in the general populations of Europe, the MV and VV alleles are rather prevalent (35–51% and 8–12% respectively). Heterozygous E219K was observed in around 7–8% of healthy Koreans and Japanese, while it is likely to be exceedingly rare among the general European population [Citation83].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Studies have shown the frequency of individuals acquiring iCJD after Hgh transplant was higher in European countries (especially in France (119 cases) & UK (65 cases) whereas US reported 29 cases and Asian countries (specifically Japan) reported 77 cases. Analysis of patients with Dura graft transmission showed that Japan comprises of two-third of the cases globally with 83 cases of iCJD being reported till 2005s this may be due to long incubation period. On the other hand, a total of 206 cases were observed in Caucasian population due to transplantation of contaminated Dura graft [Citation84]. Asian countries reported four cases of iCJD cases due to neuronal instruments and corneal transplants whereas no cases were seen in Caucasian population corresponding to former route of exposure, but four deaths were recorded among the patients who have previously undergone corneal transplant [Citation85]. Overall, the frequency of iCJD cases was observed greater in Asian population as compared to Caucasian population.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">8. Prevalence of other genetic prion diseases</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Other Genetic Prion diseases include Gerstmann – Sträussler – Scheinker disease (GSS) and Fatal Familial Insomnia (FFI). GSS can initially present as ataxia or Parkinsonism, with dementia appearing later. The duration of the disease varies, with some patients succumbing within a year and others enduring for over 10 years. Amyloid plaques containing Aβ peptide can be found in the brain, especially the cerebellum [Citation86]. The initial signs of FFI typically involve difficulty sleeping and problems with the autonomic nervous system. As the disease progresses, there are additional challenges with movement and cognition. The duration of the illness is often short, with some patients dying within 2 years of onset. FFI can affect various parts of the nervous system, such as the thalamic nerves, leading to their loss or atrophy. Additionally, there may be PrPSc deposition in the midbrain or hypothalamus [Citation87]. Prevalence of GSS and FFI cases identified in China, Japan, Korea and India are discussed in this review article.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">8.1. China</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In a study carried out in 2017, five cases of GSS with the P102L mutation were identified. The patients developed the disease at different ages. All cases began with difficulty in walking and progressive ataxia. Affected family members displayed different symptoms. Cognitive decline was common among the patients, but only two had early symptoms. Two individuals had cognitive dysfunction later. The study suggests that unknown genetic or environmental factors may contribute to the phenotypic diversity [Citation88].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In 2017, another case of GSS was identified. The female patient exhibited unstable gait and dysarthria at 44 years old. The patient displayed dysarthria, nystagmus, wide base, and unsteady gait. Brain MRI showed mild diffuse atrophy. The patient’s spinal cord was damaged by herniation [Citation89]. In a study conducted in 2019, 12 GSS patients were identified. Most of these patients exhibited motor dysfunction as an initial symptom [Citation90]. The occurrence of the P105L mutation in Chinese patients is potentially rare, evidenced by the reporting of only a single instance of GSS mutation [Citation91]. FFI with the D178N mutation is also observed frequently in China, with 39% of the analysed cases exhibiting this mutation [Citation92].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">8.2. Japan</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The P102L mutation is prevalent among Japanese patients diagnosed with GSS. The CJD Surveillance Committee has documented the occurrence of this mutation in 39 patients [Citation93]. Familial GSS is the most commonly observed form of the disease, with the age of onset varying among individuals. Additionally, positive cases of the 14-3-3 protein were detected in patients with the P102L mutation. The primary symptoms observed in these patients included spastic paraparesis, gait disturbances, ataxia, and tremor. It is worth noting that not all patients with the P105L mutation exhibited spastic paraparesis. Finally, a recent case involving the P105L mutation was characterized by severe cognitive and gait disturbances, parkinsonism, and swallowing dysfunction [Citation94].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Three cases of the D178N mutation with the MM genotype with FFI were reported. The age of onset ranged from 46 to 57 years, and no PWSC or MRI hyperintensities were observed in these patients. However, at least one case tested positive for 14-3-3 on CSF, and another case involved a 79-year-old patient with D178N and 129 MV genotype who developed sporadic prion disease and tested positive for 14-3-3 on CSF. No abnormalities were seen on MRI or EEG [Citation81]. In another study, a patient with FFI exhibited symptoms such as dysphagia, loss of appetite, abnormal sleep movements, insomnia or hypersomnolence, and later sleep apnoea, as well as tremor, excessive sweating, constipation, impotence or ataxia. MRI showed mild atrophy, but EEG revealed no abnormalities. Histology revealed spongiform changes in the cingulate gyrus and subiculum, gliosis in the thalamus and inferior olivary nucleus, and Western blot analysis indicated a low amount of type 2 PrPSc with the FFI-type glycosylation pattern [Citation95].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">8.3. Korea</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The first cases of GSS in Korea was identified in 2010. The patient was a 46-year-old female that exhibited P102L mutation and displayed symptoms of ataxic gait, language impairment, and cognitive dysfunction. The patient tested positive for the 14-3-3 protein in the CSF and had atypical non-specific slow waves in EEG [Citation96]. P102L was also observed in a patient from another study with definite GSS, but no clinical symptom details were provided by the investigators [Citation97]. In 2019, P102L was reported in another patient, without any family history and the symptoms included gait disturbance, slurred speech, hand clumsiness, and memory dysfunction. MRI showed high signal intensities in the bilateral cortices and mild cerebellar atrophy, while EEG was normal and the CSF tested positive for 14-3-3 protein [Citation98]. A recent report described the first familial case of GSS in Korea. In this family, there was phenotypic heterogeneity. Imaging showed abnormalities in the hemispheric and caudate nuclei. EEG showed mild diffuse slowing and CSF 14-3-3 was positive [Citation99].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Additionally, the first case of FFI was reported in Korea in 2014, the patient was a 34-year-old male that exhibited D178N mutation (homozygous at residue 129) [Citation100]. Another instance of the D178N mutation was described in a 57-year-old male, who was diagnosed with FFI. The patient presented with an irregular sleep-wake cycle, visual hallucinations, myoclonus, ataxic gait, and weight loss. The CSF analysis for 14-3-3 protein yielded negative results, while the EEG indicated widespread slowing without periodic discharge. MRI and PET scans revealed lesions in the white matter and reduced uptake in the bilateral thalamus, respectively [Citation101].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">8.4. India</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In accordance with a study conducted in the year 2020, the initial instance of GSS syndrome was discovered within India, specifically within an Indian household. This family exhibited a notable occurrence of the 305C > T, p.Pro102Leu mutation within the PRNP gene [Citation102].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">9. Conclusion</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The current review attempted to analyse the epidemiological characteristics, clinical examinations, and laboratory features of CJD patients in Asia. sCJD accounts for the highest number of CJD cases out of the three types and is mainly reported in China. gCJD cases have been observed in different ethnicities – T188K, E200 and E196A are most prevalent in China whereas, in Europe, the most dominant mutations are E200K, V210I, and D178N, and in America, the most common mutations are E200K, and D178N [Citation103,Citation104]. China and Japan have differences in PRNP variant profiles, and the most prevalent mutations in Japan (V180I and M232R) are not reported in China. iCJD incidence has declined with the innovation in medical procedures globally as well as in Asia. Epidemiological studies conducted in the Asian region showed the highest incidence of iCJD in Japan. On the other hand, some countries have not reported a single case which corresponds to the lack of surveillance units in those countries.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The current review establishes the presence of all variants of CJD across Asia (Figure 2). However, in most Asian countries in general and Southeast Asian countries in particular, CJD cases are misdiagnosed and often underreported. There is no proper regional surveillance system for CJD. Since Asia is the most populated continent in the world, the actual global prevalence of CJD cannot be estimated until and unless these countries are accounted for. Concrete and reliable surveillance networks are needed across Asia to evaluate the prevalence and incidence of CJD in the region. Ever since we started working on documenting CJD cases in Asia, some cases have been reported in Pakistan with patients exhibiting symptoms of CJD, however, due to a lack of awareness and proper diagnostic measures, the majority remain underdiagnosed and underreported.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Figure 2. Total cases of CJD reported in Asia from 1986 to 2023. this graph indicates the number of CJD cases reported from the year 1986 to 2023 in Asia. sCJD were the highest among all CJD cases. China (1957) and Japan (1705) reported more cases of sCJD than any Asian country and Hong Kong (1) reported the least. On the other hand, gCJD was highest in Japan (370) and least in India (2). Iatrogenic cases were found to be the least among all CJD types and showed 85 cases overall in Asia, 82 being in Japan and 3 in India.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2024.2311950" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2024.2311950</a><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">SATURDAY, SEPTEMBER 26, 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A nationwide trend analysis in the incidence and mortality of Creutzfeldt–Jakob disease in Japan between 2005 and 2014 with increasing trends of incidence and mortality</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Overall, the AAPCs of age-adjusted CJD-associated mortality rates rose significantly over the study period (3.2%; 95% confidence interval [CI] 1.4–5.1%). The AAPC of the age-adjusted incidence rates also increased (overall 6.4%; 95% CI 4.7–8.1%). The CJD-associated increases in the mortality and incidence rates were especially prominent among adults over the age of 70 years. Given this trend in aging of population, the disease burden of CJD will continue to increase in severity. Our findings thus recommend that policymakers be aware of the importance of CJD and focus on preparing to address the increasing prevalence of dementia.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/09/a-nationwide-trend-analysis-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/09/a-nationwide-trend-analysis-in.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">SATURDAY, SEPTEMBER 26, 2020 </span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">A nationwide trend analysis in the incidence and mortality of Creutzfeldt–Jakob disease in Japan between 2005 and 2014 with increasing trends of incidence and mortality </span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/09/a-nationwide-trend-analysis-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/09/a-nationwide-trend-analysis-in.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">TUESDAY, MAY 19, 2020 </span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">China Sporadic Creutzfeldt-Jakob disease: A retrospective analysis of 104 cases </span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/05/china-sporadic-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/05/china-sporadic-creutzfeldt-jakob.html</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Professor John Collinge on tackling prion diseases<br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, JANUARY 29, 2024</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''The clinical syndrome developed by these individuals can, therefore, be termed iatrogenic Alzheimer’s disease, and Alzheimer’s disease should now be recognized as a potentially transmissible disorder.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monday, January 29, 2024</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">iatrogenic Alzheimer’s disease, Alzheimer’s disease should now be recognized as a potentially transmissible disorder</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">WEDNESDAY, JANUARY 31, 2024</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" />Creutzfeldt Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's And 2024 Alzheimer’s iatrogenic Transmission</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;">26 MARCH 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, retired (medically)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: CJD or Alzheimer's or the same ???</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Sun, 29 Apr 2001 12:45:28 -0700</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: BSE-L@uni-karlsruhe.de</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Greetings List,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">thought some might be interested in this. I have always wondered if CJD and or all TSEs and Alzheimer's could be linked. i have been of the opinion that Alzheimer's is a TSE for a long time, just at the low end of the titre of infectivity scale. i also believe in the accumulation theory. by dose, you could be killed by one sitting, or one injection, or one whatever, depending on the titre of infectivity of that whatever. on the other hand, if the dose is not a lethal dose, over a period of time, the accumulation will become lethal (if consumption continued), and i believe the route/source/titre of infectivity, will be a key roll to the incubation period, and symptoms.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">just my opinion...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">kind regards,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, JULY 22, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer's Disease Update</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://betaamyloidcjd.blogspot.com/2023/07/alzheimers-disease-update.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://betaamyloidcjd.blogspot.com/2023/07/alzheimers-disease-update.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TSE Prion and Endoscopy Equipment</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: Re: CJD * Olympus Endoscope</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Tue, 12 Oct 1999 15:57:03 –0500</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: GOLDSS@...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">References: 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Mr. Goldstine, Hello again, I hope the CDC has not changed your mind, since our phone call, about sending me the information, in which we spoke of. I am still waiting for the information, re-fax. Someone had told me, you would not send me the information, but I told them you would, due to the importance of it pertaining to public safety, and the fact, you are a Doctor. I hope you don't disappoint me, and the rest of the public, and hide the facts, as the CDC and NIH have for years. Olympus can be part of the Truth, or you can be part of the cover-up. We are going to find out, sooner or later.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I already know, as do many more.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Still waiting,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Kind Regards,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, March 02, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Endoscope Maker Olympus Agrees To $646 Million Settlement Over Kickbacks, while still ignoring the elephant in the room, CJD TSE PRIONS Health Inc.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al ***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Evidence For CJD/TSE Transmission Via Endoscopes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From Terry S. Singletary</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.lawyersandsettlements.com/legal-news/olympus-duodenoscopes-linked-to-disease-failure/Olympus-Duodenoscope-Infections-21630.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.lawyersandsettlements.com/legal-news/olympus-duodenoscopes-linked-to-disease-failure/Olympus-Duodenoscope-Infections-21630.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Evidence For CJD/TSE Transmission Via Endoscopes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From Terry S. Singletary, Sr flounder@wt.net 1-24-3</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I have researched human/animal TSEs now for over 5 years due to the death of my Mother from the Heidenhain Variant Creutzfeldt Jakob disease, one of six - known - variants of the infamous 'sporadic' CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I did a little survey several years ago about CJD and ENDOSCOPY in 2001, and then went there again when another article was released recently. However, they seemed to only be concerned with the vCJD strain and risk from endoscopy equipment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">My concerns are if vCJD can be transmitted by blood, and there are now 6 variants of the infamous sporadic CJDs that they are documenting to date, how do they know that none of these 6 variants will not transmit the agent (prion) via blood?...especially since the sporadic CJDs are the only ones documented to date to transmit via the surgical arena and now that the CWD is spreading more and more, who knows about the cattle?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would always read this study and it would bring me back to reality as to how serious/dangerous this agent is in the surgical/medical arena. You might want to read this short abstract from the late, great Dr. Gibbs twice, and let it really sink in. And please remember while reading some of these transmission studies, that most all, if not ALL these agents transmit freely to primates. Humans, of course, are primates.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Regarding claims that:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">'Well, it has never been documented to transmit to humans."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There are two critical factors to think about:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A. CJD/TSEs in the USA are NOT reportable in most states and there is NO CJD/TSE questionnaire for most victims and their families, and the one they are now issuing asks absolutely nothing about route and source of the (prion) agent, only how the disease was diagnosed. Furthermore, the elderly are only very rarely autopsied, ie looking for Alzheimer's or 'FAST Alzheimer's' OR prion disease-related factors and phenomena, such as heart failure caused by disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">B. It is unethical and against the law to do transmission studies of TSEs to humans, they are 100% FATAL.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I suggest you read these case studies about medical arena CJD transmission very carefully:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Evidence For CJD/TSE Transmission Via Endoscopes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From Terry S. Singletary, Sr flounder@wt.net 1-24-3</div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thursday, January 25, 2024</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">TSE Prion Disease, Eyes, Ophthalmology Diagnostic Equipment, Iatrogenic, What If</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://itseprion.blogspot.com/2024/01/tse-prion-disease-eyes-ophthalmology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2024/01/tse-prion-disease-eyes-ophthalmology.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Friday, January 10, 2014</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 years ago;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001 Singeltary on CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 14, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, Sr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">26 MARCH 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, retired (medically)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.neurology.org/doi/10.1212/01.WNL.0000036913.87823.D6</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Singeltary 1999 THE EYES HAVE IT, CJD, AND THEY COULD BE STEALING THEM FROM YOUR LOVED ONE!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdmadcowbaseoct2007.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdmadcowbaseoct2007.blogspot.com/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France iatrogenic CJD TSE Prion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision CORRESPONDENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jacqueline Mikol1 · Jérôme Delmotte1 · Dolorès Jouy1 · Elodie Vaysset1 · Charmaine Bastian1 · Jean‑Philippe Deslys1 ·</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel Comoy1 Received: 10 July 2020 / Revised: 8 September 2020 / Accepted: 25 September 2020 / Published online: 6 October 2020 © The Author(s) 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Non-human primates appeared as the closest model to study human iatrogenic prion diseases [14]: we report here the consequences of variant Creutzfeldt–Jakob disease/bovine spongiform encephalopathy (vCJD/BSE) inoculation in a cynomolgus macaque finger, with the demonstration of an original mode of propagation and the practical risk for professional exposure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The distal right middle finger handpad of a 4-year-old macaque was incised on both lateral sides to induce local inflammation, and then injected with the equivalent of 10 mg of a BSE, orally challenged macaque brain [18]. After an 18 months period of finger clumsiness, the clinical disease (behaviour abnormalities, fear, hyperesthesia, gait disturbances, shaking) began 7.5 years after inoculation and euthanasia took place 2 months later for welfare reasons. Motor conduction velocity of the right median nerve was reduced to one-third of the left counterpart and sensory potential was not detected.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Histological and biochemical studies were performed as previously described. All the elements of the triad were present [7–9]: spongiform change was moderate in neocortex, striatum, brain stem, mild in spinal cord but severe in thalamus and cerebellum; neuronal loss was globally moderate, but severe in cerebellum and sacral spinal cord (vacuolated neurons); gliosis was severe in thalamus, cerebellum and brain stem and moderate elsewhere (Supplementary Fig. 1). ELISA and western blot (WB) showed the expected accumulation of PrPres with BSE glycophoretic pattern at all levels of brain and spinal cord (Supplementary Fig. 2).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the brain, PrPd deposits were laminar into the cortical deep layers, massive into thalamus, basal ganglia, cerebellum, and brain stem. In spinal cord, PrPd was symmetrically distributed, intense in the Substantia gelatinosa and nucleus dorsal of Clarke while decreased at sacral level. Deposits were diverse into the whole CNS: synaptic, perineuronal, reticular aggregates, mini-plaques, plaques, and incomplete florid plaques. The retinal plexiform layers were labelled (Supplementary Fig. 1i). There were no amyloid or tau deposits.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unusual PrPd deposits were observed along dendrites, short and long axons, neuritic threads tracing fne networks of straight lines or like strings of pearls (Supplementary Fig. 3). They were present into deep neocortex, basal ganglia, and motoneurons. Such long processes are not frequent but have been reported in human [13] and experimental studies [10, 22]. PrPd deposits were also noted as very mild into striato-pallidal projections, both limbs of internal capsule and fornix (Supplementary Fig. 3). The presence of PrPd in white matter has been reported (Supplementary text 4).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Peripherally, the expected PrPd was undetectable in lymphoid organs, including spleen, through biochemical or immunohistochemical analyses, while prion replication was detected in the peripheral nervous system (PNS): PrPd staining was visualized in many dorsal root ganglia (DRG) but only in nerves innervating the forelimb site of injection (median and ulnar nerves). At the cellular level, PrPd was limited to ganglia and satellite cells in DRG and Schwann cells (Scs) all along nerves whereas axons were never labelled (Fig. 1). Previously, using postmortem immunohistochemical studies (listed in Supplementary text 5), PrPd has been shown in peripheral nervous system in all forms of human neuropathies, albeit more frequently in vCJD, mostly in posterior root nerve fbres at adaxonal location and/or in ganglion and satellite cells. The restricted amount of PrPd was repeatedly underlined but, recently, prion RTQuiC was positive in all nerves examined [2]. PrPd has also been described, frst in scrapie [17] then in BSE, as limited “adaxonal deposits” or/and Sc deposits, with or without DRG cell involvement (review in [4] and Supplementary text 6). Previous studies of the mode of propagation of PrPd have reported variable observations and analyses depending on strains, host species and genotype (Supplementary text 6); the authors discussed the role of the sensory route of trafficking of prions, the modifications of axonal transport, the centrifugal versus centripetal spread of PrPd .</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">After peripheral infection, accumulation of infectious agent is reputed to occur in lymphoid tissues before direct neuroinvasion [18, 19], even with very little apparent peripheral lymphoreticular deposition [6, 20]. Here, there is no apparent replication/amplification of vCJD/BSE agent in the lymphoid tissues of the exposed macaque. In this model, the neural contamination occurred directly in the highly innervated finger while neuroinvasion appears to occur in Scs along the median nerve to the DRG, with the appearance of the classical labelling of ganglion cells which indicates the onset of the first level of neuronal infection. This model provides direct evidence of the hypothesis of a sequential infection of Scs from the periphery to the CNS, followed by a secondary diffusion into the spinal cord, as already considered by our group [15] and others [1, 3, 11, 12, 21]. It is to note that studies based on intra-sciatic nerve injections in hamsters [16] and transgenic mice [12] had established a rate of transport of infectivity of, respectively, 0.5–2 mm and 0.7 mm per day. This key role of Scs could explain both the low speed of propagation and the discrepancy between the paucity of PrPd into the distal part of the sensory nerves followed by the positivity of DRG, satellite cells and proximal roots.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In conclusion, we have observed that the exposure of a primate to vCJD/BSE through a distal finger lesion induces, after more than 7.5 years of silent incubation, a massive deposit of PrPd , strictly restricted to the nervous system and the eye.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our data suggest a new type of pure unique peripheral nervous contamination in which the Scs would have a major role in the mode of centripetal progression of PrPd in the peripheral nervous system. Moreover, considering the fact that, recently, “a variant CJD diagnosed 7.5 years after occupational exposure” (cryomicrotomy) in a technician was observed [5], this experimental case report supports the risk linked to professional exposure and reinforces the necessity of adequate measures of prevention.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Second death in France in a laboratory working on prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease has killed a person who handled this infectious agent at Inrae in Toulouse. After a first death in 2019, a moratorium on work on this pathogen has been extended.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">By Hervé Morin</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working with these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of mice infected with prions, in another unit of INRAE, in Jouy-en-Josas.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Computer representation of part of a prion protein on a light micrograph of pyramidal nerve cells (neurons, in black) in the cerebellum of the brain. ALFRED PASIEKA / SCIENCE PHOTO LIBRARY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Temporary suspension of work on prions in French public research laboratories</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRESS RELEASE - The general directorates of ANSES, CEA, CNRS, INRAE and Inserm, have decided jointly and in agreement with the Ministry of Higher Education, Research and Innovation to suspend as a precaution all their research and experimentation work relating to prion diseases, for a period of three months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This precautionary measure is motivated by the knowledge of a possible new case of a person suffering from Creutzfeldt-Jakob disease and who worked in a laboratory for research on prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted on July 27, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The suspension period put in place as of this day will make it possible to study the possibility of a link between the observed case and the person's former professional activity and to adapt, if necessary, the preventive measures in force in the research laboratories.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The person with Creutzfeldt-Jakob disease (CJD)1, whose form is not yet known, is a retired INRAE agent. This could be the second case of infectious CJD affecting a scientist who worked on prions, after that of an assistant engineer who died of the disease in 2019, and who was injured in 2010 during of an experiment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Following this death, a general inspection mission was launched in July 2019 by the ministries of research and agriculture with French laboratories handling prions. Submitted in October 2020, the report concluded on the regulatory compliance of the laboratories visited as well as the presence of a risk control culture within the research teams.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research around prion proteins, with high public health issues, allows major advances in the understanding of the functioning of these infectious pathogens, and contributes to results that are transferable to other related degenerative diseases such as Alzheimer's and Alzheimer's diseases. Parkinson's.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At the level of each establishment, regular and transparent information will be provided to all the working communities concerned by this measure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 The disease Creutzfeldt-Jakob disease (CJD) is one of prion diseases - still called encephalopathies subacute spongiform transmitted(TSE) - of diseases rare, characterized by a degeneration rapid and fatal the system nervous central. They are caused by the accumulation in the brain of a normally expressed protein but poorly conformed - the prion protein - which leads to the formation of deleterious aggregates for neurons. For now , no treatment will allow to change the course of these diseases. It can be of origin sporadic , form the most frequent , original genetic or finally to form infectious following a contamination.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Eye procedure raises CJD concerns</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BySTEVE MITCHELL, Medical Correspondent</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WASHINGTON, Nov. 18 (UPI) 2004</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Snip…</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Snip…</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all cases of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20161202052429/https://www.upi.com/Eye-procedure-raises-CJD-concerns/29741100811678/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20161202052429/https://www.upi.com/Eye-procedure-raises-CJD-concerns/29741100811678/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CORRESPONDENCE| VOLUME 20, ISSUE 12, P981, DECEMBER 01, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Safe laboratory management of prions and proteopathic seeds</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Simon Mead Thomas Evans</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">on behalf of the Advisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: December, 2021DOI: https://doi.org/10.1016/S1474-4422(21)00379-3</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prions, the infectious agents of fatal and transmissible neurodegenerative disorders in humans and animals, are comprised of assemblies of misfolded forms of prion protein (PrP). The death of a 33-year-old researcher of prion diseases from variant Creutzfeldt-Jakob disease (ie, the strain of disease that is derived from bovine spongiform encephalopathy) 9 years after a percutaneous exposure to prion-contaminated material, and the death from or diagnosis of prion disease in two other people in Europe after working in prion research, emphasises the importance of statutory guidance for laboratory safety when working with dangerous pathogens.1 People in numerous laboratories handling diagnostic blood, CSF, and other low-risk biofluid samples from patients with or suspected to have Creutzfeldt-Jakob disease have contacted us to suggest that the existing guidance was not sufficiently clear or proportionate. Evidence has accrued for the potential for proteins that are linked to neurodegenerative diseases, other than PrP, to adopt abnormal conformations, self-propagate, and cause transmissible pathologies and diseases in humans and laboratory animals.2, 3 These proteins share a range of pathological properties but are also distinct from prions in important ways, including that there are no known animal or human epidemics or established occupational risks. Experiments that involve inoculating, concentrating, or synthesising these so-called proteopathic seeds have become routine in the past decade, but no statutory guidance is available for safety. Human–human transmission of amyloid β proteopathic seeds has been observed in some specific circumstances that were also shown to transmit prion infection (eg, use of cadaver-derived human pituitary hormones or dura mater in neurosurgery) and can cause iatrogenic cerebral amyloid angiopathy and fatal brain haemorrhage after long latencies.4 The popularity of this field of research, and the long latencies that are to be expected for diseases that are caused by these proteopathic seeds, mean that occupational exposures might not yet have resulted in any clinical consequences. It is prudent, therefore, to consider potential risks from laboratory work involving these agents.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The UK's Advisory Committee for Dangerous Pathogens convened a subgroup to revise guidance for safe working with prions and to consider whether any measures were needed for work with proteopathic seeds, involving experts from research laboratories for prion and other neurodegenerative diseases, infectious disease specialists, pathologists, veterinarians, and health and safety experts. In the new guidance, we emphasise a distinction between high-risk CNS tissues and research samples that contain high concentrations of prions, which need to be managed in specialised laboratories with strict policies, and low-risk biofluids, such as blood and CSF, from patients who are suspected to have Creutzfeldt-Jakob disease with no or low concentrations of prions, which can be managed in a high-throughput diagnostic laboratory setting through adherence to appropriate general laboratory practices.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We also concluded that the poorly defined pathogenicity in humans of proteopathic seeds when prepared in concentrated forms for biochemical, structural, or transmission studies means that they should now be considered as hazard group 2 pathogens, necessitating work in a containment level 2 facility. We recommend a range of safety measures,5 including special attention to risk assessment and staff training; recording of accidental exposures; special caution with the use of any sharp tools to avoid percutaneous injury; work inside a microbiological safety cabinet; and the use of spill trays, absorbent material, and defined procedures to decontaminate equipment and spills to avoid contamination of the laboratory environment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Importantly, we do not recommend any changes to existing procedures for the routine handling of tissues and biofluids from patients with non-prion neurodegenerative conditions for diagnostic or research purposes. We hope that this new guidance will be seen as proportionate and precautionary and help organisations to have increased confidence about the safety of their employees.5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We declare no competing interests. Members of the Advisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup are listed in the appendix.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplementary Material</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Download .pdf (.45 MB)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplementary appendix</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.thelancet.com/cms/10.1016/S1474-4422(21)00379-3/attachment/8e8e4017-5165-46a4-a60a-b8ae6525418e/mmc1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.thelancet.com/cms/10.1016/S1474-4422(21)00379-3/attachment/8e8e4017-5165-46a4-a60a-b8ae6525418e/mmc1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">References 1.Brandel JP Vlaicu MB Culeux A et al. Variant Creutzfeldt-Jakob disease diagnosed 7·5 years after occupational exposure. N Engl J Med. 2020; 383: 83-85 View in Article Google Scholar</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2.Lauwers E Lalli G Brandner S et al.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Potential human transmission of amyloid β pathology: surveillance and risks.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Lancet Neurol. 2020; 19: 872-878</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">View in Article Google Scholar</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3.Jaunmuktane Z Brandner S</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Invited review: the role of prion-like mechanisms in neurodegenerative diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Neuropathol Appl Neurobiol. 2020; 46: 522-545</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">View in Article Google Scholar</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4.Jaunmuktane Z Mead S Ellis M et al.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nature. 2015; 525: 247-250</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">View in Article Google Scholar</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">5.Department of Health and Social Care</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Guidance: minimise transmission risk of CJD and vCJD in healthcare settings.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.gov.uk/government/publications/guidance-from-the-acdp-tse-risk-management-subgroup-formerly-tse-working-group" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gov.uk/government/publications/guidance-from-the-acdp-tse-risk-management-subgroup-formerly-tse-working-group</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Nov 27, 2012 Date accessed: November 2, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Article Info Publication History Published: December 2021 Identification DOI: <a href="https://doi.org/10.1016/S1474-4422(21)00379-3" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1016/S1474-4422(21)00379-3</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Copyright © 2021 Elsevier Ltd. All rights reserved.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.thelancet.com/article/S1474-4422(21)00379-3/fulltext" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.thelancet.com/article/S1474-4422(21)00379-3/fulltext</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a class="enhancr_card_8193914350" href="https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(21)00379-3/fulltext" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">Safe laboratory management of prions and proteopathic seeds</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.thelancet.com/action/showPdf?pii=S1474-4422%2821%2900379-3" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.thelancet.com/action/showPdf?pii=S1474-4422%2821%2900379-3</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, DECEMBER 02, 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The importance of ongoing international surveillance for Creutzfeldt–Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Neil Watson1, Jean-Philippe Brandel2, Alison Green1, Peter Hermann3, Anna Ladogana 4, Terri Lindsay1, Janet Mackenzie1, Maurizio Pocchiari 4, Colin Smith1, Inga Zerr3 and Suvankar Pal 1 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract | Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/s41582-021-00488-7.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/s41582-021-00488-7.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see also;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://thedaily.case.edu/researchers-find-infectious-prions-creutzfeldt-jakob-disease-patient-skin/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://thedaily.case.edu/researchers-find-infectious-prions-creutzfeldt-jakob-disease-patient-skin/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187701/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187701/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nih.gov/news-events/nih-research-matters/prions-found-skin-people-creutzfeldt-jakob-disease" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nih.gov/news-events/nih-research-matters/prions-found-skin-people-creutzfeldt-jakob-disease</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ACDP TSE subgroup minutes, agendas and papers, history</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://app.box.com/s/hhhhg857fjpu2bnxhv6e/folder/2936396377" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://app.box.com/s/hhhhg857fjpu2bnxhv6e/folder/2936396377</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 1, 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SEAC Scientific Steering Committee on TSE Prion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bovineprp.blogspot.com/2022/11/seac-scientific-steering-committee-on.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bovineprp.blogspot.com/2022/11/seac-scientific-steering-committee-on.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">iatrogenic TSE PrP</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, DECEMBER 31, 2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0001330" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0001330</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thursday, November 14, 2013 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion diseases in humans: Oral and dental implications http://transmissiblespongiformencephalopathy.blogspot.com/2013/11/prion-diseases-in-humans-oral-and.html</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;">O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ?<br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full Singeltary Nature comment here;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer's disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by flounder on 05 Nov 2014 at 21:27 GMT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">end...tss</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by flounder on 05 Nov 2014 at 21:27 GMT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><a href="http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492" rel="nofollow" style="color: #338fe9; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Eur J Epidemiol. 2023; 38(7): 757–764.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMCID: PMC10276107</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMID: 37191829</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Angéline Denouel, corresponding author1 Jean-Philippe Brandel,1,2 Danielle Seilhean,1 Jean-Louis Laplanche,3,4 Alexis Elbaz,5 and Stéphane Haik1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Eur J Epidemiol. 2023; 38(7): 757–764.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10276107/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10276107/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, DECEMBER 18, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, DECEMBER 12, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, NOVEMBER 26, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, APRIL 24, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USA 50 State Emergency BSE Conference Call 2001cjd</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, DECEMBER 22, 2023<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Mad Cow That Stole Christmas, 20 Years Later</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, May 24, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/5067</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MAY 19, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">spontaneous my ass, big outbreak of spontaneous mad cow disease evidently, around the same time, strange;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WEDNESDAY, NOVEMBER 08, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE confirmed November 3 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 14, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE case, 3 progeny of case cow to be culled</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, JULY 16, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Atypical BSE detected in a cow in the canton of St. Gallen</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monday, March 20, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE START DATE 2023/01/18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE CONFIRMATION DATE 2023/02/22</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE END DATE 2023/03/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE START DATE 2023/01/21</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE CONFIRMATION DATE 2023/02/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE END DATE 2023/02/06</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE START DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE CONFIRMATION DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE END DATE 2023/03/13</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4876</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE ATYPICAL USA: Netherlands Bovine Spongiform Encephalopathy BSE, atypical strain, L-type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monday, May 22, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NOW, BE AWARE, OIE AND USDA HAVE NOW MADE ATYPICAL SCRAPIE AND ATYPICAL BSE A LEGAL TRADING COMMODITY, WITH NO REPORTING OF SAID ATYPICAL CASES, EXCEPT FOR A VOLUNTARY NOTE ON ANNUAL REPORT...i don't make this stuff up...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">cwd scrapie pigs oral routes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONFIDENTIAL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">LINE TO TAKE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Tue, 9 Jan 2001 16:49:00 -0800</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: BSE-L@uni-karlsruhe.de</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (Tg338 and Tg501).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: No evidence of transmission was found on a first passage in Tg338 nor Tg501ovinized mice, but on second passage, 4/10 Tg338 mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12 Tg501 mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: In this poster, the neuropathological features of the resulting strain are discussed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***is the third potentially zoonotic PD (with BSE and L-type BSE),</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***thus questioning the origin of human sporadic cases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">==============</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2015 CONFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2016 TOKYO</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, April 23, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 1933-690X</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tuesday, December 16, 2014</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Evidence for zoonotic potential of ovine scrapie prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject terms: Biological sciences• Medical research At a glance</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Suspect symptoms</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">28 Mar 01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Like lambs to the slaughter</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">31 March 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">by Debora MacKenzie Magazine issue 2284.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt– Jakob disease: Implications for human health</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Corinne Ida Lasmézas, Jean-Guy Fournier, Virginie Nouvel, +8, and Jean-Philippe DeslysAuthors Info & Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">March 20, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Herein, we made the striking observation that the French natural scrapie strain (but not the U.S. scrapie strain) has the same lesion profile and transmission times in C57BL/6 mice as do the two human TSE strains studied. This strain “affiliation” was confirmed biochemically. There is no epidemiological evidence for a link between sheep scrapie and the occurrence of CJD in humans (25). However, such a link, if it is not a general rule, would be extremely difficult to establish because of the very low incidence of CJD as well as the existence of different isolates in humans and multiple strains in scrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there is still a possibility that in some instances TSE strains infecting humans do share a common origin with scrapie, as pointed out by our findings.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.pnas.org/doi/10.1073/pnas.041490898" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.pnas.org/doi/10.1073/pnas.041490898</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">why do we not want to do TSE transmission studies on chimpanzees $ 5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip... R. BRADLEY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">TUESDAY, JANUARY 16, 2024 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">CIDRAP launches international effort to prepare for possible chronic wasting disease spillover </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Chronic Wasting Disease CWD TSE Prion Spillover to other Species, What If? </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2024/01/cidrap-launches-international-effort-to.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2024/01/cidrap-launches-international-effort-to.html</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;">new prion disease in a new livestock species...</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Friday, May 12, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Camel prion disease, a new emerging disease in North Africa, Lymphoid Tropism, Neuropathological Characterization Update 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">11th Iberian Congress on Prions Barcelona 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;">A Camelid Anti-PrP Antibody Abrogates PrPSc Replication in Prion-Permissive Neuroblastoma Cell Lines</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Daryl Rhys Jones,William Alexander Taylor,Clive Bate,Monique David,Mourad Tayebi </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: March 22, 2010</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1371/journal.pone.0009804" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1371/journal.pone.0009804</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">15 Apr 2018 23:13 GMT MOST RECENT </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion Disease in Dromedary Camels, Algeria </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by flounder on 15 Apr 2018 at 23:13 GMT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div><div dir="ltr" style="outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net</div></div><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-11179971147653297812024-02-05T17:24:00.002-06:002024-02-06T07:11:19.626-06:00Mad Cow Scaremongers, The Center For Consumer Freedom Team, Terry Singeltary Sr Revisited 2024<p><span style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Mad Cow Scaremongers, The Center For Consumer Freedom Team, Terry Singeltary Sr Revisited 2024</span></p><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Mad Cow Scaremongers<br /><br />The Center For Consumer Freedom Team<br /><br />December 20, 2003<br /><br />Secretary of Agriculture Ann Veneman says that “beef is absolutely safe to eat.” Harvard University experts note that the risk of Americans contracting mad cow disease is “as close to zero as you can get.” Every reputable expert tells us that the American meat supply is still safe. And yet a cabal of animal-rights activists and radical opponents of modern farming are already hitting the airwaves for one purpose: to spread fear and needless alarm.<br /><br />These people are activists, not knowledgeable scientists. Their expertise is in scare mongering, not livestock agriculture. Their goal is to promote animal rights and organic-only, 1800s-style agriculture. And their track record is full of doom-and-gloom predictions that never came true.<br /><br />Who are these masters of disaster? A rogues gallery follows:<br /><br />John Stauber — director of the anti-corporate Center for Media & Democracy, and co-author of the 1997 book Mad Cow USA, which was supported financially by the eco-religious Foundation for Deep Ecology. Stauber sits on the national advisory board of the Organic Consumers Association, as reliable a scaremonger as any about the American food supply. Stauber has become a near-ubiquitous media presence in mad-cow-related stories. Just minutes after Secretary Veneman finished her press conference announcing the discovery of a single sick cow, Stauber told CNN — without any evidence whatsoever — that it was just “the tip of an invisible iceberg” and that “mad cow disease is spread throughout North America.”<br /><br />Ronnie Cummins — head of the Organic Consumers Association, a group founded by radical anti-technology guru Jeremy Rifkin. Cummins has openly expressed his hope that a U.S. mad-cow epidemic would fuel a “crisis of confidence” in American food, similar to the one that he claims drove British consumers to “organic” and other high-priced options. In 1998 Cummins told the Minneapolis City Pages that “consumers and farmers would both be better off if people paid twice as much for their meat and ate half as much.” This June he confidently told a Canadian Press reporter that “no case of mad cow has ever been found in a cow raised on an organic farm.” This, actually, is not true. The British Central Veterinary Laboratory reports that in 1995 (at the height of the UK outbreak), there were 215 confirmed cases of mad cow disease from 36 different organic farms. And Germany’s very first case of mad cow disease was diagnosed in a slaughterhouse that only processed organically-raised cattle.<br /><br />Michael Greger — a vegetarian activist doctor who maintains a brisk animal-rights speaking schedule and edits the mad-cow-scare web page of the Organic Consumers Association. He recently provided PETA with a laughable treatise suggesting that the SARS outbreak came from livestock farming. Greger titled his mad-cow stump speech “Mad Cow Disease: Plague of the 21st Century?.” He argues: “although no pigs or chickens have been found with the disease … any animal with a brain has the potential to become infected.” Greger has yet to produce any evidence to support this claim, largely because there isn’t any. Neither hogs nor hens (nor fish, for that matter) suffer from mad-cow-like illnesses. Greger is planning to hit the lecture circuit in an effort to “keep hammering” the meat industry and “ keep this momentum going.”<br /><br />Michael Hansen — the Consumers Union of the United States’ self-proclaimed “expert” on genetically enhanced food, bovine growth hormone, mad cow disease, and any other food issue he deems ripe for scaremongering. When the Canadian mad-cow story broke earlier this year, Hansen blithely suggested that American consumers should eat only grass-fed, “organic,” and other specialty beef. Hansen’s statements on mad cow have appeared in hundreds of media outlets, and his boss, Jean Halloran, has weighed in as well.<br /><br />Howard Lyman — one part animal-rights scold, one part revival tent preacher [click here for video]. Lyman trades on the fact that he was brought up in a cattle-ranching family to imply that his strict vegetarianism is somehow more informed than everyone else’s dietary choices. Lyman famously (and incorrectly) predicted on the “Oprah” show that mad cow disease among Americans would “make AIDS look like the common cold.” Just 14 hours after the U.S. mad-cow announcement, animal-rights terroristand Sierra Club board member Paul Watson published an op-ed asserting that “Howard Lyman predicted this outbreak years ago. Perhaps now the public might pay more attention to this Montana rancher turned vegan. He knows that of which he speaks.” It’s no coincidence that Lyman is on the advisory board of Watson’s violent Sea Shepard Conservation Society. The front page of The Washington Post’s “Style” section seconded Watson’s misleading praise of Lyman with an article titled “Ex-Cattleman’s Warning Was No Bum Steer.”<br /><br />Dave Louthan — a disgruntled former employee of the Washington state meat processing plant where the first U.S. mad-cow case was detected. After losing the job he loved (slaughtering cows), Louthan launched a crusade against beef producers and the U.S. Department of Agriculture, a personal jihad supported by animal rights activists who must otherwise recoil at his admitted passion for bloodying beef cattle. This is a man who clearly enjoyed his work — using a bolt gun to kill cows, buffalo, ostrich, emu and alpaca for Vern’s Moses Lake Meats. He told the New York Times that killing is “really fun,” and beats deboning, which he calls “girls’ work.” In the Seattle Times, Louthan added: “I liked to kill cows. I don’t care if I’m hauling them, feeding them or killing them.”<br /><br />Like many in the meat business, Louthan lost his source of income because of the mad cow scare recklessly promoted by activist groups. But he’s mad, and he’s fighting back. Despite copious evidence to the contrary, he continues to claim that the famous cow he killed (the one that later tested positive) and many others like it were ground into hamburger and entered the human food chain. “The hamburger surprise in your kids’ school lunch,” Dave claims, “has come from mad cows … Your kids will get mad cow from it.”<br /><br />A man of many contradictions, Louthan warns that the U.S. government “is trying to kill you.” He’s calling on anti-beef and animal-rights groups to send him money so he can “keep up the fight.” Yet he admits continuing to eat beef on a daily basis. The verdict is still out on whether or not this former trucker from Texas can successfully change careers from killing cows to assassinating the character of cattlemen.<br /><br />Terry Singletary — A retired machinist and high school dropout, Terry Singletary suffered the tragic loss of his mother to “sporadic” Creutzfeldt-Jakob disease (CJD) in 1997. Desperate to find an explanation for his mother’s death, he has devoted himself to the sad and fruitless task of connecting her death to her diet. Various reports confirm that Mrs. Singletary’s life was claimed by the most common sub-type of CJD (one that accounts for 70 percent of “sporadic” cases). Sporadic CJD, unlike its newer “variant,” is not linked to meat.<br /><br />As the self-appointed international coordinator of CJD Watch, an organization he co-founded with social worker Deborah Oney, Singletary is cited in media reports as an apparent expert on tracking mad cow disease. This despite his lack of formal education and the absence for support from any credible academic, medical or scientific authority. His sensationalist allegations about the safety of U.S. beef have found their way into hundreds of newspapers and broadcasts. Singletary moderates a mad-cow discussion forum run by a vegetarian activist group; his contributions account for more than half the traffic on the “BSE-L” mailing list, which is generally read by real scientists. Animal rights activists and other food-scare artists frequently refer to him as “Dr. Terry Singletary,” apparently an honorary degree as he has yet to finish high school.<br /><br />Like many activists, Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef. Relying entirely on shallow circumstantial evidence and frequent repetition of claims which have been publicly refuted as false, he also blindly insists upon a mad-cow with Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease. His specific allegations have been clearly refuted by Centers for Disease Countrol and Prevention scientists in the journal Neurology.<br /><br />Marion Nestle — New York University’s food scold extraordinaire. Although Nestle concedes that the risk of getting mad cow disease is extremely low, she has nonetheless exploited mad cow fears to promote an anti-corporate, pro-organic creed. She has complained: “Until we have a little consumer protection going on in government, consumers have to take care of themselves.” How do consumers do that? By purchasing organic food, of course. Nestle told Fox News: “This is a very good time to buy organic.”<br /><br />Bruce Friedrich — PETA’s director of vegan outreach. Friedrich revealed his true agenda when he argued: “I think it would be a great thing if all of these fast-food outlets, and these slaughterhouses, and these laboratories, and the banks that fund them exploded tomorrow. I think it’s perfectly appropriate for people to take bricks and toss them through the windows, and everything else along the line. Hallelujah to the people who are willing to do it” [click here for audio]. There is seemingly nothing Bruce won’t do to scare people away from meat, including raising fears about mad cow disease. Under Friedrich’s leadership, PETA representatives have been handing out “emergency vegetarian starter kits,” and holding anti-meat posters outside restaurants and grocery stores all over the country. PETA has also started an aggressive and misleading (what’s new?) advertising campaign to frighten the public into vegetarianism.<br /><br />Neal Barnard — the animal-rights movement’s not-so-secret weapon against meat. Sure he’s a doctor (a non-practicing psychiatrist, actually), but his tirades against dairy foods, beef, chicken, and Atkins-style diets are all informed by his connections to PETA. Barnard sits on the board of The PETA Foundation along with PETA co-founder Ingrid Newkirk. And PETA has funneled nearly $1 million to Barnard’s misnamed “Physicians Committee for Responsible Medicine.” His virulent opposition to animal-based foods was clear in a recent speech at a Food and Drug Administration hearing, where he referred to cheese as “morphine on a cracker” and “dairy crack.” PCRM wasted no time after the mad cow news broke, sending out a press release attacking meat and offering a “vegetarian starter kit” for suddenly fearful carnivores.<br /><br />Caroline Smith DeWaal — director of the food safety program at the quintessential food cop, the Center for Science in the Public Interest. Smith DeWaal, who has been advising consumers to grind their own beef, told The Washington Post: “Taco filling, pizza toppings, hot dogs, processed meats, these are all likely products that can expose consumers to mad cow disease.”<br /><br />Wayne Pacelle — senior vice president of the Humane Society of the United States, a radical animal rights group masquerading as an animal-welfare organization. Pacelle immediately began to lobby the federal government on mad cow, petitioning the U.S. Department of Agriculture the morning after Veneman’s announcement. Pacelle’s goal is to create “a National Rifle Association of the animal rights movement.” His opposition to eating meat is so strong that he has “no problems with the extinction of domestic animals.”<br /><br />Gene Bauston — co-founder of the animal rights group Farm Sanctuary. This group was recently convicted of 210 counts of election fraud, in connection with the $465,000 it illegally trucked into a Florida ballot initiative that gave constitutional rights to pigs. Farm Sanctuary even paid a $50,000 fine. The group issued an e-mail alert to its approximately 18,000 members, requesting that they write to the USDA about mad cow. It also e-mailed a boilerplate “letter to the editor” to over 1,900 activists, asking them to send it to their local newspapers under their own individual signatures. In a gushing article, The New York Times praised the group for its crusade against the processing of “downer” cows. But the paper of record manages to overlook Farm Sanctuary’s early association with the domestic-terrorist Animal Liberation Front, as well as its electioneering mischief.<br /><br />Mark Ritchie — scaremonger-in-chief at the anti-corporate, anti-modern-agriculture, anti-free-trade Institute for Agriculture and Trade Policy (IATP). He blames “industrialized beef production and liberalized trade” for mad cow disease. IATP has started distributing sound-bites for radio broadcast via a 1-800 number.<br /><br />Eric Schlosser — anti-fast food activist and author of Fast Food Nation, which was essentially a screed against the consumption of hamburgers. It’s no surprise that Schlosser is using mad-cow fears to buttress his case. He flatly told CNN: “I don’t think anyone should eat ground beef.” Schlosser’s op-ed in The New York Times was similarly full of doom and gloom.<br /><br />Andrew Knight — a Seattle veterinarian who recently took over the day-to-day operations of the radical Northwest Animal Rights Network. Knight is raising mad-cow panic levels without disclosing his animal-rights agenda. His letter in The Washington Times (Excerpt: “I, for one, will be stocking up on veggie burgers“) identified him only as “Dr. Andrew Knight, Seattle.” The San Diego Union-Tribune printed an expanded version of his Times letter as an op-ed. There he wrote: “it is not improbable that for the one mad cow detected thus far, some 1,700 have passed undetected into the food chain, and that the human form of this lethal disease is silently incubating in numerous unsuspecting beef eaters at present.” A subsequent op-ed in the Toronto Star used the exact same line.<br /><br />Karen Hudson — a consultant for GRACE Factory Farm Project, which can’t stand the idea of efficient, large-scale agriculture. The group even compares animal husbandry to the post-apocalyptic movie, The Matrix. Hudson insists — without providing any evidence — that “mad cow disease is the product of an increasingly industrialized food system.”<br /><br />Katherine DiMatteo — executive director of the Organic Trade Association, which represents the $11 billion organic industry in North America. The group issued a media release arguing: “while the retail price of organic meat is generally greater than conventional, to many consumers, the greater peace of mind is priceless.”<br /><br />Larry Bohlen and Brent Blackwelder — from the radical green group Friends of the Earth. The organization issued a “fact sheet” on mad cow that includes such topics as “mad cow on the rampage.” Blackwelder peddles the familiar and false story that the “best way for people to avoid the risk of mad cow disease is to eat organic, grass fed beef or beef alternatives.” Bohlen’s mad cow comments have found their way into the Los Angeles Times, The Chicago Tribune, and The Seattle Times.<br /><br />Peter Lurie and Wenonah Hauter — food safety “experts” at Ralph Nader’s Public Citizen. Their public comments are designed to raise unfounded fears about eating conventionally-raised beef.<br /><br />Felicia Nestor — chief food-safety worrier at the “whistleblowers’ rights” Government Accountability Project. Nestor worked with Public Citizen to author reports called “The Jungle 2000: Is America’s Meat Fit to Eat?” and “Hamburger Hell: The Flip Side of USDA’s Salmonella Testing Program.” She co-founded the Global Safe Food Alliance, which includes mad cow scaremongers like Public Citizen, the Institute for Agriculture and Trade Policy, and the Organic Consumers Association, along with animal-rights groups like Farm Sanctuary, the Physicians Committee for Responsible Medicine, United Poultry Concerns, and the Humane Society of the United States. Nestor’s hysterical complaints about mad cow have turned up in The New York Times.<br /><br />Andrew Kimbrell and Joseph Mendelson — executive director and legal director of the Center for Food Safety. The Center for Food Safety (not to be confused with the Food and Drug Administration’s Center for Food Safety and Applied Nutrition) gets most of its money from the organic food industry and the lunatic Foundation for Deep Ecology.<br /><br />Robert Cohen — an animal-rights radical who is convinced that cow’s milk is the root of all evil. Cohen warned cattlemen and dairy farmers that mad cow disease would be “a sign” that Americans should “reject your poisons.”<br /><br /><a href="https://consumerfreedom.com/articles/138-mad-cow-scaremongers/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://consumerfreedom.com/articles/138-mad-cow-scaremongers/</a></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary — A retired machinist and high school dropout, Terry Singletary suffered the tragic loss of his mother to “sporadic” Creutzfeldt-Jakob disease (CJD) in 1997. Desperate to find an explanation for his mother’s death, he has devoted himself to the sad and fruitless task of connecting her death to her diet. Various reports confirm that Mrs. Singletary’s life was claimed by the most common sub-type of CJD (one that accounts for 70 percent of “sporadic” cases). Sporadic CJD, unlike its newer “variant,” is not linked to meat.<br /><br />Mad Cow Scaremongers , The Center For Consumer Freedom Team As the self-appointed international coordinator of CJD Watch, an organization he co-founded with social worker Deborah Oney, Singletary is cited in media reports as an apparent expert on tracking mad cow disease. This despite his lack of formal education and the absence for support from any credible academic, medical or scientific authority. His sensationalist allegations about the safety of U.S. beef have found their way into hundreds of newspapers and broadcasts. Singletary moderates a mad-cow discussion forum run by a vegetarian activist group; his contributions account for more than half the traffic on the “BSE-L” mailing list, which is generally read by real scientists. Animal rights activists and other food-scare artists frequently refer to him as “Dr. Terry Singletary,” apparently an honorary degree as he has yet to finish high school.<br /><br />Mad Cow Scaremongers , The Center For Consumer Freedom Team Like many activists, Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef. Relying entirely on shallow circumstantial evidence and frequent repetition of claims which have been publicly refuted as false, he also blindly insists upon a mad-cow with Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease. His specific allegations have been clearly refuted by Centers for Disease Countrol and Prevention scientists in the journal Neurology.</div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><div><div><a href="https://consumerfreedom.com/articles/138-mad-cow-scaremongers/" rel="noreferrer noopener" style="color: #338fe9;" target="_blank">https://consumerfreedom.com/articles/138-mad-cow-scaremongers/</a></div><div><br /></div></div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div dir="ltr">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary “he also blindly insists upon a mad-cow with Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease. His specific allegations have been clearly refuted by Centers for Disease Countrol and Prevention scientists in the journal Neurology.”<br /></div><div><br />2024, iatrogenic AD, what if?<br /><br />Price of TSE prion poker goes up drastically…Terry </div><div><br /></div><div><div><div>Alzheimer’s disease acquired from historic medical treatments</div><div><br /></div><div>30 January 2024</div></div><div><br /></div><div><div>“However, the recognition of transmission of amyloid-beta pathology in these rare situations should lead us to review measures to prevent accidental transmission via other medical or surgical procedures, in order to prevent such cases occurring in future.</div><div><br /></div><div>“Importantly, our findings also suggest that Alzheimer's and some other neurological conditions share similar disease processes to CJD, and this may have important implications for understanding and treating Alzheimer’s disease in the future.”</div><div><br /></div><div><a href="https://www.ucl.ac.uk/prion/news/2024/jan/alzheimers-disease-acquired-historic-medical-treatments" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ucl.ac.uk/prion/news/2024/jan/alzheimers-disease-acquired-historic-medical-treatments</a></div></div><br />not that it matters, but i started telling them from 2001, that Alzheimer’s and other neurological disorders were a TSE prion disease, and were capable of transmitting by iatrogenic routes…kind regards, Terry</div><div><br /></div><div><div>Alzheimer's disease</div><div><br /></div><div>let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease...</div><div><br /></div><div>Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div><br /></div><div>Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?</div><div><br /></div><div>Posted by flounder on 05 Nov 2014 at 21:27 GMT</div><div><br /></div><div>Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?</div><div><br /></div><div>Background</div><div><br /></div><div>Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</div><div><br /></div><div>Methods</div><div><br /></div><div>Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</div><div><br /></div><div>Results</div><div><br /></div><div>I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</div><div><br /></div><div>Conclusions</div><div><br /></div><div>There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</div><div><br /></div><div>end...tss</div><br />Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?<br /><br />Terry Singeltary Sr. Posted by flounder on 05 Nov 2014 at 21:27 GMT<br /><br /><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div><br />MONDAY, JANUARY 29, 2024<br /><br />Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone<br /><br />''The clinical syndrome developed by these individuals can, therefore, be termed iatrogenic Alzheimer’s disease, and Alzheimer’s disease should now be recognized as a potentially transmissible disorder.''</div><div><br /></div><div><a href="https://www.nature.com/articles/s41591-023-02729-2" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.nature.com/articles/s41591-023-02729-2</a></div><div><br /></div><div dir="ltr">Monday, January 29, 2024<br /><br />iatrogenic Alzheimer’s disease, Alzheimer’s disease should now be recognized as a potentially transmissible disorder<br /><br />Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html</a><div><br /></div></div><div dir="ltr"><div dir="ltr">iatrogenic Alzheimer’s disease, Alzheimer’s disease should now be recognized as a potentially transmissible disorder</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html</a><br /></div><br />WEDNESDAY, JANUARY 31, 2024<br /><br />Creutzfeldt Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's And 2024 Alzheimer’s iatrogenic Transmission</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html</a></div><div dir="ltr"><br /></div><div dir="ltr">Singeltary 2001 CJD or Alzheimer's or the same ???</div><div dir="ltr"><br />Subject: CJD or Alzheimer's or the same ???<br /><br />Date: Sun, 29 Apr 2001 12:45:28 -0700<br /><br />From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy<br /><br />To: <span dir="ltr">BSE-L@uni-karlsruhe.de</span><br /><br />Bovine Spongiform Encephalopathy<br /><br />Greetings List,<br /><br />thought some might be interested in this. I have always wondered if CJD and or all TSEs and Alzheimer's could be linked. i have been of the opinion that Alzheimer's is a TSE for a long time, just at the low end of the titre of infectivity scale. i also believe in the accumulation theory. by dose, you could be killed by one sitting, or one injection, or one whatever, depending on the titre of infectivity of that whatever. on the other hand, if the dose is not a lethal dose, over a period of time, the accumulation will become lethal (if consumption continued), and i believe the route/source/titre of infectivity, will be a key roll to the incubation period, and symptoms.<br /><br />just my opinion...end</div><div dir="ltr"><br /></div><div dir="ltr">50 State Emergency BSE Conference Call <span dir="ltr">2001</span></div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div><div><br /></div><div dir="ltr"><div>Professor John Collinge on tackling prion diseases, sCJD accounts for around 1 in 5000 deaths worldwide</div><div><br /></div><div>MONDAY, SEPTEMBER 11, 2023</div><div><br /></div><div>Professor John Collinge on tackling prion diseases</div><div><br /></div><div>“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div><br /></div><div>There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div><br /></div><div><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div><br /></div><div><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br /></div><div><br /></div></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary “A retired machinist and high school dropout”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"> </div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">or</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team “Animal rights activists and other food-scare artists frequently refer to him as “Dr. Terry Singletary,” apparently an honorary degree as he has yet to finish high school.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Terry Singeltary Sr., G.E.D. US Navy!</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">**> Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary “Desperate to find an explanation for his mother’s death, he has devoted himself to the sad and fruitless task of connecting her death to her diet. Various reports confirm that Mrs. Singletary’s life was claimed by the most common sub-type of CJD (one that accounts for 70 percent of “sporadic” cases). Sporadic CJD, unlike its newer “variant,” is not linked to meat.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">or</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team “Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>REPORT OF THE MEETING OF THE OIE AD HOC GROUP ON BOVINE SPONGIFORM ENCEPHALOPATHY RISK ASSESSMENT AND SURVEILLANCE Paris, 18-21 March 2019</div><div><br /></div><div>snip...</div><div><br /></div><div>Potential link between atypical BSE and sporadic Creutzfeldt-Jacob disease (sCJD)</div><div><br /></div><div>It has been reported that the biochemical signature of L-BSE in an intracerebrally inoculated macaque was similar to that of the MM2 cortical subtype of human sCJD (Comoy et al., 2013) raising the possibility that if L-BSE crossed the species barrier into humans it could present as sCJD. In a study involving humanized transgenic mice, Kong et al., 2008, also reported that similarities between L-BSE and sCJD where the electrophoretic pattern of L-BSE and that of Type 2 PrPres from sCJD patients were indistinguishable. The possibility that the two diseases are causally linked was subsequently investigated by Jaumain et al., 2016, who compared the phenotypic traits of L-BSE isolates with those from representative human sCJD cases. Although evidence of an aetiological link was not found, they nevertheless cautioned that an unrecognised form of CJD may emerge from the accidental transfer of L-BSE to humans.</div><div><br /></div><div>At this stage it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that although may be different between atypical strains, nevertheless justifies a consideration of measures to prevent recycling in the cattle population to protect both the human food supply and the ruminant feed chain.</div><div><br /></div><div><a href="https://www.woah.org/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.woah.org/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div class="ydpeef41de5yiv5645556739ydpf282c9e5yiv5209210737"><br /></div><div class="ydpeef41de5yiv5645556739ydpf282c9e5yiv5209210737"><a href="https://web.archive.org/web/20221207232109/https://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://web.archive.org/web/20221207232109/https://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a></div><div class="ydpeef41de5yiv5645556739ydpf282c9e5yiv5209210737"><br /></div><div class="ydpeef41de5yiv5645556739ydpf282c9e5yiv5209210737">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, <br /><br />***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), <br /><br />***is the third potentially zoonotic PD (with BSE and L-type BSE), <br /><br />***thus questioning the origin of human sporadic cases. <br /><br />============== <br /><br />PRION 2015 CONFERENCE</div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a><br /><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">PRION <span dir="ltr">2016 TOKYO</span><br /><br />Saturday, April 23, 2016<br /><br />SCRAPIE <span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential 2016<br /><br />Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr">1933-6896</span> 1933-690X <br /><br /><span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential<br /><br />“Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.”<br /><br />"These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions." <br /><br /><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div dir="ltr"><div><div>***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div><br /></div><div>Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div><br /></div><div><a href="https://www.nature.com/articles/srep11573" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div><br /></div><div><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div></div><div dir="ltr"><br /></div>PLoS One. 2008; 3(8): e3017. Published online 2008 <span dir="ltr">Aug 20.</span> doi: 10.1371/journal.pone.0003017 PMCID: PMC2515088PMID: <span dir="ltr">18714385</span> </div><div><br /></div><div>Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate</div><div><br /></div><div>Emmanuel E. Comoy, 1 , * Cristina Casalone, 2 Nathalie Lescoutra-Etchegaray, 1 Gianluigi Zanusso, 3 Sophie Freire, 1 Dominique Marcé, 1 Frédéric Auvré, 1 Marie-Magdeleine Ruchoux, 1 Sergio Ferrari, 3 Salvatore Monaco, 3 Nicole Salès, 4 Maria Caramelli, 2 Philippe Leboulch, 1 , 5 Paul Brown, 1 Corinne I. Lasmézas, 4 and Jean-Philippe Deslys 1 Neil Mabbott, Editor Author information Article notes Copyright and License information PMC Disclaimer Associated Data</div><div><br /></div><div>Supplementary Materials Go to: Abstract Background</div><div><br /></div><div>Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.</div><div><br /></div><div>Methodology/Principal Findings</div><div><br /></div><div>Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.</div><div><br /></div><div>''Conclusion/Significance''</div><div><br /></div><div>''Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.''</div><div><br /></div><div><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515088/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515088/</a></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0003017" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0003017</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">“Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. “</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>Casalone C Zanusso G Acutis P Ferrari S Capucci L Tagliavini F Monaco S Caramelli M. 2004.</div><div><br /></div><div>Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease.</div><div><br /></div><div dir="ltr">Proc Natl Acad Sci USA 101:<span dir="ltr">3065–3070</span>.</div><div dir="ltr"><br /></div><div dir="ltr">''Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.''<br /></div><div dir="ltr"><br /></div><div dir="ltr">snip...</div><div dir="ltr"><br /></div><div dir="ltr">''To assess molecular and neuropathological characteristics in Italian BSE cases, we have over the last few months collected whole brains of eight Italian cattle that were PrPSc-positive in Western immunoblots. In two cattle, older than other affected bovines, the PrPSc glycotype was clearly different from the BSE-associated PrPSc molecule, and widespread PrP-amyloid plaques were seen in supratentorial brain regions. Unlike typical BSE, the brainstem was less involved and no PrP deposition was detected in the dorsal nucleus of the vagus nerve. Given the biochemical and pathological similarities with sporadic CJD (sCJD) cases linked to type-2 PrPSc (9) and methionine/valine (M/V) polymorphism at codon 129 in the prion protein gene (PRNP), these findings have prompted ongoing strain typing in inbred mice. Although the present findings dictate caution, here we show that a PrPSc type associated with sCJD and the previously undescribed bovine PrPSc show convergent molecular signatures.''<br /></div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://www.pnas.org/doi/full/10.1073/pnas.0305777101" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.pnas.org/doi/full/10.1073/pnas.0305777101</a></div><div><br /></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>News</div><div><br /></div><div>Published: 05 December 2002</div><div><br /></div><div>Prion data suggest BSE link to sporadic CJD</div><div><br /></div><div>Declan Butler</div><div><br /></div><div>Predicting the number of cases of Creutzfeldt–Jakob disease (CJD) in people as a result of transmission of bovine spongiform encephalopathy (BSE) has just got more difficult.</div><div><br /></div><div>Whereas it was thought that BSE only caused a new form of the disease called variant CJD (vCJD), a study in mice from a team led by John Collinge at University College London suggests that it may also cause a disease indistinguishable from the commonest form of classical, or 'sporadic', CJD (E. A. Asante et al. EMBO J. 21, <span dir="ltr">6358–6366; 2002</span>). If the group's mouse model is relevant to the human disease, the results also suggest that the true extent of infection may be difficult to assess because of the large number of asymptomatic carriers.</div><div><br /></div><div>The latest work uses mice engineered to carry the human gene for a cell-membrane protein called PrP. Prion diseases occur when PrP is converted to the abnormal 'prion' form, PrPSc. Collinge has developed a test, based on a standard western blot for analysing proteins, to study PrPSc extracted from the brain. This previously showed disease caused by BSE or vCJD to give a characteristic molecular signature that is distinct from sporadic CJD (J. Collinge, K. C. L. Sidle, J. Meads, J. Ironside and A. F. Hill Nature 383, 685–690; 1996).</div><div><br /></div><div>In their latest experiments, Collinge and his team injected material from the brains of cows with BSE or people with vCJD directly into the brains of two strains of mice with a human PrP genotype known as 129MM. Almost 40% of the British population has this genotype. In one mouse strain, those that became infected showed the usual BSE pattern in the western blot. But in the other, Collinge's team tested 11 mice infected with BSE material using the western blot. Ten of them showed a pattern consistent with sporadic CJD.</div><div><br /></div><div>The number of cases of sporadic CJD have been rising in Britain since the 1970s, and this had been attributed to better monitoring for the condition. But in July, researchers led by Adriano Aguzzi of the University Hospital Zurich reported a sudden increase in sporadic CJD figures in Switzerland in 2001, and suggested that infection with BSE might be to blame (see Nature 418, 266; 2002). Collinge's new data provide worrying molecular evidence that BSE might be to blame for the rise in sporadic CJD.</div><div><br /></div><div>In previous experiments, Collinge had injected BSE and vCJD material into mice with another human PrP genotype, known as 129VV. The new data are thought to be more relevant to the transmission of BSE because all of the known human victims of vCJD have the 129MM genotype. Another worrying finding is that the 129MM mice seem to be more susceptible to developing a subclinical infection, with no obvious symptoms.</div><div><br /></div><div>If a large pool of the British population is carrying a subclinical BSE infection, this would have serious consequences for the potential transmission of the disease, for instance through contaminated surgical instruments. And although laboratory mice are short-lived, infected humans might go on to develop the disease later in life.</div><div><br /></div><div>The UK Department of Health, which has been briefed by Collinge on his findings, says that it will ask its Spongiform Encephalopathy Advisory Committee to consider the results closely at its next meeting in February. Collinge says that an urgent nationwide screening of tonsil material is needed to get a better estimate of the level of infection in the population.</div><div><br /></div><div><a href="https://www.nature.com/articles/420450a" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.nature.com/articles/420450a</a></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">“Collinge's new data provide worrying molecular evidence that BSE might be to blame for the rise in sporadic CJD.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>BSE prions propagate as either variant CJD‐like or sporadic CJD‐like prion strains in transgenic mice expressing human prion protein</div><div><br /></div><div>Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge <span dir="ltr">j.collinge@prion.ucl.ac.uk</span></div><div><br /></div><div>The EMBO Journal (2002) 21: <span dir="ltr">6358 - 6366</span></div><div><br /></div><div><span dir="ltr">https://doi.org/10.1093/emboj/cdf653</span></div><div><br /></div><div>Abstract</div><div><br /></div><div>Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD‐like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE‐derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.</div><div><br /></div><div><a href="https://www.embopress.org/doi/full/10.1093/emboj/cdf653" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.embopress.org/doi/full/10.1093/emboj/cdf653</a></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">“These data suggest that more than one BSE‐derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>Exposure of non-human primates to low doses of BSE/vCJD prions: an update</div><div><br /></div><div>Emmanuel Comoy, Jacqueline Mikol, Jérôme Delmotte, and Jean-Philippe Deslys</div><div><br /></div><div>Direction of Fundamental Research, Division of Prions and Related Diseases (SEPIA), CEA, Fontenay-aux-Roses, France</div><div><br /></div><div>Aims: The occurrence of a high prevalence of healthy carriers (1/2,000) in UK, as revealed by appendix studies, constitutes a sharp contrast with the limited number of clinical cases of variant Creutzfeldt-Jakob disease (vCJD) and the absence of new cases during the past years.</div><div><br /></div><div>The high heterogeneity of consumers’ exposure may explain this apparent paradox: a low number of people were exposed to a high amount of infectivity, whereas a high number of people were exposed to a very low amount of infectivity. Our macaque model might help to assess the clinical evolution of these latter ones and their potential as a source of secondary exposure, notably through blood donations.</div><div><br /></div><div>Material and Methods: We exposed cynomolgus macaques to serial dilutions of BSE-infected material or blood products from different sources. Post mortem histological and biochemical analyses were performed on clinically-affected animals.</div><div><br /></div><div>Results: High dose-inoculated animals developed typical clinical vCJD disease with all the pathognomonic hallmarks after incubation periods ranging from 3 to 8 years. Some low-dosed animals developed clinical signs with atypical patterns after extensive incubation periods, exhibiting lesion and biochemical profiles that differed markedly from the typical disease. Despite the presence of neurological signs and neuronal lesions, classical lesions of spongiform change and presence of cerebral PrPres were inconstant, or even absent, whereas prion infectivity was evidenced after successive transmissions.</div><div><br /></div><div>Conclusions: These observations suggest that low-dose exposure, which would have been the most frequent occurrence during the period of risk and would correspond to healthy carriers, could induce non-typical pathologies that may not be recognized as ‘prion disease’.</div><div><br /></div><div>Funded by: European Commission, French Research Funding Agency, Health Canada</div><div><br /></div><div dir="ltr">''These observations suggest that low-dose exposure, which would have been the most frequent occurrence during the period of risk and would correspond to healthy carriers, could induce non-typical pathologies that may not be recognized as ‘prion disease’.''<br /></div><div><br /></div><div>Non-human primates: a renewed gold standard for prion(-like) diseases?</div><div><br /></div><div>Emmanuel Comoy, Jacqueline Mikol, Jérôme Delmotte, and Jean-Philippe Deslys</div><div><br /></div><div>Direction of Fundamental Research, Division of Prions and Related Diseases (SEPIA), CEA, Fontenay-aux-Roses, France</div><div><br /></div><div>During decades non-human primates (NHP) were considered as gold standard to model human prion diseases until the onset of humanized transgenic mice. The NIH group of Carleton Gajdusek first brought from these models pivotal and founding information about transmissibility, pathogeny and resistance of the different forms of human prion diseases (familial, sporadic or iatrogenic CJD, Kuru …), and in a second time, our primate studies provided the first experimental evidence for a zoonotic potential of BSE.</div><div><br /></div><div>The BSE crisis opened the field to studies about the zoonotic potential of the other animal prion diseases and the iatrogenic (mainly transfusional) risk of subsequent human prion diseases. Transgenic models of mice expressing human PrP emerged at that time and were widely used to assess these questions, with respect to their numerous advantages (expression of the prion protein of concern, little size, limited cost, ethical considerations, availability of dedicated facilities) in comparison to primates. However, primate (mainly macaque) models persisted since they provided in these two domains complementary answers according to their specific features: their lifespan (25–30 years versus 2 years for mice) is more compatible with long incubation periods (as expected) in humans, and most of all their size, their phylogeny and their physiology make them unique to model the potential natural routes of human contamination (oral, intravenous, accidental).</div><div><br /></div><div>During the last decade, we and others described, in different independent primate studies, unexpected observations after prion exposure in several non-optimal conditions, mostly through peripheral routes. The animals developed neurological diseases with either incomplete prion phenotypes, or pathological pictures that would not be suspected as linked to prion. These observations that will be updated here question the real expanse of prion diseases and our capacities do detect them. At a time where structural, conjectural and ethical issues hamper the use of NHP in all the areas of scientific research, these observations together with new technical approaches for refined animal monitoring, renew the interest of these large animal models for prion diseases but also for prion-like diseases, with the first description in a non transgenic model of the transmissibility of Alzheimer’s disease.</div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Prion Conference 2022</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9467582/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9467582/</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div><div><div dir="ltr"><div>''At a time where structural, conjectural and ethical issues hamper the use of NHP in all the areas of scientific research, these observations together with new technical approaches for refined animal monitoring, renew the interest of these large animal models for prion diseases but also for prion-like diseases, with the first description in a non transgenic model of the transmissibility of Alzheimer’s disease.''</div><div><br /></div></div><div>Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease</div><div><br /></div><div>Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div><br /></div><div>***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div><br /></div><div>***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div><br /></div><div>****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div><br /></div><div>***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div><br /></div><div>=====</div><div><br /></div><div><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div><br /></div><div>Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div><br /></div><div>Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div><br /></div><div> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div><br /></div><div> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr">tg650</span> with fecal homogenates. </div><div><br /></div><div> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div><br /></div><div><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div><br /></div><div dir="ltr"><div dir="ltr">''This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.''<br /></div><div><br /></div><div>The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div><br /></div><div>Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div><br /></div><div>Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div><br /></div><div>Published</div><div><br /></div><div>22 August 2022</div><div><br /></div><div><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div><br /></div><div>Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div><br /></div><div>Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div><br /></div><div>Accepted: 7 August 2022</div><div><br /></div><div>HIGHLIGHTS OF THIS STUDY</div><div><br /></div><div>Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div><br /></div><div>Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute.</div><div><br /></div><div>Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div><br /></div><div>suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div><br /></div><div><p class="ydpeef41de5yiv5645556739ydpefb04b2dyiv8229448581p1" style="font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpeef41de5yiv5645556739ydpefb04b2dyiv8229448581s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</span></p></div><div><br /></div><div>Supplementary Information The online version contains supplementary material available at </div><div><br /></div><div><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div><br /></div><div>snip...see full text;</div><div><br /></div><div><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="noreferrer noopener" style="color: #338fe9;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div><br /></div><div><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div></div></div><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div style="font-size: 10pt;"><div style="font-family: arial, helvetica; font-size: 12px; margin-bottom: 24px;"><div dir="ltr" style="margin-bottom: 24px;"><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;"><span style="font-family: arial;">''Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.''</span></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ??? </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Date: September 30, 2002 at 7:06 am PST </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">From: "Belay, Ermias" </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Sent: Monday, September 30, 2002 9:22 AM </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: <span dir="ltr"><span dir="ltr"><span dir="ltr">404-639-3091</span></span></span>). </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated. </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Ermias Belay, M.D. Centers for Disease Control and Prevention </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">-----Original Message----- From: </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Sent: Sunday, September 29, 2002 10:15 AM </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">To: <span dir="ltr"><span dir="ltr"><span dir="ltr">rr26k@nih.gov</span></span></span>; <span dir="ltr"><span dir="ltr"><span dir="ltr">rrace@niaid.nih.gov</span></span></span>; <span dir="ltr"><span dir="ltr"><span dir="ltr">ebb8@CDC.GOV</span></span></span> </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans” <br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">Thursday, April 03, 2008 </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ. </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">snip... *** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***, </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;">snip... full text ;</span></div><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div dir="ltr"><a href="https://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a></div><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div dir="ltr"><span face="Roboto, sans-serif" style="font-size: 16px;">> However, to date, no CWD infections have been reported in people. </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way; </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">sporadic = 54,983 hits </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a><span face="Roboto, sans-serif" style="font-size: 16px;"> </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">spontaneous = 325,650 hits </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a><span face="Roboto, sans-serif" style="font-size: 16px;"> </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;"><br /></span></div><div><span face="Roboto, sans-serif" style="font-size: 16px;">> However, to date, no CWD infections have been reported in people.<br /></span></div></div></div></div><div style="font-size: 10pt;"><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry</div><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***</div><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a></div><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a></div><div style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="noreferrer noopener" style="color: #196ad4; font-family: arial; font-size: 10pt;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a></div><div dir="ltr" style="font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><div>CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div><br /></div><div>Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div><br /></div><div>Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div><br /></div><div>From: Steve Dealler </div><div><br /></div><div>Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div><br /></div><div>To: BSE-L@ References: </div><div><br /></div><div>Dear Terry,</div><div><br /></div><div>An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div><br /></div><div>What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currently then you wont find any!</div><div><br /></div><div>Steve Dealler </div><div><br /></div><div>====</div><div><br /></div><div>''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div><br /></div><div>CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div><br /></div><div>Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div><br /></div><div>Table 9 presents the results of an analysis of these data.</div><div><br /></div><div>There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div><br /></div><div>Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div><br /></div><div>There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div><br /></div><div>The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div><br /></div><div>There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div><br /></div><div>The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div><br /></div><div>snip...</div><div><br /></div><div>It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div><br /></div><div>snip...</div><div><br /></div><div>In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div><br /></div><div>snip...</div><div><br /></div><div>In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div><br /></div><div>snip...see full report ;</div><div><br /></div><div><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div><br /></div><div> <a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div><br /></div><div><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div></div></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary “As the self-appointed international coordinator of CJD Watch, an organization he co-founded with social worker Deborah Oney, Singletary is cited in media reports as an apparent expert on tracking mad cow disease. This despite his lack of formal education and the absence for support from any credible academic, medical or scientific authority”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> Mad Cow Scaremongers , The Center For Consumer Freedom Team “his contributions account for more than half the traffic on the “BSE-L” mailing list, which is generally read by real scientists.”</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>Singeltary 2003</div><div><br /></div><div>January 28, 2003; 60 (2) VIEWS & REVIEWS</div><div><br /></div><div>RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Terry S. Singeltary, retired (medically)</div><div><br /></div><div>Published March 26, 2003</div><div><br /></div><div>26 March 2003</div><div><br /></div><div>Terry S. Singeltary, retired (medically) CJD WATCH</div><div><br /></div><div>I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div><br /></div><div><a href="http://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div><div><br /></div><div>***> Mad Cow Scaremongers , The Center For Consumer Freedom Team Terry Singletary “Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef. Relying entirely on shallow circumstantial evidence and frequent repetition of claims which have been publicly refuted as false”</div><div><br /></div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>14th ICID International Scientific Exchange Brochure -</div><div><br /></div><div>Final Abstract Number: ISE.114</div><div><br /></div><div>Session: International Scientific Exchange</div><div><br /></div><div>Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009</div><div><br /></div><div>T. Singeltary</div><div><br /></div><div>Bacliff, TX, USA</div><div><br /></div><div>Background:</div><div><br /></div><div>An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.</div><div><br /></div><div>Methods:</div><div><br /></div><div>12 years independent research of available data</div><div><br /></div><div>Results:</div><div><br /></div><div>I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.</div><div><br /></div><div>Conclusion:</div><div><br /></div><div>I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.</div><div><br /></div><div><span dir="ltr">http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf</span> </div><div><br /></div><div><a href="https://web.archive.org/web/20110417201250/http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://web.archive.org/web/20110417201250/http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf</a></div><div><br /></div><div>2001 Singeltary on CJD</div><div><br /></div><div>February 14, 2001</div><div><br /></div><div>Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div><br /></div><div>Terry S. Singeltary, Sr</div><div><br /></div><div>Author Affiliations</div><div><br /></div><div>JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214</div><div><br /></div><div dir="ltr">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div dir="ltr"><br /></div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div dir="ltr"><div dir="ltr"><div dir="ltr"><div dir="ltr"><div><div dir="ltr"><div dir="ltr"><div>Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div><br /></div><div>August 10, 2009</div><div><br /></div><div>Greetings,</div><div><br /></div><div>I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. </div><div><br /></div><div>But, while sub-clinical, how many can one exposed human infect? </div><div><br /></div><div>Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? </div><div><br /></div><div>why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? </div><div><br /></div><div>would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? </div><div><br /></div><div>The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div><br /></div><div>The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div><br /></div><div>I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. </div><div><br /></div><div>I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div><br /></div><div>please see history, and the ever evolving TSE science to date ;</div><div><br /></div><div>Saturday, June 13, 2009</div><div><br /></div><div>Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div><br /></div><div><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></div><div><br /></div><div data-setdir="false" dir="ltr"><div>*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;</div><div><br /></div><div><span dir="ltr">http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143</span></div><div><br /></div><div>No competing interests declared.</div><div><br /></div><div><a href="https://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/4f9be886-69fe-4c7c-922b-85b0ecbe6d53" rel="noreferrer noopener" target="_blank">https://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/4f9be886-69fe-4c7c-922b-85b0ecbe6d53</a></div><div><br /></div><div>PLOS ONE Journal</div><div><br /></div><div>IBNC Tauopathy or TSE Prion disease, it appears, no one is sure</div><div><br /></div><div>Terry S. Singeltary Sr., 03 Jul 2015 at 16:53 GMT</div><div><br /></div><div>***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.</div><div><br /></div><div>***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.</div><div><br /></div><div>*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***</div><div><br /></div><div><a href="http://www.plosone.org/annotation/listThread.action?root=86610" rel="noreferrer noopener" target="_blank">http://www.plosone.org/annotation/listThread.action?root=86610</a></div><div><br /></div><div>Singeltary 2000<br /></div></div><div><br /></div><div>BMJ 2000; 320 doi: <a href="https://doi.org/10.1136/bmj.320.7226.8/b" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://doi.org/10.1136/bmj.320.7226.8/b</a> (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div><br /></div><div>02 January 2000 Terry S Singeltary retired</div><div><br /></div><div>Rapid Response: </div><div><br /></div><div>U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div><br /></div><div>In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div><br /></div><div>The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div><br /></div><div>"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div><br /></div><div>Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div><br /></div><div>Something else I find odd, page 16;</div><div><br /></div><div>"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div><br /></div><div>A few more factors to consider, page 15;</div><div><br /></div><div>"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div><br /></div><div>"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div><br /></div><div>"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div><br /></div><div>Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div><br /></div><div>Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div><br /></div><div>To be continued...</div><div><br /></div><div>Terry S. Singeltary Sr. Bacliff, Texas USA</div><div><br /></div><div>Competing interests: No competing interests</div><div><br /></div><div><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div><div><br /></div><div data-setdir="false" dir="ltr"><div>Singeltary 1999</div><div><br /></div><div>US scientists develop a possible test for BSE</div><div><br /></div><div>BMJ 1999; 319 doi: <span dir="ltr">https://doi.org/10.1136/bmj.319.7220.1312b</span> (Published 13 November 1999)</div><div><br /></div><div>Cite this as: BMJ 1999;319:1312</div><div><br /></div><div>15 November 1999</div><div><br /></div><div>Terry S Singeltary</div><div><br /></div><div>NA</div><div><br /></div><div>medically retired</div><div><br /></div><div>Rapid Response:</div><div><br /></div><div>Re: vCJD in the USA * BSE in U.S.</div><div><br /></div><div>In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease.</div><div><br /></div><div>Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know.</div><div><br /></div><div>My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following;</div><div><br /></div><div>vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD.</div><div><br /></div><div>The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?</div><div><br /></div><div>CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.</div><div><br /></div><div>So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.</div><div><br /></div><div>No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;</div><div><br /></div><div>Since 1990 the U.S. has raised 1,250,880,700 cattle;</div><div><br /></div><div>Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;</div><div><br /></div><div>There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;</div><div><br /></div><div>Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;</div><div><br /></div><div>Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.</div><div><br /></div><div>I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.</div><div><br /></div><div>Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.</div><div><br /></div><div>It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........</div><div><br /></div><div>The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.</div><div><br /></div><div>Terry S. Singeltary Sr.</div><div><br /></div><div>Bacliff, Texas 77518 USA</div><div><br /></div><div><span dir="ltr">flounder@wt.net</span></div><div><br /></div><div>Competing interests: No competing interests</div><div><br /></div><div><a href="https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us" rel="noreferrer noopener" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us</a></div><div><br /></div><div>doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div></div><div><br /></div><div>Tracking spongiform encephalopathies in North America</div><div><br /></div><div>Xavier Bosch</div><div><br /></div><div>Available online 29 July 2003. </div><div><br /></div><div>Volume 3, Issue 8, August 2003, Page 463 </div><div><br /></div><div>Volume 3, Number 8 01 August 2003</div><div><br /></div><div>Newsdesk</div><div><br /></div><div>Tracking spongiform encephalopathies in North America</div><div><br /></div><div>Xavier Bosch</div><div><br /></div><div>My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div><br /></div><div>49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div><br /></div><div>Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div><br /></div><div>Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div><br /></div><div>To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div><br /></div><div>Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div><br /></div><div>Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div><br /></div><div>Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div><br /></div><div>Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div><br /></div><div>Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div><br /></div><div>CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div><br /></div><div><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a></div></div></div></div><br /></div><div dir="ltr"><div>Singeltary 2007</div><div><br /></div><div>The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </div><div><br /></div><div>by Philip Yam </div><div><br /></div><div>''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</div><div><br /></div><div>Revisiting Sporadic CJD</div><div><br /></div><div>It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous <span dir="ltr">www.madcow.org</span> Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</div><div><br /></div><div>223</div><div><br /></div><div>prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</div><div><br /></div><div>Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on <span dir="ltr">www.vegsource.com</span> and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</div><div><br /></div><div>Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</div><div><br /></div><div>224 CHAPTER 14</div><div><br /></div><div>Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</div><div><br /></div><div>Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</div><div><br /></div><div>Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</div><div><br /></div><div>Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</div><div><br /></div><div>Laying Odds 225</div><div><br /></div><div>(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</div><div><br /></div><div>Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</div><div><br /></div><div>The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</div><div><br /></div><div>226 CHAPTER 14</div><div><br /></div><div>infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</div><div><br /></div><div>A Case for Undercounting</div><div><br /></div><div>The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</div><div><br /></div><div>The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</div><div><br /></div><div>Laying Odds 227</div><div><br /></div><div>(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</div><div><br /></div><div>In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</div><div><br /></div><div>One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a one in-a-million lottery, it’s more like one-in-2.5-million for African Americans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</div><div><br /></div><div>Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</div><div><br /></div><div>SNIP...SEE FULL TEXT;</div><div><br /></div><div><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div><div><br /></div><div>Singeltary Submission SEAC 2007</div><div><br /></div><div>SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</div><div><br /></div><div>This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</div><div><br /></div><div>SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </div><div><br /></div><div>ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</div><div><br /></div><div>41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </div><div><br /></div><div><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a></div></div></div><div><br /></div><div>2023</div><div><br /></div><div>Eur J Epidemiol. 2023; 38(7): 757–764.</div><div><br /></div><div>Published online 2023 <span dir="ltr">May 16.</span> doi: 10.1007/s10654-023-01004-5</div><div><br /></div><div>PMCID: PMC10276107</div><div><br /></div><div>PMID: <span dir="ltr">37191829</span></div><div><br /></div><div>The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div><br /></div><div>Angéline Denouel, corresponding author1 Jean-Philippe Brandel,1,2 Danielle Seilhean,1 Jean-Louis Laplanche,3,4 Alexis Elbaz,5 and Stéphane Haik1,2</div><div><br /></div><div>snip...</div><div><br /></div><div>Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.</div><div><br /></div><div>snip...</div><div><br /></div><div dir="ltr">Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.</div><div dir="ltr"><br /><div><div>Eur J Epidemiol. 2023; 38(7): 757–764.</div><div><br /></div><div dir="ltr"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10276107/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10276107/</a></div><div dir="ltr"><br /></div><div dir="ltr">''These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.''</div></div></div></div></div><div dir="ltr"><br /></div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div dir="ltr"><div>MONDAY, DECEMBER 18, 2023</div><div><br /></div><div>Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div><br /></div><div><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html</a></div><div><br /></div><div>TUESDAY, DECEMBER 12, 2023</div><div><br /></div><div>CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023</div><div><br /></div><div><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a></div><div><br /></div><div>SUNDAY, NOVEMBER 26, 2023</div><div><br /></div><div>The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div><br /></div><div><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html</a></div><div><br /></div><div>MONDAY, APRIL 24, 2023 </div></div><div dir="ltr"><div><br /></div><div>2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older </div><div><br /></div><div><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html</a></div><div><br /></div></div></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">USA 50 State Emergency BSE Conference Call <span dir="ltr">2001cjdhttps://<span dir="ltr">tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</span></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html?m=0" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html?m=0</a></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">FRIDAY, DECEMBER 22, 2023<br /><br />The Mad Cow That Stole Christmas, 20 Years Later<br /><br /><a href="https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Wednesday, May 24, 2023<br /><br />***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br /><br /><a href="https://wahis.woah.org/#/in-review/5067" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/5067</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="noreferrer noopener" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><a href="https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification" rel="noreferrer noopener" target="_blank">https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">MAY 19, 2023<br /><br /><a href="https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse" rel="noreferrer noopener" target="_blank">https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;</div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;<br /><br />Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023<br /><br />''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...<br /><br /><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="noreferrer noopener" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="noreferrer noopener" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />spontaneous my ass, big outbreak of spontaneous mad cow disease evidently, around the same time, strange;<br /><br />WEDNESDAY, NOVEMBER 08, 2023 <br /><br />Ireland Atypical BSE confirmed November 3 2023 <br /><br /><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />TUESDAY, NOVEMBER 14, 2023 <br /><br />Ireland Atypical BSE case, 3 progeny of case cow to be culled <br /><br /><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">SUNDAY, JULY 16, 2023 <br /><br />Switzerland Atypical BSE detected in a cow in the canton of St. Gallen <br /><br /><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type<br /><br />Switzerland Bovine Spongiform Encephalopathy Atypical L-Type<br /><br />Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><a href="https://wahis.woah.org/#/in-review/4962" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/4962</a></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Monday, March 20, 2023 <br /><br />WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type <br /><br /><span dir="ltr"><a href="https://wahis.woah.org/#/in-review/4977" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/4977</a></span><br /><br /><span dir="ltr"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="noreferrer noopener" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="noreferrer noopener" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">BRAZIL BSE START DATE 2023/01/18<br /><br />BRAZIL BSE CONFIRMATION DATE 2023/02/22<br /><br />BRAZIL BSE END DATE 2023/03/03<br /><div><br /></div><a href="https://wahis.woah.org/#/in-review/4918" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><a href="https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />SPAIN BSE START DATE 2023/01/21<br /><br />SPAIN BSE CONFIRMATION DATE 2023/02/03<br /><br />SPAIN BSE END DATE 2023/02/06<br /><br /><a href="https://wahis.woah.org/#/in-review/4888" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /><a href="https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html" rel="noreferrer noopener" target="_blank">https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html</a></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">NETHERLANDS BSE START DATE 2023/02/01<br /><br />NETHERLANDS BSE CONFIRMATION DATE 2023/02/01<br /><br />NETHERLANDS BSE END DATE 2023/03/13<br /><br /><a href="https://wahis.woah.org/#/in-review/4876" rel="noreferrer noopener" target="_blank">https://wahis.woah.org/#/in-review/4876</a><br /><br /><a class="enhancr_card_7873935313" href="https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html" rel="noreferrer noopener" target="_blank">BSE ATYPICAL USA: Netherlands Bovine Spongiform Encephalopathy BSE, atypical strain, L-type</a><br /><br />PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...<br /><br />Monday, May 22, 2023 <br /><br />***> BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES? <br /><br /><a class="enhancr_card_8088187668" href="https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html" rel="noreferrer noopener" target="_blank">BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?</a><br /><br />NOW, BE AWARE, OIE AND USDA HAVE NOW MADE ATYPICAL SCRAPIE AND ATYPICAL BSE A LEGAL TRADING COMMODITY, WITH NO REPORTING OF SAID ATYPICAL CASES, EXCEPT FOR A VOLUNTARY NOTE ON ANNUAL REPORT...i don't make this stuff up...terry<br /><br />cwd scrapie pigs oral routes <br /><br />***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** <br /><br /><blockquote type="cite">*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** <br /></blockquote><br />***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in <span dir="ltr">8/18</span> (44%), and the tonsil in <span dir="ltr">10/25</span> (40%). <br /><br />***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a><br /><br /><span dir="ltr"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.<br /><br /><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">CONFIDENTIAL<br /><br />EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY<br /><br />LINE TO TAKE<br /><br />3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- <br /><br />"There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.<br /><br />DO Hagger RM 1533 MT Ext 3201<br /><br /><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...<br /><br /><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a><br /><br />May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a><br /><br />3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a><br /><br /><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...<br /><br /><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....<br /><br /><div><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a><br /></div><div><br /></div><div>BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001<br /><br />Date: Tue, 9 Jan 2001 16:49:00 -0800<br /><br />From: "Terry S. Singeltary Sr."<br /><br />Reply-To: Bovine Spongiform Encephalopathy<br /><br />To: <span dir="ltr">BSE-L@uni-karlsruhe.de</span> <br /><br /><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div>Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (<span dir="ltr">Tg338</span> and <span dir="ltr">Tg501</span>).</div><div><br /></div><div>Results: No evidence of transmission was found on a first passage in <span dir="ltr">Tg338</span> nor Tg501ovinized mice, but on second passage, <span dir="ltr">4/10</span> <span dir="ltr">Tg338</span> mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12 <span dir="ltr">Tg501</span> mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing.</div><div><br /></div><div>Conclusions: In this poster, the neuropathological features of the resulting strain are discussed.</div><div><br /></div><div><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a> <br /></div></div><div style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations <br /><br />*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, <br /><br />***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), <br /><br />***is the third potentially zoonotic PD (with BSE and L-type BSE), <br /><br />***thus questioning the origin of human sporadic cases. <br /><br />============== <br /><br />PRION 2015 CONFERENCE<br /><br /><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">PRION <span dir="ltr">2016 TOKYO</span><br /><br />Saturday, April 23, 2016<br /><br />SCRAPIE <span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential 2016<br /><br />Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr">1933-6896</span> 1933-690X <br /><br /><span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential<br /><br />Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. <br /><br />These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. <br /><br /><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Transmission of scrapie prions to primate after an extended silent incubation period<br /><br />*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.<br /><br />*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.<br /><br />*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.<br /><br /><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.<br /><br />***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.<br /><br />***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.<br /><br /><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***<br /><br />Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.<br /><br /><span dir="ltr"><a href="https://www.nature.com/articles/srep11573">https://www.nature.com/articles/srep11573</a><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Tuesday, December 16, 2014 <br /><br />Evidence for zoonotic potential of ovine scrapie prions <br /><br />Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,<span dir="ltr">1, Naima Aron</span>,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications <br /><br />Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 <br /><br />Abstract <br /><br />Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. <br /><br />***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. <br /><br />***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. <br /><br />Subject terms: Biological sciences• Medical research At a glance<br /><br /><div><div><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a><br /></div><div><br /></div><div>2001</div><div><br /></div><div>Suspect symptoms</div><div><br /></div><div>What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?</div><div><br /></div><div>28 Mar 01</div><div><br /></div><div>Like lambs to the slaughter</div><div><br /></div><div>31 March 2001</div><div><br /></div><div>by Debora MacKenzie Magazine issue 2284.</div><div><br /></div><div>FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.</div><div><br /></div><div>Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.</div><div><br /></div><div>Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.</div><div><br /></div><div>"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.</div><div><br /></div><div><a href="http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html</a></div><div><br /></div><div>Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt– Jakob disease: Implications for human health</div><div><br /></div><div>Corinne Ida Lasmézas, Jean-Guy Fournier, Virginie Nouvel, +8, and Jean-Philippe DeslysAuthors Info & Affiliations</div><div><br /></div><div>March 20, 2001</div><div><br /></div><div>The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.</div><div><br /></div><div>snip...</div><div><br /></div><div>Herein, we made the striking observation that the French natural scrapie strain (but not the U.S. scrapie strain) has the same lesion profile and transmission times in C57BL/6 mice as do the two human TSE strains studied. This strain “affiliation” was confirmed biochemically. There is no epidemiological evidence for a link between sheep scrapie and the occurrence of CJD in humans (25). However, such a link, if it is not a general rule, would be extremely difficult to establish because of the very low incidence of CJD as well as the existence of different isolates in humans and multiple strains in scrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there is still a possibility that in some instances TSE strains infecting humans do share a common origin with scrapie, as pointed out by our findings.</div><div><br /></div></div><div><a href="https://www.pnas.org/doi/10.1073/pnas.041490898">https://www.pnas.org/doi/10.1073/pnas.041490898</a></div><div><br /></div>why do we not want to do TSE transmission studies on chimpanzees $ 5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis. <br /><br />snip... R. BRADLEY <br /><br /><a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a> </div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br />1: J Infect Dis 1980 Aug;142(2):205-8 <br /><br />Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates<br /><br />Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. <br /><br />Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. <br /><br />snip... <br /><br />The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: <span dir="ltr">6997404</span><br /><br /><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 <br /><br /><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Nature. 1972 Mar 10;236(5341):73-4. <br /><br />Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis) <br /><br />Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 <br /><br />Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) <br /><br />C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland <br /><br />SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).<br /><br /><span dir="ltr"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><a href="http://scrapie-usa.blogspot.com/">http://scrapie-usa.blogspot.com/</a><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><span dir="ltr"><br /></span></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><a href="http://nor-98.blogspot.com/">http://nor-98.blogspot.com/</a><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;">Singeltary Comment Docket No: 2002N-0273 (formerly Docket No. 02N-0273)</div><div data-setdir="false" dir="ltr" style="-webkit-text-size-adjust: auto; font-family: arial; font-size: 16px;"><div><div><br /></div><div>MY comments/questions are as follows ;</div><div><br /></div><div>1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?</div><div><br /></div><div>*** Suppressed peer review of Harvard study October 31, 2002 *** </div><div><br /></div><div><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div><br /></div><div>2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today ? </div><div><br /></div><div>snip...see full text; </div><div><br /></div><div><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div><br /></div><div>Singeltary Full Comments Submissions;</div><div><br /></div><div><a href="http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div><br /></div><div><a href="http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf</a></div><div><br /></div><div>FSIS, HARVARD, REPLY TO SINGELTARY </div><div><br /></div><div><a href="https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div><br /></div><div dir="ltr"><a href="https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf</a><br /></div><div><br /></div><div data-setdir="false" dir="ltr"><div>Wednesday, May 24, 2023 </div><div><br clear="none" /></div><div>WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div><br clear="none" /></div><div><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="noreferrer noopener" shape="rect" style="color: #196ad4;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div><br clear="none" /></div><div>ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div><br clear="none" /></div><div>May 2, 2023, i submitted this to the USDA et al;</div><div><br clear="none" /></div><div>Docket No. APHIS–<span dir="ltr">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div><br clear="none" /></div><div>ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div><br clear="none" /></div><div>ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div><div><br clear="none" /></div><div>Document</div></div><div><br /></div><div>Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</div><div><br /></div><div><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a></div><div><br /></div><div>APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission Comment from Terry Singeltary Posted by the Animal and Plant Health Inspection Service on Jun 19, 2019</div><div><br /></div><div><a href="https://www.regulations.gov/comment/APHIS-2018-0087-0002" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/APHIS-2018-0087-0002</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf</a></div><div><br /></div><div>Control of Chronic Wasting Disease OMB Control Number: 0579-0189APHIS-2021-0004 Singeltary Submission</div><div><br /></div><div><a href="https://www.regulations.gov/comment/APHIS-2021-0004-0002" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0004-0002</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf</a></div><div><br /></div><div>Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification</div><div><br /></div><div><a href="https://www.regulations.gov/document/APHIS-2018-0011-0003" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/document/APHIS-2018-0011-0003</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf</a></div><div><br /></div><div>APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</div><div><br /></div><div>Comment from Singeltary Sr., Terry</div><div><br /></div><div>Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022</div><div><br /></div><div><a href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div><div><br /></div><div>Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed </div><div><br /></div><div>PUBLIC SUBMISSION</div><div><br /></div><div>Comment from Terry Singeltary Sr.</div><div><br /></div><div>Posted by the Food and Drug Administration on May 17, 2016 Comment</div><div><br /></div><div>Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission </div><div><br /></div><div><a href="https://www.regulations.gov/comment/FDA-2003-D-0432-0011" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/FDA-2003-D-0432-0011</a><br /></div><div><br /></div><div dir="ltr"><div>***> Monday, November 13, 2023</div><div><br /></div><div>Food and Drug Administration's BSE Feed Regulation (21 CFR <span dir="ltr">589.2000</span>) Singeltary Another Request for Update 2023</div><div><br /></div><div><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a></div></div></div><div><div dir="ltr"><span style="color: #222222; font-family: Harding, Palatino, serif; font-size: 18px;"><br /></span></div><div data-setdir="false" dir="ltr"><div>Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie</div><div><br /></div><div>Marina Betancor, Belén Marín, Alicia Otero, Carlos Hedman, Antonio Romero, Tomás Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan José Badiola, Olivier Andréoletti & Rosa Bolea Veterinary Research</div><div><br /></div><div>volume 54, Article number: 89 (2023)</div><div><br /></div><div>Abstract</div><div><br /></div><div>The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle. Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease and tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prion seeding activity was detected during in vitro propagation of brain samples from the inoculated animals. These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.</div><div><br /></div><div>Snip…</div><div><br /></div><div>Further in vivo experiments challenging different mouse lines have been started in order to confirm the infectivity of the PMCA products obtained in this study. However, in conclusion, our findings show that the propagation of atypical scrapie in cattle leads to the emergence of BSE-like seeding activity. This is a concerning issue with far-reaching implications for public health and food safety. The possibility of interspecies transmission of prion diseases and the emergence of new prion strains highlight the critical need for continued surveillance and monitoring of these diseases in both animal and human populations. Early detection of prion diseases is crucial, and highly sensitive detection techniques such as PMCA can play an important role in this regard.</div><div><br /></div><div><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2" rel="noreferrer noopener" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2</a></div><div><br /></div><div>Volume 26, Number <span dir="ltr">6—June</span> 2020 Research </div><div><br /></div><div>Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice</div><div><br /></div><div>Alba Marín-Moreno1, Alvina Huor1, Juan Carlos Espinosa, Jean Yves Douet, Patricia Aguilar-Calvo2, Naima Aron, Juan Píquer, Sévérine Lugan, Patricia Lorenzo, Cecile Tillier, Hervé Cassard, Olivier Andreoletti, and Juan María TorresComments to Author Author affiliations: Centro de Investigación en Sanidad Animal, Madrid, Spain (A. Marín-Moreno, J.C. Espinosa, P. Aguilar-Calvo, J. Píquer, P. Lorenzo, J.M. Torres); Interactions Hôte Agent Pathogène–École Nationale Vétérinaire de Toulouse, Toulouse, France (A. Huor, J.Y. Douet, N. Aron, S. Lugan, C. Tiller, H. Cassard, O. Andreoletti)</div><div><br /></div><div>Abstract</div><div><br /></div><div>Classical bovine spongiform encephalopathy (BSE) is the only zoonotic prion disease described to date. Although the zoonotic potential of atypical BSE prions have been partially studied, an extensive analysis is still needed. We conducted a systematic study by inoculating atypical BSE isolates from different countries in Europe into transgenic mice overexpressing human prion protein (PrP): TgMet129, TgMet/Val129, and TgVal129. L-type BSE showed a higher zoonotic potential in TgMet129 mice than classical BSE, whereas Val129-PrP variant was a strong molecular protector against L-type BSE prions, even in heterozygosis. H-type BSE could not be transmitted to any of the mice. We also adapted 1 H- and 1 L-type BSE isolate to sheep-PrP transgenic mice and inoculated them into human-PrP transgenic mice. Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.</div><div><br /></div><div>Snip…</div><div><br /></div><div>The transmission of atypical BSEs into sheep resulted in the emergence of prions similar to types 1 and 2 sCJD in terms of mean survival times, attack rates, PrPres profile, and PrPres deposition pattern in the brain of human-PrP transgenic mice. The similarities between the sheep-adapted atypical BSE prions propagated into our human-PrP transgenic mouse lines and sCJD prions could suggest a link between them. The well-established dogma that sCJD is a spontaneous disorder unrelated to animal prion disease has been questioned in a previous study given the resemblance of scrapie prions transmitted into human transgenic mouse models to sCJD strains (26); however, the data from that study do not unequivocally establish a causative link between exposure to sheep scrapie and the subsequent appearance of sCJD in humans, and the same could apply to our findings. An alternative explanation that cannot be ruled out is that, although being different strains, only a limited number of phenotypes could be generated for the human-PrP, indicating phenotypic convergence. Updates to old epidemiologic research is needed to reconsider all these results involving a possible infectious origin of sCJD. In any case, continuing the characterization of this newly emerged prion strain would be useful to finally discarding or refuting a link with sCJD prions.</div><div><br /></div><div><a href="https://wwwnc.cdc.gov/eid/article/26/6/18-1790_article" rel="noreferrer noopener" target="_blank">https://wwwnc.cdc.gov/eid/article/26/6/18-1790_article</a></div><div><br /></div><div>“The transmission of atypical BSEs into sheep resulted in the emergence of prions similar to types 1 and 2 sCJD in terms of mean survival times, attack rates, PrPres profile, and PrPres deposition pattern in the brain of human-PrP transgenic mice. The similarities between the sheep-adapted atypical BSE prions propagated into our human-PrP transgenic mouse lines and sCJD prions could suggest a link between them.”</div><div><br /></div><div>Article Published: 16 December 2014 Evidence for zoonotic potential of ovine scrapie prions Hervé Cassard, Juan-Maria Torres, Caroline Lacroux, Jean-Yves Douet, Sylvie L. Benestad, Frédéric Lantier, Séverine Lugan, Isabelle Lantier, Pierrette Costes, Naima Aron, Fabienne Reine, Laetitia Herzog, Juan-Carlos Espinosa, Vincent Beringue & Olivier Andréoletti </div><div><br /></div><div>Article number: 5821 (2014)</div><div><br /></div><div>Abstract Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div><br /></div><div>Snip…</div><div><br /></div><div>Do our transmission results in tgHu imply that sheep scrapie is the cause of sCJD cases in humans? This question challenges well-established dogma that sCJD is a spontaneous disorder unrelated to animal prion disease. In our opinion, our data on their own do not unequivocally establish a causative link between natural exposure to sheep scrapie and the subsequent appearance of sCJD in humans. However, our studies clearly point out the need to re-consider this possibility. Clarification on this topic will be aided by informed and modern epidemiological studies to up-date previous analysis that was performed at the end of the last century3,4. The value of such an approach is highlighted by the implementation in the year 2000 of large-scale active animal TSE surveillance programs around the world that provided an informed epidemiological-based view of the occurrence and geographical spread of prion disease in small ruminant populations51. The fact that both Australia and New-Zealand, two countries that had been considered for more than 50 years as TSE-free territories, were finally identified positive for atypical scrapie in their sheep flocks provides an example of how prion dogma can be reversed52. However, the incubation period for prion disease in humans after exposure to prions via the peripheral route, such as in iatrogenic CJD transmission and Kuru, can exceed several decades53,54. In this context, it will be a challenge to combine epidemiological data collected contemporarily in animal populations and humans to investigate the existence of a causative link between prion disease occurrence in these different hosts. Furthermore, it is crucial to bear in mind that sporadic sCJD in humans is a rare disease (1–2 individuals per million of the population per year) and that scrapie has been circulating in small ruminants populations used for food purposes for centuries. Consequently, it is our opinion that even if a causative link was established between sheep scrapie exposure and the occurrence of certain sCJD cases, it would be wrong to consider small ruminant TSE agents as a new major threat for public health. Despite this, it remains clear that our data provide a new impetus to establish the true zoonotic potential of sheep scrapie prions.</div><div><br /></div><div><a href="https://www.nature.com/articles/ncomms6821" rel="noreferrer noopener" target="_blank">https://www.nature.com/articles/ncomms6821</a></div></div><div dir="ltr"><br /></div><div data-setdir="false" dir="ltr"><div><div>90th General Session • Paris, 21 – 25 May 2023 Final Report 2023</div><div><br /></div><div>snip...</div><div><br /></div><div>China (People’s Rep. of), noting that cases of atypical BSE were being reported almost every year globally and that research of atypical BSE have shown that atypical BSE agents could be transmitted in cattle through feed and could be transformed into classical BSE after passage, recommended that WOAH continue to keep atypical BSE as a listed disease. China (People’s Rep. of) emphasised that, with regard to Article 11.4.5bis., after the occurrence of atypical BSE, relevant Members should conduct investigations to find the cause of the disease or rule out the possibility of feed transmission, take control measures, and submit investigation reports, so as to maintain their WOAH-recognised status of negligible/controlled BSE risk.</div><div><br /></div><div>Republic of Korea generally supported the comment from China (People’s Rep. of) on atypical BSE.</div><div><br /></div><div>Austria, speaking on behalf of the 27 Member States of the EU, thanked the Commission for its work. Austria expressed general support for adoption of the revised chapter but considered that, for text related to recycling, it would be better to consider all BSE agents and not only the classical BSE agent, noting that the risk of recycling of atypical BSE could not be ruled out, as stated in point 1 of Article 11.4.1. Austria also suggested some editorial changes to the chapter for consideration by the Code Commission at its next meeting.</div><div><br /></div><div>In response to the comment by Zimbabwe on behalf of the 54 Member States of the African Union and the WOAH Africa Region, Dr Bonbon highlighted that one of objectives of the revision was to address Members’ concerns about the feasibility of meeting the surveillance requirements outlined in the chapter, and he reminded Members that the new surveillance requirements had been developed by an ad hoc Group, and took these concerns into consideration. Dr Bonbon explained that it was up to each Member to decide what the targeted population was and how many of those animals were to be tested in accordance with the provisions of Article 11.4.18. He reiterated that the provisions would not require a lot of testing. Furthermore, with regard to the second concern raised, Dr Bonbon emphasised that it was not possible to rely only on clinical signs as they were not pathognomonic for either classical or atypical BSE and it was not possible to rely only on there being no reported BSE cases in the past when granting official BSE risk status, given that atypical BSE was assumed to occur spontaneously in any bovine population. Dr Bonbon explained that if Members wanted to obtain official BSE status, the recommendations needed to be complied with. He also highlighted that even Members with undetermined BSE risk could export bovine commodities in accordance with the relevant articles in the chapter and that no trade problem had been reported to WOAH by the concerned countries in this respect.</div></div><br /></div><div dir="ltr"><div class="ydpa31d830yiv3263669459c-article-section__content" id="ydpa31d830yiv3263669459Abs1-content" style="color: #222222; font-family: Harding, Palatino, serif; font-size: 18px; margin-bottom: 40px; padding-left: 0px; padding-top: 8px;"><p style="margin-bottom: 24px; margin-top: 0px;"><a href="https://www.woah.org/app/uploads/2023/06/a--fr-sg-2023.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.woah.org/app/uploads/2023/06/a--fr-sg-2023.pdf</a></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Cattle with the <span dir="ltr">EK211</span> </span><span class="ydpa31d830yiv3263669459s2" style="font-family: UICTFontTextStyleEmphasizedItalicBody; font-size: 18.36px; font-style: italic; font-weight: bold;">PRNP</span><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Justin J. Greenleea, Eric D. Cassmanna, S. Jo Moorea,b, and M. Heather West Greenleec</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">aVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA; bOak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, US; cDepartment of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, US</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Aims</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent </span><span class="ydpa31d830yiv3263669459s4" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">PRNP</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure.</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Material and Methods</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">: Wild type (EE211) or heterozygous (<span dir="ltr">EK211</span>) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n = 3) or from the US 2006 case with the E211K polymorphism (n = 4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation.</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Results</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">: Three-out-of-four (75%) calves with the <span dir="ltr">EK211</span> genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive <span dir="ltr">EK211</span>steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57–4.0) in the brainstem, and IHC demonstrated PrPScthroughout the brain. All wild type recipient cattle and a single <span dir="ltr">EK211</span> steer remained asymptomatic for the duration of the experiment (approximately 7 years post-inoculation) and no abnormal prion protein was detected in these cattle by EIA.</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Conclusions</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">: This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the <span dir="ltr">EK211</span> genotype are oronasally susceptible to small doses of the H-BSE agent from either <span dir="ltr">EK211</span> or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1 g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Funded by</span><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">: US Department of Agriculture</span></p><p class="ydpa31d830yiv3263669459p2" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><span class="ydpa31d830yiv3263669459s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9467582/" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9467582/</a></span></p><p class="ydpa31d830yiv3263669459p1" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><br /></p><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Presentation Type: Oral Presentation</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Grant Number: ALMA/APRI: 201400006, HC 414250</div></div></div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div data-setdir="false" dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><b style="font-family: Helvetica, Arial, sans-serif; margin-top: 0px;">Research Project: </b><span face="Helvetica, Arial, sans-serif"></span><a href="https://www.ars.usda.gov/research/project/?accnNo=440677" rel="noreferrer noopener" style="color: #4c2c92; font-family: Helvetica, Arial, sans-serif;" target="_blank">Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies </a><span face="Helvetica, Arial, sans-serif"></span><p style="font-family: Helvetica, Arial, sans-serif; line-height: 1.5; margin: 1em 0px; max-width: none;"><b>Location: <a href="https://www.ars.usda.gov/midwest-area/ames/nadc/virus-and-prion-research/" rel="noreferrer noopener" style="color: #4c2c92;" target="_blank">Virus and Prion Research</a></b></p><b style="font-family: Helvetica, Arial, sans-serif;">Title:</b><span face="Helvetica, Arial, sans-serif"> Disease phenotype of classical sheep scrapie is changed upon experimental passage through white-tailed deer </span><br style="font-family: Helvetica, Arial, sans-serif;" /><b style="font-family: Helvetica, Arial, sans-serif;">Author</b><br style="font-family: Helvetica, Arial, sans-serif;" /><div align="left" style="font-family: Helvetica, Arial, sans-serif;"><table cellpadding="3" cellspacing="0" style="border-collapse: collapse; margin: 0px; min-width: 100%;"><tbody><tr><td valign="top" width="20px"><img _mf_state="1" alt="item" border="0" data-inlineimagemanipulating="true" id="96UUTJAPMSKaIarPetEr" src="blob:https://www.blogger.com/8ce62ef9-a2d4-498b-a2c6-89378687defd" style="border: 0px; vertical-align: middle;" title="null" webkitattachmentid="60558063-0277-4441-95dd-76bfbe7beaac" /></td><td align="left" background="https://www.ars.usda.gov/ARSUserFiles/incme/images/spacer.gif" valign="top">Kokemuller, Robyn</td></tr><tr><td valign="top" width="20px"><img _mf_state="1" alt="item" border="0" data-inlineimagemanipulating="true" id="AmkAXzC1ZW8omerr0wZa" src="blob:https://www.blogger.com/307340c2-7cfd-47d1-b173-22ca48ce9bd0" style="border: 0px; vertical-align: middle;" title="null" webkitattachmentid="fcdd5e97-32db-4e9c-a1b1-e312d442ef94" /></td><td align="left" background="https://www.ars.usda.gov/ARSUserFiles/incme/images/spacer.gif" valign="top">MOORE, S.JO - Oak Ridge Institute For Science And Education (ORISE)</td></tr><tr><td valign="top" width="20px"><img _mf_state="1" alt="item" border="0" data-inlineimagemanipulating="true" id="LUGHmoTWh3geQUGGHQkI" src="blob:https://www.blogger.com/04669fef-4681-4edd-955c-a324e59ffc01" style="border: 0px; vertical-align: middle;" title="null" webkitattachmentid="a3d9a084-c267-4b54-a6d8-db83e8f3d182" /></td><td align="left" background="https://www.ars.usda.gov/ARSUserFiles/incme/images/spacer.gif" valign="top"><a href="https://www.ars.usda.gov/people-locations/person?person-id=57305" id="ydp5847dd67yiv4365128736anch_57" rel="noreferrer noopener" style="color: #4c2c92;" target="_blank">Bian, Jifeng</a></td></tr><tr><td valign="top" width="20px"><img _mf_state="1" alt="item" border="0" data-inlineimagemanipulating="true" id="CNn5OxQ30RzpKRIpVWBO" src="blob:https://www.blogger.com/638a71c7-f645-458a-bdac-3bfcdfb43d4f" style="border: 0px; vertical-align: middle;" title="null" webkitattachmentid="88cc6371-eafb-4359-bc9e-b9277f36e024" /></td><td align="left" background="https://www.ars.usda.gov/ARSUserFiles/incme/images/spacer.gif" valign="top">WEST GREENLEE, HEATHER - Iowa State University</td></tr><tr><td valign="top" width="20px"><img _mf_state="1" alt="item" border="0" data-inlineimagemanipulating="true" id="MvsTV6ngkURkdFBhCGKR" src="blob:https://www.blogger.com/fa451daa-1ec0-4cec-a1e5-5aa0fb5aa213" style="border: 0px; vertical-align: middle;" title="null" webkitattachmentid="7caba040-2638-4c75-bda0-0600ac0a6fe0" /></td><td align="left" background="https://www.ars.usda.gov/ARSUserFiles/incme/images/spacer.gif" valign="top"><a href="https://www.ars.usda.gov/people-locations/person?person-id=44813" id="ydp5847dd67yiv4365128736anch_57" rel="noreferrer noopener" style="color: #4c2c92;" target="_blank">Greenlee, Justin</a></td></tr></tbody></table></div><table border="0" cellpadding="0" cellspacing="2" style="border-collapse: collapse; color: #333333; font-family: Helvetica, Arial, sans-serif; font-size: 17px; margin: 0px; min-width: 100%; width: 100%;"><tbody><tr><td valign="top"><div align="left"><b>Submitted to:</b> PLoS Pathogens<br /><b>Publication Type:</b> Peer Reviewed Journal<br /><b>Publication Acceptance Date:</b> 11/9/2023<br /><b>Publication Date:</b> 12/4/2023<br /><b>Citation:</b> Kokemuller, R., Moore, S., Bian, J., West Greenlee, H.M., Greenlee, J.J. 2023. Disease phenotype of classical sheep scrapie is changed upon experimental passage through white-tailed deer. PLoS Pathogens. https://doi.org/10.1371/journal.ppat.1011815.<br /><b>DOI:</b> <a href="https://doi.org/10.1371/journal.ppat.1011815" rel="noreferrer noopener" style="color: #4c2c92;" target="_blank">https://doi.org/10.1371/journal.ppat.1011815</a><p style="line-height: 1.5; margin: 1em 0px; max-width: none;"><b>Interpretive Summary:</b> Transmissible spongiform encephalopathies (TSEs) are a group of fatal diseases caused by the accumulation of misfolded prion protein in the brain. Ruminant species such as sheep, deer, and elk can get prion diseases. In sheep the disease is called scrapie. In deer and elk, the disease is called chronic wasting disease (CWD). The source of CWD is unknown, but one possibility is that scrapie jumped from sheep to deer. When we experimentally exposed white-tailed deer to the sheep scrapie agent, all deer developed scrapie. The purpose of the current experiment was to determine if sheep can get scrapie derived from white-tailed deer. Some sheep developed scrapie after oronasal exposure to the scrapie agent from white-tailed deer. Passage through white-tailed deer results in a scrapie isolate with different strain properties than the original inoculum. The detection of new strain properties was an unexpected result that will be the subject of further studies. These results indicate that sheep could be susceptible to the scrapie agent after passage through deer if exposed to the agent in natural or agricultural settings, which could be a confounding factor to the scrapie eradication program. National and state regulatory and wildlife officials should consider this information when developing plans to reduce or eliminate TSEs.</p><div style="line-height: 1.5; margin: 1em 0px; max-width: none;"><b>Technical Abstract:</b> Transmissible spongiform encephalopathy (TSE) agents have strain variations that influence disease phenotype and may affect the potential for interspecies transmission. Since deer and sheep may use the same grazing land, it is important to understand the potential transmission of TSEs between these species. The US scrapie isolate (No.13-7) had a 100% attack rate in white-tailed deer after oronasal challenge. The purpose of this study was to determine if sheep are susceptible to oronasal challenge with the scrapie agent from white-tailed deer. Suffolk lambs of various prion protein genotypes were challenged by the oronasal route with a 10% brain homogenate from scrapie-affected white-tailed deer. Sheep were euthanized and necropsied upon development of clinical signs or at the end of the experiment (72 months post-inoculation). Tissues were tested for PrPSc by enzyme immunoassay, western blot, and immunohistochemistry. The first sheep (2/2) to develop clinical signs at approximately 29 months post-inoculation (MPI) had the VRQ/VRQ genotype. One of the two sheep with the ARQ/ARQ genotype also developed clinical signs at 48 MPI. This is in contrast to the original No.13-7 inoculum that has a faster incubation period in sheep with the ARQ/ARQ genotype compared to sheep of the VRQ/VRQ genotype. The shorter incubation period in VRQ/VRQ sheep than ARQ/ARQ sheep after passage through deer indicates a phenotype change. This is important because scrapie infected deer could transmit disease to sheep resulting in new scrapie strain properties. This work raises the concern that scrapie infected deer could serve as a confounding factor to scrapie eradication programs as the scrapie agent from deer is transmissible to sheep by the oronasal route.</div><div style="line-height: 1.5; margin: 1em 0px; max-width: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=405929" rel="noreferrer noopener" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=405929</a><br /></div><p style="line-height: 1.5; margin: 1em 0px; max-width: none;">“This is important because scrapie infected deer could transmit disease to sheep resulting in new scrapie strain properties. This work raises the concern that scrapie infected deer could serve as a confounding factor to scrapie eradication programs as the scrapie agent from deer is transmissible to sheep by the oronasal route.”</p></div></td></tr></tbody></table><div><div>***> Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div><br /></div><div>Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div><br /></div><div>Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA</div><div><br /></div><div>Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.</div><div><br /></div><div>Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).</div><div><br /></div><div>Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.</div><div><br /></div><div>Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.</div><div><br /></div><div>Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div><br /></div><div>"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div><br /></div><div>=====end</div><div><br /></div><div>Strain characterization of chronic wasting disease in bovine-PrP transgenic mice</div><div><br /></div><div>Nuria Jerez-Garrido1, Sara Canoyra1, Natalia Fernández-Borges1, Alba Marín Moreno1, Sylvie L. Benestad2, Olivier Andreoletti3, Gordon Mitchell4, Aru Balachandran4, Juan María Torres1 and Juan Carlos Espinosa1. 1 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain. 2 Norwegian Veterinary Institute, Ås, Norway. 3 UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France. 4 Canadian Food Inspection Agency, Ottawa, Canada.</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) is an infectious prion disease that affects cervids. Various CWD prion strains have been identified in different cervid species from North America and Europe. The properties of the infectious prion strains are influenced by amino acid changes and polymorphisms in the PrP sequences of different cervid species. This study, aimed to assess the ability of a panel of CWD prion isolates from diverse cervid species from North America and Europe to infect bovine species, as well as to investigate the properties of the prion strains following the adaptation to the bovine-PrP context.</div><div><br /></div><div>Materials and Methods: BoPrP-Tg110 mice overexpressing the bovine-PrP sequence were inoculated by intracranial route with a panel of CWD prion isolates from both North America (two white-tailed deer and two elk) and Europe (one reindeer, one moose and one red deer).</div><div><br /></div><div>Results: Our results show distinct behaviours in the transmission of the CWD isolates to the BoPrP-Tg110 mouse model. Some of these isolates did not transmit even after the second passage. Those able to transmit displayed differences in terms of attack rate, survival times, biochemical properties of brain PrPres, and histopathology.</div><div><br /></div><div>Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.</div><div><br /></div><div>Funded by: MCIN/AEI /10.13039/501100011033 and by European Union NextGeneration EU/PRTR</div><div><br /></div><div>Grant number: PCI2020-120680-2 ICRAD</div><div><br /></div><div>"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div><br /></div><div>=====end</div><div><br /></div><div><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="noreferrer noopener" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div><br /></div><div>Ruminant feed ban for cervids in the United States ?</div><div><br /></div><div>Posted by flounder on 31 Jan 2015 at 20:14 GMT</div><div><br /></div><div>Friday, December 14, 2012</div><div><br /></div><div>DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012</div><div><br /></div><div>snip...</div><div><br /></div><div>In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.</div><div><br /></div><div>Animals considered at high risk for CWD include:</div><div><br /></div><div>1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and</div><div><br /></div><div>2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.</div><div><br /></div><div>Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.</div><div><br /></div><div>The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.</div><div><br /></div><div>Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.</div><div><br /></div><div>There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.</div><div><br /></div><div>snip...</div><div><br /></div><div>36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011). The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.</div><div><br /></div><div>snip...</div><div><br /></div><div>The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).</div><div><br /></div><div>snip...</div><div><br /></div><div>In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.</div><div><br /></div><div>snip...</div><div><br /></div><div>In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.</div><div><br /></div><div>snip...</div><div><br /></div><div>Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.</div><div><br /></div><div>snip...</div><div><br /></div><div><a href="https://web.archive.org/web/20170404125557/http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf" rel="noreferrer noopener" target="_blank">https://web.archive.org/web/20170404125557/http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf</a></div><div><br /></div><div>Friday, December 14, 2012</div><div><br /></div><div>DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012</div><div><br /></div><div><a href="https://web.archive.org/web/20160128180140/http://chronic-wasting-disease.blogspot.com/2012/12/defra-uk-what-is-risk-of-chronic.html" rel="noreferrer noopener" target="_blank">https://web.archive.org/web/20160128180140/http://chronic-wasting-disease.blogspot.com/2012/12/defra-uk-what-is-risk-of-chronic.html</a></div><div><br /></div><div>*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.</div><div><br /></div><div><a href="http://web.archive.org/web/20121022162853/http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf" rel="noreferrer noopener" target="_blank">http://web.archive.org/web/20121022162853/http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf</a></div><div><br /></div><div>OIE Conclusions on transmissibility of atypical BSE among cattle</div></div></div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">34 Scientific Commission/September 2019</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">3. Atypical BSE</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a></div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion</div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a></div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a></div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">PMID: 21266763</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Abstract</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a></div><div dir="ltr" style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">see full text;</div><div dir="ltr" style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a></div></div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a></div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 16px; line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin </div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019. </div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues. </div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: <a href="https://doi.org/10.3389/fvets.2019.00430" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://doi.org/10.3389/fvets.2019.00430</a> </div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305</a></div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665</a></div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668</a></div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;">Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.</div><div style="line-height: 1.6em; margin: 0px 0px 0.75em; outline-color: currentcolor; outline-style: initial;"><a href="https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0" rel="noreferrer noopener" style="color: #196ad4; font-weight: bold; outline-color: currentcolor; outline-style: initial;" target="_blank">https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0</a></div></div><div data-setdir="false" dir="ltr" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><div style="font-family: arial; font-size: 16px;"><div dir="ltr"><div dir="ltr"><div>Friday, May 12, 2023</div><div><br /></div><div>Camel prion disease, a new emerging disease in North Africa, Lymphoid Tropism, Neuropathological Characterization Update 2023</div><div><br /></div><div>11th Iberian Congress on Prions Barcelona 2023</div><div><br /></div><div><a href="http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">http://prioncongressbcn.ppmclab.com/book_abstracts_v4.pdf</a></div><div><br /></div><div><a href="https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://camelusprp.blogspot.com/2023/05/camel-prion-disease-new-emerging.html</a><br /></div><div><br /></div></div><div>A Camelid Anti-PrP Antibody Abrogates PrPSc Replication in Prion-Permissive Neuroblastoma Cell Lines</div><div><br /></div><div>Daryl Rhys Jones,William Alexander Taylor,Clive Bate,Monique David,Mourad Tayebi </div><div><br /></div><div>Published: March 22, 2010</div><div><br /></div><div><a href="https://doi.org/10.1371/journal.pone.0009804" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://doi.org/10.1371/journal.pone.0009804</a><br /></div><div><br /></div><div dir="ltr"><a href="https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0009804</a><br /></div></div><div><br /></div><div>15 Apr 2018 23:13 GMT MOST RECENT </div><div><br /></div><div>Prion Disease in Dromedary Camels, Algeria </div><div><br /></div><div>Posted by flounder on 15 Apr 2018 at 23:13 GMT</div><div><br /></div><div><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/fea97a95-2600-42a6-b289-0f490896a3aa</a></div><div><br /></div><div><a href="https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0009804</a><br /></div></div><div style="font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="font-family: arial; font-size: 16px;"><a href="https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/6/17-2007_article</a></div></div><div style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;"><br /></div><div data-setdir="false" dir="ltr" style="color: black; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px;">WOAH OIE BSE 2022<div><br /></div><div><a href="https://www.woah.org/app/uploads/2022/04/a-scad-feb2022-2.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.woah.org/app/uploads/2022/04/a-scad-feb2022-2.pdf</a><br id="ydpa31d830yiv3263669459lineBreakAtBeginningOfSignature" /><div dir="ltr"><br /></div><div dir="ltr">WOAH OIE ATYPICAL BSE 2022</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://www.woah.org/app/uploads/2023/04/a-qanda-bse-2023.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.woah.org/app/uploads/2023/04/a-qanda-bse-2023.pdf</a></div><div dir="ltr"><br /></div><div data-setdir="false" dir="ltr">Deregulation atypical BSE</div><div dir="ltr"><br /></div><div dir="ltr"><a href="https://www.woah.org/app/uploads/2023/05/a-r20-2023-procedures-update.pdf" rel="noreferrer noopener" style="color: #196ad4;" target="_blank">https://www.woah.org/app/uploads/2023/05/a-r20-2023-procedures-update.pdf</a></div><div dir="ltr"><br /></div><div dir="ltr"><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 16px; outline-color: currentcolor; outline-style: initial;">YOU CAN SEE THE MARKED UP BSe HERE;</div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 16px; outline-color: currentcolor; outline-style: initial;"><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 16px; outline-color: currentcolor; outline-style: initial;"><a href="https://www.woah.org/app/uploads/2023/05/a-90sg-10cs1-terrestrial-animal-health-standards-commission.pdf" rel="noreferrer noopener" style="color: #196ad4; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.woah.org/app/uploads/2023/05/a-90sg-10cs1-terrestrial-animal-health-standards-commission.pdf</a><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 16px; outline-color: currentcolor; outline-style: initial;"><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 16px; outline-color: currentcolor; outline-style: initial;"><div style="outline-color: currentcolor; outline-style: initial;"><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;">World Organization for Animal Health 90th General Session of the World Assembly of Delegates (BSE TSE Prion) From 21/05/2023 to 25/05/2023 Singeltary Concerns...<br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;"><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;">***> THIS PRETTY MUCH SAYS IT ALL!</div><div style="font-family: arial; outline-color: currentcolor; outline-style: initial;"><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;"><div style="outline-color: currentcolor; outline-style: initial;"><div style="outline-color: currentcolor; outline-style: initial;">Q9. What should my country do if an atypical BSE case is detected on the day after the new BSE standards are adopted?</div><div style="outline-color: currentcolor; outline-style: initial;"><br style="outline-color: currentcolor; outline-style: initial;" /></div><div style="outline-color: currentcolor; outline-style: initial;">A. The notification to WOAH of the occurrence of BSE cases would be limited to classical BSE. The information on atypical BSE cases should be provided as part of the annual reconfirmation (and when submitting a dossier for the official recognition of a BSE risk status) in substantiating the effectiveness of the BSE surveillance system. </div></div><br style="outline-color: currentcolor; outline-style: initial;" /></div><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;"><a href="https://www.woah.org/app/uploads/2023/04/a-qanda-bse-2023.pdf" rel="noreferrer noopener" style="color: #196ad4; outline-color: currentcolor; outline-style: initial;" target="_blank">https://www.woah.org/app/uploads/2023/04/a-qanda-bse-2023.pdf</a></div><div dir="ltr" style="font-family: arial; outline-color: currentcolor; outline-style: initial;"><br /></div></div></div></div></div></div><p style="margin-bottom: 24px; margin-top: 0px;">CHAPTER 11.4.</p><p style="margin-bottom: 24px; margin-top: 0px;">BOVINE SPONGIFORM ENCEPHALOPATHY</p><p style="margin-bottom: 24px; margin-top: 0px;">Article 11.4.1.</p><p style="margin-bottom: 24px; margin-top: 0px;">General provisions and safe commodities</p><div style="margin-bottom: 24px; margin-top: 0px;">The recommendations in this chapter are intended to manage the human and animal health risks associated with the presence of the bovine spongiform encephalopathy (BSE) agent in cattle (Bos taurus and B. indicus) only. For the purposes of official BSE risk status recognition, BSE excludes 'atypical BSE' as a condition believed to occur spontaneously in all cattle populations at a very low rate.</div><div style="margin-bottom: 24px; margin-top: 0px;"><a href="https://www.woah.org/fileadmin/Home/eng/Health_standards/tahc/2018/en_chapitre_bse.htm" rel="noreferrer noopener" target="_blank">https://www.woah.org/fileadmin/Home/eng/Health_standards/tahc/2018/en_chapitre_bse.htm</a><br /></div><p style="margin-bottom: 24px; margin-top: 0px;"><span style="color: black; font-family: arial; font-size: 16px;">Terry S. Singeltary Sr.</span></p></div></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-66004422617635501352024-01-31T11:22:00.003-06:002024-01-31T11:22:50.760-06:00Creutzfeldt Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's And 2024 Alzheimer’s iatrogenic Transmission <p>Creutzfeldt Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's And 2024 Alzheimer’s iatrogenic Transmission </p><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">SENATE COMMUNITY AFFAIRS REFERENCES COMMITTEE</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">INQUIRY INTO CHILDREN IN INSTITUTIONAL CARE</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><table cellpadding="0" cellspacing="0" class="t1" style="-webkit-text-size-adjust: auto; border-collapse: collapse; color: black; font-variant-caps: normal;"><tbody><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Organizations Name making submission</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Shortkids Downunder</span></p></td></tr><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Address</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">P.O. Box 250 KINGLAKE VIC. 3763</span></p></td></tr><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Telephone</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;"><a href="tel:(03)%205786%201938">(03) 5786 1938</a></span></p></td></tr><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Author</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Email</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Mr. Michael O’Meara </span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">(Chairperson)</span></p><p class="p4" style="color: #e4af0a; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">shortkids_1999@yahoo.com</span></p></td></tr><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Date Submitted</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Method (Email)</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p4" style="color: #e4af0a; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">community.affairs.sen@aph.gov.au</span></p></td></tr><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Is this Submission available for public comment?</span></p></td><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Yes</span></p></td></tr></tbody></table><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Note; </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Information contained in this report refers to The “Allars Report” (Senate Inquiry, June 1994) in which does not make a decision of a Criminal nature that is referred to in this submission. However this submission does refer to questionable “Criminal Activity” as disclosed in the “Allars Report” namely;</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">“We believe that the report completed by Professor Allars, her executive summaries and Dr Lawrence's speech to the House prove beyond any question that there had been significant breaches of the law as it applies to a wide variety of areas. These include the manufacture of the hormone, including the collection of glands, distribution of the hormone by various agencies, including HPAC, CSL and others as detailed in the Allars report, and non-disclosure of possible side effects.” </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;"><br />“Neither compensation nor the issue of criminal or civil liability of medical practitioners were part of my terms of reference. Consequently I made no recommendation on these issues. I make no comment now. (Submission No.92, p.4. Allars Report)”</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">“…harvesting the pituitary glands under completely uncontrolled conditions must border on criminal negligence - has ever been held to account. Nor have any members of HPAC, who sought kudos and career advancement ahead of prudent medical practice and patient safety.”</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;"> </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">1 Aims of this submission</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">It has been reported back in 1997, that “experimental” drugs were administered to Clients in Orphanages by medical practitioner (s) in conjunction with CSL who at the same period were producing/administering Pituitary derived Human Growth Hormone (hGH) with the Medical Practitioners administering (toxic drugs) to clients (St Joseph’s Orphanage, Broadmeadows) who were legislated (under the Health Act) members of Human Pituitary Advisory Committee (H.P.A.C.)</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">To investigate the connection with CSL, Walter and Eliza Institute, Department of Health and Family Services, and Medical Practitioners practicing in Orphanages who were also practicing privately using hGH and Anabolic Steroids.</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">To submit documentation pertaining to “sterilization” procedures upon the clients of Orphanages as reports in Red Deer (Canadian) Orphanage and other Institutions, using Anabolic Steroids and anti-psychotic drugs, which appear to be the guidelines of the American Eugenics Society & with CSL </span><span class="s6" style="font-family: UICTFontTextStyleEmphasizedItalicBody; font-size: 18.36px; font-style: italic; font-weight: bold;">et al </span><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">who report to follow the American standards.</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">To investigate “Clinical Trials” and procedures upon the clients of Orphanages as reported at St Joseph’s Orphanage, Broadmeadows, by doctors of HPAC. </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">To investigate “Clinical Trials”, “The Testes, Clinical and Experimental Studies” and procedures upon the clients of Institutions as reported at Kingston Centre, Kingston, by doctors of HPAC. </span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">2 Overview of Submission</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">An investigation by the Melbourne Age newspaper revealed that hundreds of children in orphanages and babies' homes, including wards of state, were used in the experiments and studies over 25 years, with investigation showing that medical practitioners of the Australian Human Pituitary Hormone Programme experimenting on Humans institutionalized.</span><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"> </span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">These “human guinea pigs” , living and/or deceased, carry a wide range of side effects from this experimentation from clinical trials (without consent) and could be unknowingly incubating Creutzfeldt-Jacob Disease. (CJD)</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">An investigation by the “Shortkids Downunder” revealed that hundreds of children in orphanages and babies' homes, together with a Melbourne Geriatric Institution were subjected to experimental trials by treating doctors of HPAC using Gonadrophins and Anabolic Steroids.</span></p><p class="p5" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 0px 36px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">3 Scope of the Submission</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Title;</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Orphans experimented on without consent. Alberta sterilization victims also used as guinea pigs.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">As many as 100 of the children at the centre of the Alberta sterilization scandal of the late 1960s and early 1970s were also used as guinea pigs in drug trials, the National Post has learned. Parents, who did not consent to the sterilization or medical experimentation, which included the administration of powerful steroids and anti-psychotic drugs. Its effect on children is said to be akin to hitting them over the head with a sledge hammer.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="http://mc2.vicnet.net.au/home/shortboys/web/guineepigs.html">http://mc2.vicnet.net.au/ho</a>me/shortboys/web/guineepigs.html</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"> </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Title;</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Orphan fears she was used as a drug guinea pig. More links to CSL using kids as drug guinea pigs. The Age newspaper in Melbourne has revealed that the experiments on children at some babies' homes and orphanages included trials of vaccines that did not work, or failed to pass safety tests on animals. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Broadmeadows Babies' Home, one of the orphanages used by scientists for medical experiments up until 1970. The cluster of red-brick buildings on the site of the former babies' home are now part of Penola Co-Ed Catholic College's Broadmeadows Campus. But seeing them yesterday made Mrs Di-Federico angry. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="http://mc2.vicnet.net.au/home/shortboys/web/Difederico.html">http://mc2.vicnet.net.au/home/shortboys</a>/web/Difederico.html</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Title;</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Orphans experimented on without consent. The Age who gave consent for the use of the infants. CSL's company secretary, Mr Peter Toohy, said in a statement to The Age: CSL Limited, an independent public company, could not comment on clinical trial protocols of the era when the then laboratories were an arm of the Commonwealth Department of Health.'' </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">An investigation by the Melbourne Age newspaper revealed that hundreds of children in orphanages and babies' homes, including wards of state, were used in the experiments and studies over 25 years. They were used to test vaccines and antigens for toxic effects before the new products were used on children in the wider community.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="http://mc2.vicnet.net.au/home/shortboys/web/broadmeadows.html">http://mc2.vicnet.net.au/home/shortboys/w</a>eb/broadmeadows.html</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Title;</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Link between St Joseph's Broadmeadows (Orphanage), Kingston Centre, Kingston, CSL, hGH, Anabolic Steroids, Short Statured Boys and Tall Girls administered by treating Endocrinologists and Pediatricians of HPAC</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s7" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">The writer of this submission was admitted to St Joseph's Babies Home in Broadmeadows in 1961 for relinquishment. It was the same year Wettenahall (a member of HPAC) was experimenting on babies with CSL vaccines. In 1972, Wettenhall </span><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">et al</span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">, Burger </span><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">et al</span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"> and de Krester </span><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">et al </span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">were experimenting on boys, girls, men and women using toxic drugs such as hGH, HPG produced by CSL and anabolic steroids to produce the male contraceptive pill. As members of HPAC both doctors experimented in two institutions in Melbourne under grants by the National Health and Medical Research Council. Wettenhall </span><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">et al </span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">and Burger </span><span class="s5" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic;">et al </span><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">were both members of HPAC and were known to be experimenting on kids as young as 2 years of age.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Members of HPAC treated children with hGH, Anabolic steroids, Diethylstilbestrol (DES) as private and Public patients without consent. CSL supplied drugs to members of HPAC with the approval of the National Biological Standards Laboratory a.k.a. T.G.A.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Shortkids Downunder <a href="http://mc2.vicnet.net.au/home/shortboys/web/index.html"><span class="s7" style="font-size: 18.36px;">http://mc2.vicnet.net.au/home/shortboys/web/index.html</span></a></span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><a href="https://web.archive.org/web/20031111062656/http://mc2.vicnet.net.au/home/shortboys/web/index.html">https://web.archive.org/web/20031111062656/http://mc2.vicnet.net.au/home/shortboys/web/index.html</a></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Tall Girls Inc. <a href="http://www.users.bigpond.com/jadetg/"><span class="s7" style="font-size: 18.36px;">http://www.users.bigpond.com/jadetg/</span></a></span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><a href="https://web.archive.org/web/20090223191619/http://www.users.bigpond.com/jadetg/">https://web.archive.org/web/20090223191619/http://www.users.bigpond.com/jadetg/</a></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s8" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; text-decoration: underline;"><a href="https://www.aph.gov.au/~/media/wopapub/senate/committee/clac_ctte/completed_inquiries/2004_07/inst_care/submissions/sub121_doc.ashx">https://www.aph.gov.au/~/media/wopapub/senate/committee/clac_ctte/completed_inquiries/2004_07/inst_care/submissions/sub121_doc.ashx</a></span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p6" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 30.2px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px 0px 3px;"><span class="s9" style="font-family: UICTFontTextStyleBody; font-size: 30.24px; font-weight: bold;">Creutzfeldt-Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Michael <a href="https://web.archive.org/web/20040910222725/mailto:shortkids_1999@yahoo.com"><span class="s7" style="font-size: 18.36px;">shortkids_1999@yahoo.com</span></a></span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p7" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><img alt="unknown.jpg" src="blob:https://www.blogger.com/ae27dacf-446a-4282-bb67-26c38de7b410" webkitattachmentid="6bbdb1da-823c-4159-92fd-daef3bf6e199" /></span></p><table cellpadding="0" cellspacing="0" class="t1" style="-webkit-text-size-adjust: auto; border-collapse: collapse; color: black; font-variant-caps: normal;"><tbody><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p8" style="font-family: Helvetica; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 12px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 13.8px;"><span class="s10"></span><br /></p></td></tr></tbody></table><p class="p7" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><img alt="unknown_1.jpg" src="blob:https://www.blogger.com/9233e303-fa9f-4065-9cf8-01010a15a6ad" webkitattachmentid="446d9c94-054c-45d9-8f63-4d3d8c371c44" /></span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Two thousand one hundred Australians were treated with human pituitary hormones under the Australian Human Pituitary Hormone Program (AHPHP) which ran in Australia from 1967 until 1985.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">No-one from CSL - whose actions in harvesting the pituitary glands under completely uncontrolled conditions must border on criminal negligence - has ever been held to account. Nor have any members of HPAC, who sought kudos and career advancement ahead of prudent medical practice and patient safety.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">De Kretser (Andrologist) and Burger (Endocrinologist) both of Prince Henry's Hospital,and Hudson of Melbourne University, leading a United Nations' task force which could give the world a male birth control pill. </span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Cryptorchidism, ectopic testes caused through steroid priming was another way to sterilize boys with the use of anabolic steroids.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Submission No.92, p.4.in the Allars Report states;</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Neither compensation nor the issue of criminal or civil liability of medical practitioners were part of my terms of reference. Consequently I made no recommendation on these issues. I make no comment now.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><table cellpadding="0" cellspacing="0" class="t1" style="-webkit-text-size-adjust: auto; border-collapse: collapse; color: black; font-variant-caps: normal;"><tbody><tr><td class="td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://www.cjdsupport.org.au/">Australian CJD Support Group</a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">The Inquiry also found that HPAC had seriously breached both Federal and State laws, it breached its own guidelines and engaged in human experimentation. The report was damning. Commentators described the program as the worst case of medical negligence in Australia's history. It was not in the Inquiry's terms of reference to draw conclusions regarding issues of professional or criminal misconduct and unlawful negligence</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://members.aol.com/larmstr853/cjdvoice/index.htm">Link - CJD Voice (U.S.A.)</a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">We are a grass roots organization of individuals formed in May 1997 to support families during and after a CJD crisis. We also discuss various issues surrounding CJD and other related illnesses. As of October 1999, we have over 400 members.</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://www.health.gov.au/pubhlth/strateg/phi/">THE USE OF HUMAN PITUITARY HORMONES IN AUSTRALIA AND CREUTZFELDT-JAKOB DISEASE (CJD)</a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Creutzfeldt-Jakob Disease (CJD) is a rare disease of the central nervous system, which results in death after a relatively rapid course of muscle weakness and dementia. There is no definitive diagnostic test and no known cure</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://www.aph.gov.au/senate/committee/clac_ctte/cjd/report/c07.htm">Unapproved recipients of hGH</a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">The Allars Inquiry found that there were a group of recipients of hormones including those who received hormones who were not approved to do so by HPAC or its Subcommittees. These include individuals who received hormones before the official commencement of the AHPHP, those who received hormone produced by other groups (VPG and Dr Brown), those who only received a simulation test, those who were treated for cervical mucus problems, those treated with research allocation of hormones for example, the Egg Project and IVF Program and those who were treated with left over' hormones by a treating practitioner.</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://pub35.bravenet.com/chat/show.php/2949257574">CJD Voice's Chat Room </a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">CJD Voice's Chat Room from the U.S.A. Welcome to our Live Chat Room! Chat Schedule </span><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">7-8pm Wed</span><span class="s3" style="font-family: UICTFontTextStyleBody;">(Australian EST) </span><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">12pm-1pm Friday</span><span class="s3" style="font-family: UICTFontTextStyleBody;"> (Australian EST) </span><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;">1pm to 3pm Sunday</span><span class="s3" style="font-family: UICTFontTextStyleBody;"> (Australian EST) Stop by and visit! After the applet loads, click the button below to enter the Bravenet Chat Room Server. When the Chat window appears, enter a nickname for yourself where it says 'Your Name' and a short 'Profile' about yourself. Then click the button to 'CHAT!'. </span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/chapter7.html">...all of the agencies that were connected with the Australian human pituitary hormone program are at fault. </a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Many recipients believe however, that the Allars Inquiry did not adequately address the issue of accountability because the terms of reference did not go to the question of liability. ...all of the agencies that were connected with the Australian human pituitary hormone program are at fault. The revelations of the Allars inquiry are mind-boggling to say the least. The irony of all of this is that no one will ever be made accountable for their improper actions, actions that have left a number of people dead, others at risk and families devastated by a dreadful disease</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/humanrights.html">Both the Family Law Council and the Law Reform Commission of Western Australia have argued that the distinction between therapeutic and non-therapeutic sterilisations is too uncertain a test of which matters require court authorisatio</a>n</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">The legal framework regulating sterilisation of children in Australia was set out by the High Court in Marion's Case in 1992. It sought to ensure heightened accountability in decision making in an area where children are at significant risk of grave abuse of their fundamental human right to bodily integrity</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/mussclespazms.html">CJD proteins found outside brain - musscle tissue</a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">The good news is that the availability of highly sensitive techniques for the detection of pathological prions will make it possible to make a firm diagnosis of Creutzfeldt-Jakob disease without resorting to brain biopsy</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/38years.html">CJD can develop even after a low dose of human growth hormone. </a></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;">Dutch researchers report on the case of a man who developed CJD 38 years after receiving human derived growth hormone. A 47 year old man was given only a low dose as part of a diagnostic procedure, rather than being given full treatment, which may explain why the incubation period lasted so long -- the longest on record -- say the authors</span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"><br /></span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/index.html">Homepage</a></span></p><p class="p9" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 22px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"></span><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s4" style="font-family: UICTFontTextStyleBody; text-decoration: underline;"><a href="https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/cjdaustralia.html">https://web.archive.org/web/20040910222725/http://mc2.vicnet.net.au/home/shortboys/web/cjdaustralia.html</a></span></p><p class="p9" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 22px;"><span class="s3" style="font-family: UICTFontTextStyleBody;"></span><br /></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s12" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold;">Creutzfeldt-Jakob Disease. Australia's </span></p><p class="p3" style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s12" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold;">Michael <a href="https://web.archive.org/web/20040815054355/mailto:shortkids_1999@yahoo.com"><span class="s11">shortkids_1999@yahoo.com</span></a></span></p></td></tr></tbody></table></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><img alt="image" src="blob:https://www.blogger.com/c3ae7613-5a2c-4bbc-87c0-4dce7446af91" webkitattachmentid="33af3c40-28f1-467e-8d13-20eb014a2255" /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><table cellpadding="0" cellspacing="0" style="border-collapse: collapse;"><tbody><tr><td style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p style="font-family: Helvetica; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 12px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 13.8px;"><br /></p></td><td style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span style="font-weight: bold;">In Australia, from the late 50's, prepubertal and adolescent boys with a prediction of 'short stature' were treated with synthetic androgens to excelerate their growth. Originally Norethandrolone was used and then from the early 70's Oxandrolone. No thorough multidisciplinary, long-term follow-up has ever been done with regard to this treatment. Many of the Australian Men and some Women now report a range of medical problems which they fear may be the result of their treatment with androgens. There is however evidence about their concerns since the fact that Oxandrolone treatment was discontinued in 1990 and other Growth promotors such as Human Growth Hormone (hGH)has now ceased being manufactured from Pituiarty Glands in 1985.</span></p></td></tr></tbody></table><br /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><table cellpadding="0" cellspacing="0" style="border-collapse: collapse;"><tbody><tr><td style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span style="font-weight: bold;">Two thousand one hundred Australians were treated with human pituitary hormones under the Australian Human Pituitary Hormone Program (AHPHP) which ran in Australia from 1967 until 1985. No-one from CSL - whose actions in harvesting the pituitary glands under completely uncontrolled conditions must border on criminal negligence - has ever been held to account. Nor have any members of HPAC, who sought kudos and career advancement ahead of prudent medical practice and patient safety. Overall, 1 in about 300 people treated with hGH got CJD. All CJD patients received some hGH before 1977. Of those treated before 1977, 1 in 104 got CJD. In 1977, the NHPP changed the way it made hGH. Scientists added a new purification step that greatly reduced and may have removed the risk of CJD. So far, no patient who started hGH after 1977 has become ill with CJD. Since CJD takes so long to develop, we still don't know for sure that those who started treatment after 1977 are safe. The longest reported time from the start of hGH treatment to first signs of CJD is 33 years in U.S. patients. In Canada it is 38 years after diagnostic infusion of hGH to measure Growth Hormone</span></p></td></tr></tbody></table><br /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><img alt="unknown.gif" src="blob:https://www.blogger.com/737c8d69-d23f-4c2f-b625-e9eb66518c81" webkitattachmentid="39eec35b-8829-4ee7-ad86-299bb2e12e47" /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><img alt="image" src="blob:https://www.blogger.com/46068770-7d56-4c81-9fb9-a23355152765" webkitattachmentid="1881d135-1385-458e-9576-ad286359041d" /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">In Australia, from the late 50's, prepubertal and adolescent boys with a prediction of 'short stature' were treated with synthetic androgens to excelerate their growth. Originally Norethandrolone was used and then from the early 70's Oxandrolone.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">No thorough multidisciplinary, long-term follow-up has ever been done with regard to this treatment. Many of the Australian Men and some Women now report a range of medical problems which they fear may be the result of their treatment with androgens.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">There is however evidence about their concerns since the fact that Oxandrolone treatment was discontinued in 1990 and other Growth promotors such as Human Growth Hormone (hGH)has now ceased being manufactured from Pituiarty Glands in 1985.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">We believe that the report completed by Professor Allars, her executive summaries and Dr Lawrence's speech to the House prove beyond any question that there had been significant breaches of the law as it applies to a wide variety of areas. These include the manufacture of the hormone, including the collection of glands, distribution of the hormone by various agencies, including HPAC, CSL and others as detailed in the Allars report, and non-disclosure of possible side effects.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Neither compensation nor the issue of criminal or civil liability of medical practitioners were part of my terms of reference. Consequently I made no recommendation on these issues. I make no comment now. (Submission No.92, p.4.)</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">2.124 What effect does the knowledge that you have been placed at risk of death from a rare and fatal disease have - a disease for which there is currently no cure and no diagnosis of the possibility of its contraction? What effect do related issues have upon people such as the often accidental' manner by which a recipient learnt of the risk associated with their treatment, that the treatment was experimental or part of a clinical trial, that in some cases they should not have even been given the treatment for various reasons, that they were treated unofficially' by medical practitioners who had not obtained necessary departmental approvals, or the confusion and frustration arising from the inability to have accurate and comprehensive information provided at times when recipients were most vulnerable?</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><br /><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">2.126 The effects at a personal level have been described in many submissions and in evidence.[103] Some people have been effected to a greater degree than others. In an attempt to try and understand what many recipients and their families are going through, the following individual comments have been distilled from various submissions and describe a variety of effects. They are by no means comprehensive. One of the most unfortunate effects has been the problems created for some families. Family life has been disrupted and behavioural problems have occurred. A number of recipients made the following points in highlighting such problems. Individuals have withdrawn into themselves to the extent of living separate lives in their home environment. In other situations older children have been unable to cope with parental stress and anxiety and have left home; families have split up and marital breakdowns have occurred; and couples have decided not to have children to avoid any possibility of risk. In some instances reference was made to a sense of guilt which may develop within the child born through hormone treatment, while some parents who put their children through growth hormone treatment may also suffer a sense of guilt. The fear of CJD remains an issue which some people find difficulty in talking about within the family, and certainly with friends and workmates, thereby bottling up' stress and anxiety. Conversely, there are many instances where family problems have been alleviated through strong family support and commitment, or through counselling and other support measures. </span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/cjdaustralia.html">CJD Information, Links and articles. THE GOVERNMENT FAILED...All the people who have got CJD had been given hGH before 1977. .</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Two thousand one hundred Australians were treated with human pituitary hormones under the Australian Human Pituitary Hormone Program (AHPHP) which ran in Australia from 1967 until 1985. No-one from CSL - whose actions in harvesting the pituitary glands under completely uncontrolled conditions must border on criminal negligence - has ever been held to account. Nor have any members of HPAC, who sought kudos and career advancement ahead of prudent medical practice and patient safety. Overall, 1 in about 300 people treated with hGH got CJD. All CJD patients received some hGH before 1977. Of those treated before 1977, 1 in 104 got CJD. In 1977, the NHPP changed the way it made hGH. Scientists added a new purification step that greatly reduced and may have removed the risk of CJD. So far, no patient who started hGH after 1977 has become ill with CJD. Since CJD takes so long to develop, we still don't know for sure that those who started treatment after 1977 are safe. The longest reported time from the start of hGH treatment to first signs of CJD is 33 years in U.S. patients. In Canada it is 38 years after diagnostic infusion of hGH to measure Growth Hormone</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/guineepigs.html">CSL experiments on Melbourne Orphans without Consent. Alberta sterilization victims used as guinea pigs</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">CSL experiments on Melbourne Orphans without Consent.An investigation by the Melbourne Age newspaper revealed that hundreds of children in orphanages and babies' homes, including wards of state, were used in the experiments and studies over 25 years. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/recommendations.html">Senate Standing Committee of the 38th Parliament of Australia on Community Affairs, Current recommendations 30 May 2003</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Recommendation 15: That once it is established that a person did receive hPG or hGH from the AHPHP, the recipient's status should be of no difference to that of approved recipients. In the event of a dispute between the Department and a person who claims to have received human pituitary derived hormone, the matter should be referred to an independent arbitrator for resolution. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/cancer.html">Side Effects & Warnings regarding hGH and Anabolic Steroids. Includes Medical Documents, Deaths from Adrenal Crisis & Despite the limitations in their study the high incidence of cancer, and in particular of colon cancer, is worrying</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Side Effects & Warnings, icluding a follow up study overseas of hGH recipiants. Numerous recipiants have died from Adrenal Crisis. We believe that the report completed by Professor Allars, her executive summaries and Dr Lawrence's speech to the House prove beyond any question that there had been significant breaches of the law as it applies to a wide variety of areas. These include the manufacture of the hormone, including the collection of glands, distribution of the hormone by various agencies, including HPAC, CSL and others as detailed in the Allars report, and non-disclosure of possible side effects.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/records.html">Medical records</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">4.1 This term of reference primarily refers to the documents generated by and relating to the Allars Inquiry, which have been at the centre of contention between the Commonwealth and the recipients' legal advisers. An additional grouping of documents has been identified generally as other Departmental and CSL files and records, relevant to matters in issue in the legal proceedings. Within these two groups of documents are records and information held by the Department, and as well by doctors, hospitals and others which may be regarded as relevant to issues concerning individual recipients and their treatment, to which individual recipients have been seeking access. To obtain your Medical Records in regards to HPAC</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/legdoc1.html">Where you prescribed, without due care, anabolic steroids? A legal perspective </a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">You prescribed drugs of addiction for the treatment of seven patients for a period in excess of thirty days without written authorisation</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Personal Stories</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">A collection of Personal Stories regarding the growth hormone program. What happens to those experimented on.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/hgheffects.html">Human Growth Hormone Helps Small Kids Grow, But Some Call Its Wider Use Shortsighted</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">While there is evidence that HGH builds muscle mass and reduces fat in non-deficient adults, it can have side effects including edema, insulin resistance and joint pain. People who take it do so at their own risk and peril, says Pinchas Cohen, chief of pediatric endocrinology at the University of California, Los Angeles (UCLA). </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/buckbell.html">Three generation are enough. The Victorian Act of Parliament to Experiment on Humans</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Steroid Priming to sterilize children. Howard Florey Laboratories of Experimental Physiology devoted especially to the study of integrative physiology by long term experiments and these gentlemen and all other donors including the Commonwealth of Australia, the Reserve Bank of Australia and the Rockefeller Foundation made their gifts for that purpose and on these terms the Laboratories were commissioned in 1963:</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/minister.html">Questions On Notice to the Health Minister. </a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">After 10 years of asking questions of his treatment to his Endocrinologist and The Dept. of Health, and being told he wasnt at risk, FOI documents reveals the lies written by Drs and the Dept. of Health</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/index.html">https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/index.html</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; text-decoration: underline;"><a href="https://web.archive.org/web/20240000000000*/http://mc2.vicnet.net.au/home/shortboys/web/index.html">https://web.archive.org/web/20240000000000*/http://mc2.vicnet.net.au/home/shortboys/web/index.html</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><table cellpadding="0" cellspacing="0" style="border-collapse: collapse;"><tbody><tr><td style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p style="font-family: Helvetica; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 12px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 13.8px;"><br /></p></td><td style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; padding: 1px 5px;" valign="top"><p style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span style="font-weight: bold;">In the mid-1950s, scientists figured out how to extract human growth hormone from the dead, taking it from the pituitary glands of cadavers and injecting it into small children to make them grow. It was a rare and precious substance, doled out only to the neediest children, those whose bodies were deficient in the hormone. </span></p><p style="font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 17px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;">De Kretser (Andrologist) and Burger (Endocinologist) both of Prince Henry's Hospital, both sitting, Hudson of Melbourne University, standing; leading a United Nations' task force which could give the world a male birth control pill. Photo Jan. 1974 Herald and Weekly Times. The results of Androgens on boys left them sterile. They sought kudos and career advancement ahead of prudent medical practice and patient safety according to The Allars Report.</p></td></tr></tbody></table><div><br /></div><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold;">Short children and Tall Girls</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Short children and Tall Girls were treated with hGH, and sex steroids to grow taller and stunt growth</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">In Australia, from the late 50's, prepubertal and adolescent boys with a prediction of 'short stature' were treated with synthetic androgens to excelerate their growth. Originally Norethandrolone was used and then from the early 70's Oxandrolone.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">No thorough multidisciplinary, long-term follow-up has ever been done with regard to this treatment. Many of the Australian Men and some Women now report a range of medical problems which they fear may be the result of their treatment with androgens.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">There is however evidence about their concerns since the fact that Oxandrolone treatment was discontinued in 1990 and other Growth promotors such as Human Growth Hormone (hGH)has now ceased being manufactured from Pituiarty Glands in 1985.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">We believe that the report completed by Professor Allars, her executive summaries and Dr Lawrence's speech to the House prove beyond any question that there had been significant breaches of the law as it applies to a wide variety of areas. These include the manufacture of the hormone, including the collection of glands, distribution of the hormone by various agencies, including HPAC, CSL and others as detailed in the Allars report, and non-disclosure of possible side effects.</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Neither compensation nor the issue of criminal or civil liability of medical practitioners were part of my terms of reference. Consequently I made no recommendation on these issues. I make no comment now. (Submission No.92, p.4.)</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">2.124 What effect does the knowledge that you have been placed at risk of death from a rare and fatal disease have - a disease for which there is currently no cure and no diagnosis of the possibility of its contraction? What effect do related issues have upon people such as the often accidental' manner by which a recipient learnt of the risk associated with their treatment, that the treatment was experimental or part of a clinical trial, that in some cases they should not have even been given the treatment for various reasons, that they were treated unofficially' by medical practitioners who had not obtained necessary departmental approvals, or the confusion and frustration arising from the inability to have accurate and comprehensive information provided at times when recipients were most vulnerable?</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><br /><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">2.126 The effects at a personal level have been described in many submissions and in evidence.[103] Some people have been effected to a greater degree than others. In an attempt to try and understand what many recipients and their families are going through, the following individual comments have been distilled from various submissions and describe a variety of effects. They are by no means comprehensive. One of the most unfortunate effects has been the problems created for some families. Family life has been disrupted and behavioural problems have occurred. A number of recipients made the following points in highlighting such problems. Individuals have withdrawn into themselves to the extent of living separate lives in their home environment. In other situations older children have been unable to cope with parental stress and anxiety and have left home; families have split up and marital breakdowns have occurred; and couples have decided not to have children to avoid any possibility of risk. In some instances reference was made to a sense of guilt which may develop within the child born through hormone treatment, while some parents who put their children through growth hormone treatment may also suffer a sense of guilt. The fear of CJD remains an issue which some people find difficulty in talking about within the family, and certainly with friends and workmates, thereby bottling up' stress and anxiety. Conversely, there are many instances where family problems have been alleviated through strong family support and commitment, or through counselling and other support measures.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/cjdaustralia.html">CJD Information, Links and articles. THE GOVERNMENT FAILED...All the people who have got CJD had been given hGH before 1977. .</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Two thousand one hundred Australians were treated with human pituitary hormones under the Australian Human Pituitary Hormone Program (AHPHP) which ran in Australia from 1967 until 1985. No-one from CSL - whose actions in harvesting the pituitary glands under completely uncontrolled conditions must border on criminal negligence - has ever been held to account. Nor have any members of HPAC, who sought kudos and career advancement ahead of prudent medical practice and patient safety. Overall, 1 in about 300 people treated with hGH got CJD. All CJD patients received some hGH before 1977. Of those treated before 1977, 1 in 104 got CJD. In 1977, the NHPP changed the way it made hGH. Scientists added a new purification step that greatly reduced and may have removed the risk of CJD. So far, no patient who started hGH after 1977 has become ill with CJD. Since CJD takes so long to develop, we still don't know for sure that those who started treatment after 1977 are safe. The longest reported time from the start of hGH treatment to first signs of CJD is 33 years in U.S. patients. In Canada it is 38 years after diagnostic infusion of hGH to measure Growth Hormone</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/guineepigs.html">CSL experiments on Melbourne Orphans without Consent. Alberta sterilization victims used as guinea pigs</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">CSL experiments on Melbourne Orphans without Consent.An investigation by the Melbourne Age newspaper revealed that hundreds of children in orphanages and babies' homes, including wards of state, were used in the experiments and studies over 25 years. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/recommendations.html">Senate Standing Committee of the 38th Parliament of Australia on Community Affairs, Current recommendations 30 May 2003</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Recommendation 15: That once it is established that a person did receive hPG or hGH from the AHPHP, the recipient's status should be of no difference to that of approved recipients. In the event of a dispute between the Department and a person who claims to have received human pituitary derived hormone, the matter should be referred to an independent arbitrator for resolution. </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/cancer.html">Side Effects & Warnings regarding hGH and Anabolic Steroids. Includes Medical Documents, Deaths from Adrenal Crisis & Despite the limitations in their study the high incidence of cancer, and in particular of colon cancer, is worrying</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Side Effects & Warnings, icluding a follow up study overseas of hGH recipiants. Numerous recipiants have died from Adrenal Crisis. We believe that the report completed by Professor Allars, her executive summaries and Dr Lawrence's speech to the House prove beyond any question that there had been significant breaches of the law as it applies to a wide variety of areas. These include the manufacture of the hormone, including the collection of glands, distribution of the hormone by various agencies, including HPAC, CSL and others as detailed in the Allars report, and non-disclosure of possible side effects.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/records.html">Medical records</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">4.1 This term of reference primarily refers to the documents generated by and relating to the Allars Inquiry, which have been at the centre of contention between the Commonwealth and the recipients' legal advisers. An additional grouping of documents has been identified generally as other Departmental and CSL files and records, relevant to matters in issue in the legal proceedings. Within these two groups of documents are records and information held by the Department, and as well by doctors, hospitals and others which may be regarded as relevant to issues concerning individual recipients and their treatment, to which individual recipients have been seeking access. To obtain your Medical Records in regards to HPAC</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/legdoc1.html">Where you prescribed, without due care, anabolic steroids? A legal perspective </a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">You prescribed drugs of addiction for the treatment of seven patients for a period in excess of thirty days without written authorisation</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;">Personal Stories</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">A collection of Personal Stories regarding the growth hormone program. What happens to those experimented on.</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/hgheffects.html">Human Growth Hormone Helps Small Kids Grow, But Some Call Its Wider Use Shortsighted</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">While there is evidence that HGH builds muscle mass and reduces fat in non-deficient adults, it can have side effects including edema, insulin resistance and joint pain. People who take it do so at their own risk and peril, says Pinchas Cohen, chief of pediatric endocrinology at the University of California, Los Angeles (UCLA). </span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/buckbell.html">Three generation are enough. The Victorian Act of Parliament to Experiment on Humans</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">Steroid Priming to sterilize children. Howard Florey Laboratories of Experimental Physiology devoted especially to the study of integrative physiology by long term experiments and these gentlemen and all other donors including the Commonwealth of Australia, the Reserve Bank of Australia and the Rockefeller Foundation made their gifts for that purpose and on these terms the Laboratories were commissioned in 1963:</span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/minister.html">Questions On Notice to the Health Minister. </a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;">After 10 years of asking questions of his treatment to his Endocrinologist and The Dept. of Health, and being told he wasnt at risk, FOI documents reveals the lies written by Drs and the Dept. of Health</span></p><p class="p2" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px; min-height: 23.8px;"><span class="s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span><br /></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 0px;"><span class="s3" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; text-decoration: underline;"><a href="https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/index.html">https://web.archive.org/web/20040815054355/http://mc2.vicnet.net.au/home/shortboys/web/index.html</a></span></p><p class="p1" style="-webkit-text-size-adjust: auto; font-feature-settings: normal; font-kerning: auto; font-optical-sizing: auto; font-size-adjust: none; font-size: 18.4px; font-stretch: normal; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-ligatures: normal; font-variant-numeric: normal; font-variant-position: normal; font-variation-settings: normal; line-height: normal; margin: 9px 0px 8px;"><span class="s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px;"><br /></span></p></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><h2 style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1.86667rem; line-height: 1.4; margin: 0.5em 0px; padding: 0px; text-rendering: optimizelegibility;">CHAPTER 3 - WHETHER THE GOVERNMENTS RESPONSE TO THE ALLARS INQUIRY RECOMMENDATIONS HAS BEEN FAIR AND ADEQUATE</h2><h2 style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1.86667rem; line-height: 1.4; margin: 0.5em 0px; padding: 0px; text-rendering: optimizelegibility;"><a href="https://www.aph.gov.au/sitecore/content/Home/Parliamentary_Business/Committees/Senate/Community_Affairs/Completed_inquiries/1996-99/cjd/report/c03">https://www.aph.gov.au/sitecore/content/Home/Parliamentary_Business/Committees/Senate/Community_Affairs/Completed_inquiries/1996-99/cjd/report/c03</a></h2></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="font-family: UICTFontTextStyleTallBody; font-size: 17px;"><h2 style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1.86667rem; line-height: 1.4; margin: 0.5em 0px; padding: 0px; text-rendering: optimizelegibility;">Report on The CJD Settlement Offer</h2><p align="left" style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><b style="box-sizing: inherit; line-height: inherit;">OCTOBER 1997</b></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">© Commonwealth of Australia 1997</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">ISBN <a href="tel:0 642 25148 7" style="box-sizing: inherit; color: #1f538d; line-height: inherit; text-decoration: none;">0 642 25148 7</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://www.aph.gov.au/Parliamentary_Business/Committees/Senate/Community_Affairs/Completed_inquiries/1996-99/cjd/report/index" style="font-size: 1rem;">https://www.aph.gov.au/Parliamentary_Business/Committees/Senate/Community_Affairs/Completed_inquiries/1996-99/cjd/report/index</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://web.archive.org/web/20211226050406/https://www1.health.gov.au/internet/main/publishing.nsf/Content/E8C62244C221F228CA257BF0001ED9BC/$File/response.pdf">https://web.archive.org/web/20211226050406/https://www1.health.gov.au/internet/main/publishing.nsf/Content/E8C62244C221F228CA257BF0001ED9BC/$File/response.pdf</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://archivescollection.anu.edu.au/index.php/review-of-allars-inquiry-by-whitten;isad">https://archivescollection.anu.edu.au/index.php/review-of-allars-inquiry-by-whitten;isad</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://edm.parliament.uk/early-day-motion/12372/human-growth-hormone-cjd-litigation">https://edm.parliament.uk/early-day-motion/12372/human-growth-hormone-cjd-litigation</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">2024</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">Monday, January 29, 2024</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone.</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html">https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><span style="font-size: 1rem;">Monday, January 29, 2024</span></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">iatrogenic Alzheimer’s disease, Alzheimer’s disease should now be recognized as a potentially transmissible disorder.</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html">https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html</a></p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">Terry S. Singeltary Sr.</p></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-4577221187874389032024-01-30T16:19:00.002-06:002024-02-01T10:36:21.895-06:00Alzheimer’s disease acquired from historic medical treatment<p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Alzheimer’s disease acquired from historic medical treatment</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">30 January 2024 </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Five cases of Alzheimer’s disease are believed to have arisen as a result of medical treatments decades earlier, reports a team of UCL and UCLH researchers. </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Amyloid.jpeg</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Alzheimer’s disease is caused by the amyloid-beta protein, and is usually a sporadic condition of late adult life, or more rarely an inherited condition that occurs due to a faulty gene. The new Nature Medicine paper provides the first evidence of Alzheimer’s disease in living people that appears to have been medically acquired and due to transmission of the amyloid-beta protein.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">The people described in the paper had all been treated as children with a type of human growth hormone extracted from pituitary glands from deceased individuals (cadaver-derived human growth hormone or c-hGH). This was used to treat at least 1,848 people in the UK between 1959 and 1985, and used for various causes of short stature. It was withdrawn in 1985 after it was recognised that some c-hGH batches were contaminated with prions (infectious proteins) which had caused Creutzfeldt-Jakob disease (CJD) in some people. c-hGH was then replaced with synthetic growth hormone that did not carry the risk of transmitting CJD.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">These researchers previously reported that some patients with CJD due to c-hGH treatment (called iatrogenic CJD) also had prematurely developed deposits of the amyloid-beta protein in their brains.* The scientists went on to show in a 2018 paper that archived samples of c-hGH were contaminated with amyloid-beta protein and, despite having been stored for decades, transmitted amyloid-beta pathology to laboratory mice when it was injected.** They suggested that individuals exposed to contaminated c-hGH, who did not succumb to CJD and lived longer, might eventually develop Alzheimer’s disease. </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">This latest paper reports on eight people referred to UCLH’s National Prion Clinic at the National Hospital for Neurology and Neurosurgery in London, who had all been treated with c-hGH in childhood, often over several years. </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Five of these people had symptoms of dementia, and either had already been diagnosed with Alzheimer’s disease or would otherwise meet the diagnostic criteria for this condition; another person met criteria for mild cognitive impairment. These people were between 38 and 55 years old when they started having neurological symptoms. Biomarker analyses supported the diagnoses of Alzheimer’s disease in two patients with the diagnosis, and was suggestive of Alzheimer’s in one other person; an autopsy analysis showed Alzheimer’s pathology in another patient.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">The unusually young age at which these patients developed symptoms suggests they did not have the usual sporadic Alzheimer’s which is associated with old age. In the five patients in whom samples were available for genetic testing, the team ruled out inherited Alzheimer’s disease. </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">As c-hGH treatment is no longer used, there is no risk of any new transmission via this route. There have been no reported cases of Alzheimer’s acquired from any other medical or surgical procedures. There is no suggestion that amyloid-beta can be passed on in day-to-day life or during routine medical or social care.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">However, the researchers caution that their findings highlight the importance of reviewing measures to ensure there is no risk of accidental transmission of amyloid-beta via other medical or surgical procedures which have been implicated in accidental transmission of CJD.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">The lead author of the research, Professor John Collinge, Director of the UCL Institute of Prion Diseases and a consultant neurologist at UCLH, said: “There is no suggestion whatsoever that Alzheimer’s disease can be transmitted between individuals during activities of daily life or routine medical care. The patients we have described were given a specific and long-discontinued medical treatment which involved injecting patients with material now known to have been contaminated with disease-related proteins.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">“However, the recognition of transmission of amyloid-beta pathology in these rare situations should lead us to review measures to prevent accidental transmission via other medical or surgical procedures, in order to prevent such cases occurring in future.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">“Importantly, our findings also suggest that Alzheimer's and some other neurological conditions share similar disease processes to CJD, and this may have important implications for understanding and treating Alzheimer’s disease in the future.”</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Co-author Professor Jonathan Schott (UCL Queen Square Institute of Neurology, honorary consultant neurologist at UCLH, and Chief Medical Officer at Alzheimer’s Research UK) said: “It is important to stress that the circumstances through which we believe these individuals tragically developed Alzheimer’s are highly unusual, and to reinforce that there is no risk that the disease can be spread between individuals or in routine medical care. These findings do, however, provide potentially valuable insights into disease mechanisms, and pave the way for further research which we hope will further our understanding of the causes of more typical, late onset Alzheimer’s disease.”</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">First author Dr Gargi Banerjee (UCL Institute of Prion Diseases) said: “We have found that it is possible for amyloid-beta pathology to be transmitted and contribute to the development of Alzheimer’s disease. This transmission occurred following treatment with a now obsolete form of growth hormone, and involved repeated treatments with contaminated material, often over several years. There is no indication that Alzheimer’s disease can be acquired from close contact, or during the provision of routine care.”</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">The study was supported by the Medical Research Council, the National Institute for Health and Care Research (NIHR), the NIHR UCLH Biomedical Research Centre, Alzheimer’s Research UK, and the Stroke Association.</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">If you were treated with the growth hormone (c-hGH) in the UK between 1959 and 1985 and would like further information about this research, please contact the National Prion Clinic via email (<span dir="ltr">uclh.prion.help@nhs.net</span>) or by telephone (<span dir="ltr">020 7679 5142</span> or <span dir="ltr">020 7679 5036</span>).</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://www.ucl.ac.uk/prion/news/2024/jan/alzheimers-disease-acquired-historic-medical-treatments">https://www.ucl.ac.uk/prion/news/2024/jan/alzheimers-disease-acquired-historic-medical-treatments</a><br /></span></p><p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Posted by flounder on 05 Nov 2014 at 21:27 GMT </span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a><br /></span></p><p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">MONDAY, JANUARY 29, 2024</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">''The clinical syndrome developed by these individuals can, therefore, be termed iatrogenic Alzheimer’s disease, and Alzheimer’s disease should now be recognized as a potentially transmissible disorder.''</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html">https://betaamyloidcjd.blogspot.com/2024/01/iatrogenic-alzheimers-disease-in.html</a><br /></span></p><p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Monday, January 29, 2024</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">iatrogenic Alzheimer’s disease, Alzheimer’s disease should now be recognized as a potentially transmissible disorder</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Iatrogenic Alzheimer’s disease in recipients of cadaveric pituitary-derived growth hormone</span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /></p><p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html">https://itseprion.blogspot.com/2024/01/iatrogenic-alzheimers-disease.html</a><br /></span></p><p>WEDNESDAY, JANUARY 31, </p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;">Creutzfeldt Jakob Disease, CJD Support Group for short statured children of the 1970's and 1980's And 2024 Alzheimer’s iatrogenic Transmission</p><p style="-webkit-text-size-adjust: auto; box-sizing: inherit; caret-color: rgb(34, 34, 34); color: #222222; font-family: "Open Sans", "Helvetica Neue", Helvetica, Roboto, Arial, sans-serif; font-size: 1rem; line-height: 1.6; margin: 0px 0px 1.33333rem; padding: 0px; text-rendering: optimizelegibility;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html" style="font-size: 1rem;">https://creutzfeldt-jakob-disease.blogspot.com/2024/01/creutzfeldt-jakob-disease-cjd-support.html</a></p><p><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"></span><br style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;" /><span style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">terry</span></p>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-8729207114198410172024-01-20T12:51:00.001-06:002024-01-20T14:39:14.498-06:00Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms separator <p><span style="background-color: white; font-family: arial; font-size: 16px;">Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms separator </span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Angéline Denouel1 , Jean-Philippe Brandel1,2 , Laurène Peckeu-Abboud3 , Danielle Seilhean1 , Elodie Bouaziz-Amar4,5 , Isabelle Quadrio6,7 , Jean-Baptiste Oudart8,9,10 , Sylvain Lehmann11 , Pantxika Bellecave12 , Jean-Louis Laplanche4,5 ,</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Key public health message</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">What did you want to address in this study?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Prion diseases are rare, devastating brain diseases that are transmissible and always fatal. They include genetic, sporadic and infectious forms, e.g. from consumption of contaminated beef (i.e. mad cow disease) or treatment using human-derived medical products. Using the French prion surveillance data, we analysed the data collected since 1992 to understand the appearance of different forms of prion diseases over time within the general population.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">What have we learnt from this study?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Infectious forms, and notably cases after treatment with growth hormone of human origin or from consumption of contaminated beef, decreased over time. On the contrary, the number of sporadic cases, for which the cause remains unknown, tended to increase without clear explanation. </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">What are the implications of your findings for public health?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">The decreasing trend of infectious forms we observed in France demonstrates the effectiveness of measures taken to limit prion diseases in the general population. The tendency toward an increase of sporadic forms, also noted in other countries, as well as their unclear origin and the emergence of new prion diseases in animals consumed by humans, underline the need of sustaining an active surveillance.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Snip…</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">DiscussionGo to section... Following the emergence of hGH-iCJD cases and the increasing number of BSE cases in the UK, several European countries, including France, implemented an epidemiological surveillance of human TSEs [23]. This enabled the rapid identification of a new form of CJD linked to a cross-species contamination with the BSE agent (vCJD) [10,11,13-16]. The active CJD surveillance network initiated at the national level in France in 1992 has provided long-term data on this rare group of diseases.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Consistent with series collected by other surveillance systems, we report the same distribution of sporadic CJD (around 85%) [5-8]. Genetic CJD represent 10–15% of cases worldwide [4,34,35]. The occurrence of infectious forms varies between countries: vCJD is primarily reported in UK (n = 178 cases) and France (n = 27 cases), while in some other countries, only 1 to 5 cases of vCJD have been observed since the initiation of CJD surveillance [8,36]. With respect to hGH-iCJD cases, the most affected countries in Europe are the UK [5] and France (n = 79 and 116, respectively); fewer hGH-iCJD cases are observed in the other European countries [37].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">The number of TSE suspicions increased progressively over time in France, as reported in other countries with a similar surveillance system, such as the UK and Italy [7,38]. This can be explained by network implementation, improvement in case identification [39] (especially for the first years of surveillance) and by population ageing, with an increasing number of older people (≥ 65 years) (‘The National Institute of Statistics and Economic Studies’ (Insee, https://www.insee.fr/en/accueil) who are the most affected by dementias including CJD.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">We observed yearly variations in the number of sCJD cases, as reported in other countries by the EuroCJD network [38]. An annual variation of almost 50% in the sCJD mortality is not unusual and not necessarily worrying. However, in the last decades, an increasing trend of sCJD mortality over time occurred in France as well as in other countries [38,40]. Our data show that the evolution of sCJD diagnostic criteria increased the sensitivity contributing to better case detection. More recently, detection of cortical high signals on MRI sequences in at least two different regions of the brain were introduced on criteria in 2017 as well as the results of real-time quaking-induced conversion (RT-QuIC), an amplification method used to detect low amount of PrPsc in cerebrospinal fluid (CSF). The impact of improved diagnosis criteria on measured sCJD mortality should be evaluated in large series. However, even if an intense surveillance system can explain better case ascertainment [39], it cannot be excluded that an actual concurrent increase of sCJD cases occurred over time because of unknown factors [40].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Analyses of PRNP codon 129 of sCJD patients showed an excess proportion of homozygotes at codon 129 (79%, including 60% of MM) in comparison with the French general population (50% of homozygotes) [41]. This observation supports that methionine homozygosity is a susceptibility factor for sCJD occurrence [27,42]. Of the different subtypes, MM1/MV1 was the commonest sCJD subtype with the shortest disease duration (median: 3 months), as previously described [30]. In our study, we merged data from MM1 and MV1 into one subtype, as performed in previous studies, since these molecular subtypes share common clinicopathological characteristics and are both associated with the M1 sCJD prion strain as shown by strain typing in experimental models [9,43,44]. It is worth noting, however, that disease duration was longer in French MV1 than in MM1 cases. More precisely, a subgroup of MV1 cases showed a longer disease duration (data not shown) suggesting that MM1/MV1 subtype might be divided into two subtypes. Gelpi et al. [45] recently identified a new subtype of sCJD in patients carrying MV at PRNPcodon 129 with PrPres type 1. These patients presented distinctive clinicopathological features and a long duration (mean: 20.5 months). Further investigations are needed to assess whether the French MV1 patients with longer duration we studied are consistent with this recent observation from Spanish and Italian patients.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">The subtype distribution was different for younger sCJD cases with a more frequent proportion of VV1 and MM2 subtypes. The shorter frequency of typical cases (MM1/MV1) in young people compared with patients with sCJD over 50 years old remains to be explained. Indeed, taking the hypothesis of a stochastic conversion of PrP as the event causing sCJD occurrence, the subtype distribution should be the same regardless of the age of the individuals. The specific strain distribution we observed in younger patients compared with older ones might be related to a specific strain selection pressure modulated by age-related endogenous factors (such as the proteostasis system) or to a distinct causative event in some younger patients such as an exposure to exogenous factors.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">The largest series of hGH-iCJD cases has been observed in France and the present study confirms a specific time distribution of cases according to the codon 129 genotype, as suggested by Brandel et al. in 2003 [20]. The first French hGH-iCJD cases were all homozygous and the first MV patient was reported 5 years after the onset of the epidemic. More precisely, all valine homozygous hGH-iCJD cases occurred in 2000 and before except for one case that was reported in 2015. A neuropathological examination was not performed and even if the case was actually treated with at-risk batches of cadaverous human pituitary growth hormone [33], we cannot exclude the possibility of a misclassification of a sCJD into a hGH-iCJD case because of his medical history. Clinical characteristics resembled those of sCJD VV2 subtype: 3-month survival time, early ataxia and no dementia at onset, no typical EEG, positive CSF 14-3-3 detection and high signals in basal ganglia on MRI. Of note, our study from 2020 showed that the incubation period was significantly shorter in valine homozygotes than heterozygotes [46], whereas this last hGH-iCJD case showed an extreme incubation period (31 years) in comparison with the other homozygous valine (16 years or less) and with MM and MV cases (25 years or less).</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">France was the second most affected country by vCJD after the UK. In France, 27 cases of primary vCJD were reported during our study period and the last one occurred in 2014. Two additional cases were observed in 2020 and in 2021 that occurred after occupational exposure to the BSE agent in research laboratories. In 2020, we reported a definite vCJD case in a research technician who experienced an accidental occupational exposure to the classical BSE agent in a prion research laboratory 7.5 years before the disease onset [47]. Even if oral transmission related to contaminated cattle product consumption cannot be formally excluded, the hypothesis of an occupational contamination was reinforced in 2021 with the occurrence of a case of probable vCJD in a retired laboratory worker who also experienced an accidental occupational exposure to the BSE agent 15 years before clinical onset. Both patients were homozygous methionine at codon 129. In 2016, a heterozygous vCJD case was reported in the UK, raising fears of the emergence of a second wave of MV individuals related to a longer incubation period [48]. However, to date, no further heterozygous cases have been reported worldwide, which does not support this hypothesis.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">One of the remaining concerns is the risk of secondary contamination in individuals that received blood transfusion from donors incubating vCJD. In the UK, all transfusion-transmitted vCJD cases occurred within 10 years following the transfusion with non-leuco-depleted blood (measure used to prevent bloodborne transmission). In France, last vCJD donors died in 2004 and, up to 2023, no patient who has received labile leuco-depleted blood products from these donors developed symptoms of CJD, not even the patient that received red blood cells prepared from blood donation that retrospectively tested positive by PMCA in plasma [32].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Another concern comes from chronic wasting disease (CWD), a contagious form of prion diseases responsible for epidemics in cervids, the zoonotic potential of which is still debated. The emergence of new CWD strains in European countries was recently demonstrated [49-51]. </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">During our 25-year surveillance, genetic analyses of the PRNP gene revealed 240 cases of TSE caused by a genetic mutation, and identified several new mutations [24,52]. The commonest observed one was the E200K responsible for a gCJD phenotype [34]. The mutation D178N associated with two distinct phenotypes depending on the genotype at codon 129 on the allele carrying the mutation constitutes the second most frequent mutation in France. Among patients with a FFI phenotype, one had E200K mutation. This genotype/phenotype combination, which was neuropathologically confirmed, has been very rarely reported [53-55].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Our study has some limitations. Firstly, the two neurologists in charge of the French surveillance network did not systematically consult all CJD patients (except for vCJD suspected cases) meaning that clinical signs at disease onset are not known with certainty for each patient. They do not have access to all EEGs and MRI data for all patients (only medical reports) and some data might be missed or misinterpreted. Secondly, genetic analyses of PRNP gene and neuropathological examination of suspected cases are not systematically performed, which may lead to some form misclassifications, especially cases considered as sporadic instead of genetic because of missing genetic information. However, this limitation is encountered in the majority of surveillance systems. Finally, in our sCJD population, few patients were aged more than 89 years at disease onset (n = 8). This population had very short disease duration (median: 2 months) that might make the diagnosis more difficult, and we cannot exclude the fact that some cases in the oldest age group were missed by the surveillance system or misdiagnosed.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"> ConclusionGo to section... An active nationwide surveillance was implemented in France in 1992 providing 25 years of data. This enabled us to describe the epidemiology and subtypes of sCJD, including those cases observed in unexpected age groups, and on the epidemic profiles of infectious human prion diseases notably those acquired after peripheral contamination. Sustaining an active surveillance is needed regarding uncertainties about future primary or secondary vCJD cases, the recent occurrence of chronic wasting disease in European cervids with possible zoonotic potential and the tendency towards a regular increase of sCJD mortality observed in various countries.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://www.eurosurveillance.org/content/10.2807/1560-7917.ES.2023.28.50.2300101" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.eurosurveillance.org/content/10.2807/1560-7917.ES.2023.28.50.2300101</a><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><span style="outline: currentcolor;">SUNDAY, DECEMBER 24, 2023 </span></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><span style="outline: currentcolor;"><br style="outline: currentcolor;" /></span></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><span style="outline: currentcolor;">France Sporadic CJD Rising, Typical and Atypical, BSE, Scrapie, and CWD, Zoonosis, what if?</span><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">France CJD cases continue to rise, what about zoonotic TSE to humans?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France is having a serious problem with continued spontaneous atypical BSE cases?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France Chronic Wasting Disease CWD TSE Prion in cervid?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France, what about their typical and atypical Scrapie cases?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France, what about TSE Prion tainted feed for livestock?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France, sCJD, Iatrogenic CJD cases, misdiagnosis, what if?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Seems to me, if atypical BSE was as spontaneous and sporadic as they claim it to be, it would be the same across the board, however, this hypothesis is crumbling, imo. </div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">snip...</div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/france-sporadic-cjd-rising-typical-and.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/france-sporadic-cjd-rising-typical-and.html</a><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="-webkit-text-size-adjust: auto;">Tuesday, November 08, 2011</div><div style="-webkit-text-size-adjust: auto;"><br /></div><div style="-webkit-text-size-adjust: auto;">Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011</div><div style="-webkit-text-size-adjust: auto;"><br /></div><div style="-webkit-text-size-adjust: auto;">Original Paper</div><div style="-webkit-text-size-adjust: auto;"><br /></div><div style="-webkit-text-size-adjust: auto;">Conclusions: These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.</div><div style="-webkit-text-size-adjust: auto;"><br /></div><div style="-webkit-text-size-adjust: auto;"><a href="http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html" rel="nofollow" target="_blank">http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html</a></div></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Direct neural transmission of vCJD/BSE in macaque after finger incision CORRESPONDENCE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Direct neural transmission of vCJD/BSE in macaque after finger incision</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Jacqueline Mikol1 · Jérôme Delmotte1 · Dolorès Jouy1 · Elodie Vaysset1 · Charmaine Bastian1 · Jean‑Philippe Deslys1 ·</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Emmanuel Comoy1 Received: 10 July 2020 / Revised: 8 September 2020 / Accepted: 25 September 2020 / Published online: 6 October 2020 © The Author(s) 2020</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Non-human primates appeared as the closest model to study human iatrogenic prion diseases [14]: we report here the consequences of variant Creutzfeldt–Jakob disease/bovine spongiform encephalopathy (vCJD/BSE) inoculation in a cynomolgus macaque finger, with the demonstration of an original mode of propagation and the practical risk for professional exposure.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The distal right middle finger handpad of a 4-year-old macaque was incised on both lateral sides to induce local inflammation, and then injected with the equivalent of 10 mg of a BSE, orally challenged macaque brain [18]. After an 18 months period of finger clumsiness, the clinical disease (behaviour abnormalities, fear, hyperesthesia, gait disturbances, shaking) began 7.5 years after inoculation and euthanasia took place 2 months later for welfare reasons. Motor conduction velocity of the right median nerve was reduced to one-third of the left counterpart and sensory potential was not detected.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Histological and biochemical studies were performed as previously described. All the elements of the triad were present [7–9]: spongiform change was moderate in neocortex, striatum, brain stem, mild in spinal cord but severe in thalamus and cerebellum; neuronal loss was globally moderate, but severe in cerebellum and sacral spinal cord (vacuolated neurons); gliosis was severe in thalamus, cerebellum and brain stem and moderate elsewhere (Supplementary Fig. 1). ELISA and western blot (WB) showed the expected accumulation of PrPres with BSE glycophoretic pattern at all levels of brain and spinal cord (Supplementary Fig. 2).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the brain, PrPd deposits were laminar into the cortical deep layers, massive into thalamus, basal ganglia, cerebellum, and brain stem. In spinal cord, PrPd was symmetrically distributed, intense in the Substantia gelatinosa and nucleus dorsal of Clarke while decreased at sacral level. Deposits were diverse into the whole CNS: synaptic, perineuronal, reticular aggregates, mini-plaques, plaques, and incomplete florid plaques. The retinal plexiform layers were labelled (Supplementary Fig. 1i). There were no amyloid or tau deposits.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Unusual PrPd deposits were observed along dendrites, short and long axons, neuritic threads tracing fne networks of straight lines or like strings of pearls (Supplementary Fig. 3). They were present into deep neocortex, basal ganglia, and motoneurons. Such long processes are not frequent but have been reported in human [13] and experimental studies [10, 22]. PrPd deposits were also noted as very mild into striato-pallidal projections, both limbs of internal capsule and fornix (Supplementary Fig. 3). The presence of PrPd in white matter has been reported (Supplementary text 4).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Peripherally, the expected PrPd was undetectable in lymphoid organs, including spleen, through biochemical or immunohistochemical analyses, while prion replication was detected in the peripheral nervous system (PNS): PrPd staining was visualized in many dorsal root ganglia (DRG) but only in nerves innervating the forelimb site of injection (median and ulnar nerves). At the cellular level, PrPd was limited to ganglia and satellite cells in DRG and Schwann cells (Scs) all along nerves whereas axons were never labelled (Fig. 1). Previously, using postmortem immunohistochemical studies (listed in Supplementary text 5), PrPd has been shown in peripheral nervous system in all forms of human neuropathies, albeit more frequently in vCJD, mostly in posterior root nerve fbres at adaxonal location and/or in ganglion and satellite cells. The restricted amount of PrPd was repeatedly underlined but, recently, prion RTQuiC was positive in all nerves examined [2]. PrPd has also been described, frst in scrapie [17] then in BSE, as limited “adaxonal deposits” or/and Sc deposits, with or without DRG cell involvement (review in [4] and Supplementary text 6). Previous studies of the mode of propagation of PrPd have reported variable observations and analyses depending on strains, host species and genotype (Supplementary text 6); the authors discussed the role of the sensory route of trafficking of prions, the modifications of axonal transport, the centrifugal versus centripetal spread of PrPd .</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">After peripheral infection, accumulation of infectious agent is reputed to occur in lymphoid tissues before direct neuroinvasion [18, 19], even with very little apparent peripheral lymphoreticular deposition [6, 20]. Here, there is no apparent replication/amplification of vCJD/BSE agent in the lymphoid tissues of the exposed macaque. In this model, the neural contamination occurred directly in the highly innervated finger while neuroinvasion appears to occur in Scs along the median nerve to the DRG, with the appearance of the classical labelling of ganglion cells which indicates the onset of the first level of neuronal infection. This model provides direct evidence of the hypothesis of a sequential infection of Scs from the periphery to the CNS, followed by a secondary diffusion into the spinal cord, as already considered by our group [15] and others [1, 3, 11, 12, 21]. It is to note that studies based on intra-sciatic nerve injections in hamsters [16] and transgenic mice [12] had established a rate of transport of infectivity of, respectively, 0.5–2 mm and 0.7 mm per day. This key role of Scs could explain both the low speed of propagation and the discrepancy between the paucity of PrPd into the distal part of the sensory nerves followed by the positivity of DRG, satellite cells and proximal roots.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In conclusion, we have observed that the exposure of a primate to vCJD/BSE through a distal finger lesion induces, after more than 7.5 years of silent incubation, a massive deposit of PrPd , strictly restricted to the nervous system and the eye.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our data suggest a new type of pure unique peripheral nervous contamination in which the Scs would have a major role in the mode of centripetal progression of PrPd in the peripheral nervous system. Moreover, considering the fact that, recently, “a variant CJD diagnosed 7.5 years after occupational exposure” (cryomicrotomy) in a technician was observed [5], this experimental case report supports the risk linked to professional exposure and reinforces the necessity of adequate measures of prevention. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Second death in France in a laboratory working on prions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Creutzfeldt-Jakob disease has killed a person who handled this infectious agent at Inrae in Toulouse. After a first death in 2019, a moratorium on work on this pathogen has been extended.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">By Hervé Morin</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working with these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of mice infected with prions, in another unit of INRAE, in Jouy-en-Josas.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Computer representation of part of a prion protein on a light micrograph of pyramidal nerve cells (neurons, in black) in the cerebellum of the brain. ALFRED PASIEKA / SCIENCE PHOTO LIBRARY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Temporary suspension of work on prions in French public research laboratories</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRESS RELEASE - The general directorates of ANSES, CEA, CNRS, INRAE and Inserm, have decided jointly and in agreement with the Ministry of Higher Education, Research and Innovation to suspend as a precaution all their research and experimentation work relating to prion diseases, for a period of three months.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This precautionary measure is motivated by the knowledge of a possible new case of a person suffering from Creutzfeldt-Jakob disease and who worked in a laboratory for research on prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted on July 27, 2021</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The suspension period put in place as of this day will make it possible to study the possibility of a link between the observed case and the person's former professional activity and to adapt, if necessary, the preventive measures in force in the research laboratories. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The person with Creutzfeldt-Jakob disease (CJD)1, whose form is not yet known, is a retired INRAE agent. This could be the second case of infectious CJD affecting a scientist who worked on prions, after that of an assistant engineer who died of the disease in 2019, and who was injured in 2010 during of an experiment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Following this death, a general inspection mission was launched in July 2019 by the ministries of research and agriculture with French laboratories handling prions. Submitted in October 2020, the report concluded on the regulatory compliance of the laboratories visited as well as the presence of a risk control culture within the research teams.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research around prion proteins, with high public health issues, allows major advances in the understanding of the functioning of these infectious pathogens, and contributes to results that are transferable to other related degenerative diseases such as Alzheimer's and Alzheimer's diseases. Parkinson's.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">At the level of each establishment, regular and transparent information will be provided to all the working communities concerned by this measure.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1 The disease Creutzfeldt-Jakob disease (CJD) is one of prion diseases - still called encephalopathies subacute spongiform transmitted(TSE) - of diseases rare, characterized by a degeneration rapid and fatal the system nervous central. They are caused by the accumulation in the brain of a normally expressed protein but poorly conformed - the prion protein - which leads to the formation of deleterious aggregates for neurons. For now , no treatment will allow to change the course of these diseases. It can be of origin sporadic , form the most frequent , original genetic or finally to form infectious following a contamination. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">By Barbara CasassusJul. 28, 2021 , 4:35 AM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">With reporting by Martin Enserink.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted in: EuropeHealthScientific Community</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">doi:10.1126/science.abl6587</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TO THE EDITOR:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Figure 1.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">5 References</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">July 2, 2020</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">N Engl J Med 2020; 383:83-85</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">DOI: 10.1056/NEJMc2000687</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Metrics</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a> </div></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">MONDAY, SEPTEMBER 11, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">SUNDAY, NOVEMBER 26, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Prion 2023</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A Real World Evidence Study</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Funded by: Ionis Pharmaceuticals</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Grant number: N/A</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Acknowledgment: XXX</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">22 years ago;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">2001 Singeltary on CJD</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">February 14, 2001</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Terry S. Singeltary, Sr</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Author Affiliations</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Saturday, January 20, 2024 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Original histological phenotype after the experimental transmission to primate of an unusual human prionopathy (VPSPr)</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://vpspr.blogspot.com/2024/01/original-histological-phenotype-after.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://vpspr.blogspot.com/2024/01/original-histological-phenotype-after.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">TUESDAY, DECEMBER 12, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">***> CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023 <***</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">***> 2023 Professor John Collinge on tackling prion diseases <***</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">There is accumulating evidence also for iatrogenic AD. </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">TUESDAY, JANUARY 16, 2024 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">CIDRAP launches international effort to prepare for possible chronic wasting disease spillover </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Chronic Wasting Disease CWD TSE Prion Spillover to other Species, What If? </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2024/01/cidrap-launches-international-effort-to.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2024/01/cidrap-launches-international-effort-to.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Thursday, March 24, 2016 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">MONDAY, MAY 2, 2016 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France Confirms Case of Classical Mad Cow Disease BSE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE identified in France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://bovineprp.blogspot.com/2016/05/france-confirms-case-of-classical-mad.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://bovineprp.blogspot.com/2016/05/france-confirms-case-of-classical-mad.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** France stops BSE testing for Mad Cow Disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***atypical spontaneous BSE in France LOL***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.nature.com/articles/srep11573</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">spontaneous atypical BSE ???</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">if that's the case, then France is having one hell of an epidemic of atypical BSE, probably why they stopped testing for BSE, problem solved $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">As of December 2011, around 60 atypical BSE cases have currently been reported in 13 countries, *** with over one third in France.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.biomedcentral.com/1746-6148/8/74" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.biomedcentral.com/1746-6148/8/74</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">If you Compare France to other Countries with atypical BSE, in my opinion, you cannot explain this with ‘spontaneous’.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Table 1: Number of Atypical BSE cases reported by EU Member States in the period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE databases on 1 July 2014). By 2015, these data might be more comprehensive following a request from the European Commission to Member States for re-testing and retrospective classification of all positive bovine isolates in the EU in the years 2003–2009</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE type</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Country 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a) 2014(a) Total</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">H-BSE Austria 1 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France(b) 1 2 3 1 2 2 2 2 15</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Germany 1 1 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ireland 1 1 2 1 5</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Netherlands 1 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Poland 1 1 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Portugal 1 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Spain 1 1 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sweden 1 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">United Kingdom 1 1 1 1 1 5</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">L-BSE Austria 1 1 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Denmark 1 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** France(b) 1 1 1 1 2 1 3 2 1 1 14</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Germany 1 1 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Italy 1 1 1 1 1 5</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Netherlands 1 1 1 3</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Poland 1 2 2 1 2 1 2 1 12</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Spain 2 2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">United Kingdom 1 1 1 1 4</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Total Atypical cases (H + L)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2 8 6 5 4 5 8 5 7 8 8 7 5 2 80</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(a): Data for 2013-2014 are incomplete and may not include all cases/countries reported.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(b): France has performed extensive retrospective testing to classify BSE cases, which is probably the explanation for the higher number of Atypical BSE cases reported in this country.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The number of Atypical BSE cases detected in countries that have already identified them seems to be similar from year to year. In France, a retrospective study of all TSE-positive cattle identified through the compulsory EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively, which increased to 1.9 and 1.7 cases per million, respectively, in tested animals over eight years old (Biacabe et al., 2008). No comprehensive study on the prevalence of Atypical BSE cases has yet been carried out in other EU Member States. All cases of Atypical BSE reported in the EU BSE databases have been identified by active surveillance testing (59 % in fallen stock, 38 % in healthy slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported in animals over eight years of age, with the exception of two cases (one H-BSE and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et al., 2012).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.efsa.europa.eu/sites/default/files/scientific_output/files/main_documents/3798.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.efsa.europa.eu/sites/default/files/scientific_output/files/main_documents/3798.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html</a> </div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><a href="https://bovineprp.blogspot.com/2017/01/us-lifts-french-beef-mad-cow-bse-import.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bovineprp.blogspot.com/2017/01/us-lifts-french-beef-mad-cow-bse-import.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">MONDAY, MAY 2, 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France Confirms Case of Classical Mad Cow Disease BSE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bovineprp.blogspot.com/2016/05/france-confirms-case-of-classical-mad.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bovineprp.blogspot.com/2016/05/france-confirms-case-of-classical-mad.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">Thursday, March 24, 2016 </span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;"><br style="outline: currentcolor;" /></span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes </span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><a href="http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html</a><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022. Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation. Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286. DOI: https://doi.org/10.1080/19336896.2022.2091286 Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation). This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Highlights</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Location: Virus and Prion Research<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Title: The agent of transmissible mink encephalopathy passaged in sheep is similar to BSE-L<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Author CASSMANN, E - Oak Ridge Institute For Science And Education (ORISE) Greenlee, Justin BALKEMA-BUSCHMANN, A - Friedrich-Loeffler-institut GROSCHUP, M - Friedrich-Loeffler-institut MOORE, S - Oak Ridge Institute For Science And Education (ORISE) Kokemuller, Robyn Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 3/29/2019 Publication Date: 5/18/2019 Citation: Cassmann, E.D., Greenlee, J.J., Balkema-Buschmann, A., Groschup, M.H., Moore, S.J., Kokemuller, R. 2019. The agent of transmissible mink encephalopathy passaged in sheep is similar to BSE-L. Prion. 13. Article 49.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">https://doi.org/10.1080/19336896.2019.1615197.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">DOI: <a href="https://doi.org/10.1080/19336896.2019.1615197" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1080/19336896.2019.1615197</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Interpretive Summary: Technical Abstract: Transmissible mink encephalopathy (TME) is a fatal neurologic prion disease of farmed mink. Epidemiologic and experimental evidence following a Wisconsin outbreak in 1985 has linked TME to low-type bovine spongiform encephalopathy (BSE-L). Evidence suggests that farmed mink were likely exposed to BSE-L infected downer cattle that were fed to the mink. The interspecies transmission of TME to cattle has been documented. Recently, we demonstrated the susceptibility of sheep to cattle passaged TME by intracranial inoculation.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">The aim of the present study was to compare ovine passaged cattle TME to other prion diseases of food-producing animals. Using a transgenic mouse model, we characterized the disease phenotype of sheep TME to BSE-C and BSE-L. Separate inoculants of sheep passaged TME were derived from animals with the VRQ/VRQ (VV136) and ARQ/VRQ (AV136) prion protein genotype. Transgenic bovinized mice (TgBovXV) were intracranially inoculated with 20 µl of 1% w/v brain homogenate. The disease phenotypes were characterized by comparing the attack rates, incubation periods, and vacuolation profiles in TgBovXV mice.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">The attack rate for BSE-C (13/13), BSE-L (18/18), and TMEVV (21/21) was 100%; whereas, the TMEAV group (15/19) had an incomplete attack rate. The average incubation periods were 299, 280, 310, and 535 days, respectively. The vacuolation profiles of BSE-L and TMEVV were most similar with mild differences observed in the thalamus and medulla. Vacuolation profiles from the BSE-C and TMEAV experimental groups were different than TMEVV and BSE-L. Overall the phenotype of disease in TME inoculated transgenic mice was dependent on the sheep donor genotype (VV vs AV). The results of the present study indicate that TME isolated from VRQ/VRQ sheep is similar to BSE-L by incubation period, attack rate, and vacuolation profile.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Our findings are in agreement with previous research that found similarities between BSE-L and TME. In this study, the similarities between TME and BSE-L are maintained after multiple interspecies passages.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">update<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2021" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2021</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Location: Virus and Prion Research<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">2021 Annual Report<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">snip...<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">4. Passing Transmissible Mink Encephalopathy (TME) prions from cattle to sheep changed the ability of the prions to infect mice. Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal brain diseases that affect livestock species. Prion diseases have been shown to jump species as exhibited when classical bovine spongiform encephalopathy (BSE) infected cattle products were consumed by humans resulting in variant Creutzfeldt-Jakob disease. Another example of cross-species transmission results in a disease of farmed mink known as transmissible mink encephalopathy (TME), the origins of which were not previously understood; however, one possible source is L- BSE from cattle. The present study was designed to determine the effect of cross-species transmission of TSEs in livestock on the ability to infect mice expressing the cattle prion protein. We found that passing cattle adapted TME (TME that was previously passaged in cattle) to sheep changed the ability of the prions to infect bovinized mice in a laboratory inoculation. These results were compared to atypical BSE (L-BSE type) and Classical BSE in bovinized mice. Depending on the genotype of sheep used, the disease in mice appeared similar by histologic and western blot analysis to either L-BSE or C-BSE. These results indicate a shift in the disease presentation based on transmission through sheep with different genotypes. This information gives insight into origins and development of new prion strains. Because disease in one of the groups of mice resembled the L-BSE phenotype, it supports the hypothesis that TME can originate from feeding mink protein from cattle afflicted with L-BSE.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2021" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2021</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">snip...<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">NOW, back to those mad mink i.e. TME. let me throw a curve ball here ;<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* and Richard A. Bessen† Emerging Infectious<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine- passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, Htype BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profi les, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME. The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE. Transmissible mink encephalopathy (TME) is a rare prion disease in ranch-raised mink (Mustela vison) in North America and Europe (1–4). Six outbreaks have been reported from 1947 through 1985 in North America, and several have been linked to contaminated commercial feed (1). Although contamination of feed with scrapie-infected sheep parts has been proposed as the cause of TME, the origin of the disease remains elusive. The idea that scrapie in sheep may be a source of TME infection is supported by fi ndings that scrapie-infected mink have a similar distribution of vacuolar pathologic features in the brain and the same clinical signs as mink with natural and experimental TME (5). However, mink are not susceptible to scrapie infection following oral exposure for up to 4 years postinoculation, which suggests that either the scrapie agent may not be the source of natural TME infection or that only specifi c strains of the scrapie agent are able to induce TME (6,7). Epidemiologic investigations in the Stetsonville, Wisconsin, outbreak of TME in 1985 suggested a possible cattle origin, since mink were primarily fed downer or dead dairy cattle but not sheep products (8). Experimental transmission of Stetsonville TME into cattle resulted in transmissible spongiform encephalopathy (TSE) disease with an incubation period of 18.5 months. Back passage of bovine TME into mink resulted in incubation periods of 4 and 7 months after oral or intracerebral inoculation, respectively, which was similar to that found following inoculation of Stetsonville TME into mink by these same routes (8). These findings indicated that cattle are susceptible to TME, and that bovine-passaged TME did not result in a reduced pathogenicity for mink. These studies raised the question as to whether an unknown TSE in cattle was the source of TME infection in the Stetsonville outbreak. Several additional TME outbreaks in the United States have been associated with mink diet that contained downer or dead cattle (9). ...<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">snip...full text ;<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="http://www.cdc.gov/EID/content/13/12/pdfs/1887.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.cdc.gov/EID/content/13/12/pdfs/1887.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="http://transmissible-mink-encephalopathy.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://transmissible-mink-encephalopathy.blogspot.com/</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">What about that TME TSE Prion surveillance and testing program, I’m still waiting?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">WEDNESDAY, JANUARY 12, 2022<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA, what if?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://bovineprp.blogspot.com/2022/01/bovine-spongiform-encephalopathy-bse.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bovineprp.blogspot.com/2022/01/bovine-spongiform-encephalopathy-bse.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">THURSDAY, FEBRUARY 03, 2022<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Transmissible Mink Encephalopathy TME, Atypical L-Type BSE PrP, and typical C-Type BSE, which came first, the cart or the horse?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://transmissible-mink-encephalopathy.blogspot.com/2022/02/transmissible-mink-encephalopathy-tme.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissible-mink-encephalopathy.blogspot.com/2022/02/transmissible-mink-encephalopathy-tme.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://bovineprp.blogspot.com/2021/10/bovine-spongiform-encephalopathy-bse.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bovineprp.blogspot.com/2021/10/bovine-spongiform-encephalopathy-bse.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">USA 50 State Emergency BSE Conference Call 2001<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">FRIDAY, DECEMBER 22, 2023<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">The Mad Cow That Stole Christmas, 20 Years Later<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">2023-2023 TSE PRION UPDATE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...<br style="outline: currentcolor;" /></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Monday, May 22, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES? </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html</a> </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/5067</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">SATURDAY, MAY 20, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div>MAY 19, 2023</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">WEDNESDAY, NOVEMBER 08, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ireland Atypical BSE confirmed November 3 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, NOVEMBER 14, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ireland Atypical BSE case, 3 progeny of case cow to be culled </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SUNDAY, JULY 16, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Switzerland Atypical BSE detected in a cow in the canton of St. Gallen </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Monday, March 20, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BRAZIL BSE START DATE 2023/01/18</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BRAZIL BSE CONFIRMATION DATE 2023/02/22</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BRAZIL BSE END DATE 2023/03/03</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SPAIN BSE START DATE 2023/01/21</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SPAIN BSE CONFIRMATION DATE 2023/02/03</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SPAIN BSE END DATE 2023/02/06</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NETHERLANDS BSE START DATE 2023/02/01</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NETHERLANDS BSE CONFIRMATION DATE 2023/02/01</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NETHERLANDS BSE END DATE 2023/03/13</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Monday, May 22, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES? </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html</a> </div></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wahis.woah.org/#/in-review/5067</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">THURSDAY, NOVEMBER 9, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">European Food Safety Authority (EFSA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">APPROVED: 25 October 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386</a></div></div></div></div></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"></div></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">FRIDAY, JANUARY 20, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://scrapie-usa.blogspot.com/2023/01/epidemiology-of-scrapie-in-united-states.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://scrapie-usa.blogspot.com/2023/01/epidemiology-of-scrapie-in-united-states.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">FRIDAY, NOVEMBER 25, 2022 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> USA National Scrapie Eradication Program (NSEP) 2021 to 2003 A Year by Year Review<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//scrapie-usa.blogspot.com/2022/11/usa-national-scrapie-eradication.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://scrapie-usa.blogspot.com/2022/11/usa-national-scrapie-eradication.html</a></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">WEDNESDAY, FEBRUARY 03, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Scrapie TSE Prion United States of America a Review February 2021 Singeltary et al</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://scrapie-usa.blogspot.com/2021/02/scrapie-tse-prion-united-states-of.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://scrapie-usa.blogspot.com/2021/02/scrapie-tse-prion-united-states-of.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div></div><div dir="ltr" style="outline: currentcolor;"><div style="font-family: arial; outline: currentcolor;">Chronic Wasting Disease Carcass Disposal Dumpster Management and Biosecurity</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">BACKGROUND INFORMATION:</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">State and tribal wildlife agencies may identify collection points (dumpsters) within an identified chronic wasting disease (CWD) management zone for the disposal of hunter-harvested cervid carcasses to remove potentially infected carcasses off the landscape for disposal by an approved method (Gillin & Mawdsley, 2018, chap.14). However, depending on their placement and maintenance these dumpsters could potentially increase the risk of CWD transmission.</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">In several different states, photographic evidence has shown dumpsters in state identified CWD management zones overflowing with deer carcasses and limbs scattered on the land nearby. This could provide an opportunity for scavengers to potentially move infected carcass material to non-infected zones or increase contamination of the ground material around the dumpster’s location.</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">Federal guidance does not explicitly address uniform standards for collection locations for carcasses of free-ranging cervids; however, the United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services Program Standards on CWD outlines procedures for carcass disposal, equipment sanitation, and decontamination of premises for captive cervid facilities.</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">RESOLUTION:</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">The United States Animal Health Association urges the Association of Fish and Wildlife Agencies (AFWA), Wildlife Health Committee to further refine the AFWA Technical Report on Best Management Practices for Prevention, Surveillance, and Management of Chronic Wasting Disease; Chapter 14, Carcass Disposal to address the placement and management of chronic wasting disease carcass disposal dumpsters or other carcass collection containers.</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">Reference:</div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;">1. Gillin, Colin M., and Mawdsley, Jonathan R. (eds.). 2018. AFWA Technical Report on Best Management Practices for Surveillance, Management and Control of Chronic Wasting Disease. Association of Fish and Wildlife Agencies, Washington, D. C. 111 pp. </div><div style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; outline: currentcolor;"><a href="https://www.usaha.org/upload/Resolution/2022/2022_Resolution_30_CWD_Dumpste.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.usaha.org/upload/Resolution/2022/2022_Resolution_30_CWD_Dumpste.pdf</a></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">2023 UPDATED SCIENCE ON TSE PRION TO DATE</div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div><div style="outline: currentcolor;"><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====end</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Nuria Jerez-Garrido1, Sara Canoyra1, Natalia Fernández-Borges1, Alba Marín Moreno1, Sylvie L. Benestad2, Olivier Andreoletti3, Gordon Mitchell4, Aru Balachandran4, Juan María Torres1 and Juan Carlos Espinosa1. 1 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain. 2 Norwegian Veterinary Institute, Ås, Norway. 3 UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France. 4 Canadian Food Inspection Agency, Ottawa, Canada. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) is an infectious prion disease that affects cervids. Various CWD prion strains have been identified in different cervid species from North America and Europe. The properties of the infectious prion strains are influenced by amino acid changes and polymorphisms in the PrP sequences of different cervid species. This study, aimed to assess the ability of a panel of CWD prion isolates from diverse cervid species from North America and Europe to infect bovine species, as well as to investigate the properties of the prion strains following the adaptation to the bovine-PrP context. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: BoPrP-Tg110 mice overexpressing the bovine-PrP sequence were inoculated by intracranial route with a panel of CWD prion isolates from both North America (two white-tailed deer and two elk) and Europe (one reindeer, one moose and one red deer). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show distinct behaviours in the transmission of the CWD isolates to the BoPrP-Tg110 mouse model. Some of these isolates did not transmit even after the second passage. Those able to transmit displayed differences in terms of attack rate, survival times, biochemical properties of brain PrPres, and histopathology. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: MCIN/AEI /10.13039/501100011033 and by European Union NextGeneration EU/PRTR </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: PCI2020-120680-2 ICRAD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====end</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Marina Betancor1, Belén Marín1, Alicia Otero1#, Carlos Hedman1, Antonio Romero2, Tomás Barrio3, Eloisa Sevilla1, Jean Yves Douet3, Alvina Huor3, Juan José Badiola1, Olivier Andréoletti3, Rosa Bolea1.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza, Instituto Agroalimentario de Aragón - IA2, 50013, Zaragoza, Spain. 2 Servicio de Cirugía y Medicina Equina, Hospital Veterinario, Universidad de Zaragoza, 50013, Zaragoza, Spain 3 UMR École Nationale Vétérinaire de Toulouse (ENVT) - Institut National pour l’Agriculture, l’Alimentation et l’Environnement (INRAE) - 1225 Interactions Hôtes Agents Pathogènes (IHAP), 31300 Toulouse, France.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently proved in rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease between 7.2 and 11.3 years post-inoculation and tested for the accumulation of prions by conventional techniques and protein misfolding cyclic amplification (PMCA).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: None of the bovines showed signs compatible with prion disease. In addition, all tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prions was detected during in vitro propagation of brain samples from the inoculated animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: This work was supported financially by the following Spanish and European Interreg grants: Ministerio de Ciencia, Innovación y Universidades (Spanish Government), cofunded by Agencia Estatal de Investigación and the European Union and POCTEFA, which was 65% co-financed by the European Regional Development Fund (ERDF) through the Interreg V-A Spain-France-Andorra program (POCTEFA 2014– 2020).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: n° PID2021-125398OB-I00, EFA148/16 REDPRION</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: The authors would like to thank Sandra Felices and Daniel Romanos for their excellent technical assistance. Authors would also like to acknowledge the use of Servicio General de Apoyo a la Investigación-SAI, Universidad de Zaragoza</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Evolution of Nor98/ Atypical scrapie by iterative propagation in a homologous ovine PrPC context</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sara Canoyra1, Alba Marín-Moreno1, Juan Carlos Espinosa1, Natalia Fernández-Borges1, Nuria Jerez-Garrido1, Sylvie L. Benestad2, Enric Vidal3, Leonor Orge4, Olivier Andreoletti5 and Juan María Torres1.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain. 2Norwegian Veterinary Institute, Ås, Norway. 3Centre de Recerca en Sanitat Animal, Universitat Autònoma de Barcelona (UAB)–Institut de Recerca i Tecnologia Agroalimentàries, Barcelona, Spain. 4Laboratory of Pathology, National Institute for Agrarian and Veterinary Research, Oeiras, Portugal 5UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Nor98/ Atypical scrapie (AS) is a prion disease that causes sporadic casesin sheep and goats. Previous studies have shown that the transmission of AS to otherspecies led to the emergence of new prion strains. In the bovine and porcine PrP, there has been reported the emergence of classical BSE prions (Huor et al., 2019, Espinosa et al., 2009, Marin et. al., 2021) and in the bank vole M109I-PrP context, a classical scrapie-like prion strain emerges(Pirisinu et al., 2022). In this study, we analysed the possible evolution of the AS prion within the same specie by modelling the transmission in a homologous ovine PrP context.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: A panel of AS isolates with different genotypes and geographical origins both from sheep and goats were inoculated in the wild-type transgenic mice model (ARQ-PrP, Aguilar-Calvo et al., 2014).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: The isolates infect the ovine ARQ-PrP mice with homogeneous survival time and a complete attack rate. For several AS isolatesthe transmission led to the emergence of 19kDa (with BSE-like characteristics), 21kDa or atypical prions and mixtures of these agents.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Iterative subpassages of AS isolates into transgenic mice carrying ovine PrP showed an emergence of classical prions during in vivo propagation. This could be caused by the coexistence of strains in the isolate or the evolution of the AS through propagation in the ovine PrP.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These results allow us to hypothesize whether atypical prions might be the origin of prion diversity, where atypical prions tend to acquire classical forms. These results are relevant to control the exposure of farmed animals and humans to AS.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: MCIN/AEI/ 10.13039/501100011033 Grant number: PID2019-105837RB-I00</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div></div></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Monday, November 13, 2023<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">FRIDAY, JULY 07, 2023<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">***> TME, 589.2000 (21 C.F.R. 589.2000), atypical L-BSE, who’s testing MINK for TSE?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: Disease phenotype of classical sheep scrapie is changed upon experimental passage through white-tailed deer</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item Kokemuller, Robyn item MOORE, S.JO - Oak Ridge Institute For Science And Education (ORISE) item Bian, Jifeng item WEST GREENLEE, HEATHER - Iowa State University item Greenlee, Justin</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Submitted to: PLoS Pathogens Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/9/2023 Publication Date: 12/4/2023 Citation: Kokemuller, R., Moore, S., Bian, J., West Greenlee, H.M., Greenlee, J.J. 2023. Disease phenotype of classical sheep scrapie is changed upon experimental passage through white-tailed deer. PLoS Pathogens. https://doi.org/10.1371/journal.ppat.1011815. DOI: https://doi.org/10.1371/journal.ppat.1011815 Interpretive Summary: Transmissible spongiform encephalopathies (TSEs) are a group of fatal diseases caused by the accumulation of misfolded prion protein in the brain. Ruminant species such as sheep, deer, and elk can get prion diseases. In sheep the disease is called scrapie. In deer and elk, the disease is called chronic wasting disease (CWD). The source of CWD is unknown, but one possibility is that scrapie jumped from sheep to deer. When we experimentally exposed white-tailed deer to the sheep scrapie agent, all deer developed scrapie. The purpose of the current experiment was to determine if sheep can get scrapie derived from white-tailed deer. Some sheep developed scrapie after oronasal exposure to the scrapie agent from white-tailed deer. Passage through white-tailed deer results in a scrapie isolate with different strain properties than the original inoculum. The detection of new strain properties was an unexpected result that will be the subject of further studies. These results indicate that sheep could be susceptible to the scrapie agent after passage through deer if exposed to the agent in natural or agricultural settings, which could be a confounding factor to the scrapie eradication program. National and state regulatory and wildlife officials should consider this information when developing plans to reduce or eliminate TSEs.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Technical Abstract: Transmissible spongiform encephalopathy (TSE) agents have strain variations that influence disease phenotype and may affect the potential for interspecies transmission. Since deer and sheep may use the same grazing land, it is important to understand the potential transmission of TSEs between these species. The US scrapie isolate (No.13-7) had a 100% attack rate in white-tailed deer after oronasal challenge. The purpose of this study was to determine if sheep are susceptible to oronasal challenge with the scrapie agent from white-tailed deer. Suffolk lambs of various prion protein genotypes were challenged by the oronasal route with a 10% brain homogenate from scrapie-affected white-tailed deer. Sheep were euthanized and necropsied upon development of clinical signs or at the end of the experiment (72 months post-inoculation). Tissues were tested for PrPSc by enzyme immunoassay, western blot, and immunohistochemistry. The first sheep (2/2) to develop clinical signs at approximately 29 months post-inoculation (MPI) had the VRQ/VRQ genotype. One of the two sheep with the ARQ/ARQ genotype also developed clinical signs at 48 MPI. This is in contrast to the original No.13-7 inoculum that has a faster incubation period in sheep with the ARQ/ARQ genotype compared to sheep of the VRQ/VRQ genotype. The shorter incubation period in VRQ/VRQ sheep than ARQ/ARQ sheep after passage through deer indicates a phenotype change. This is important because scrapie infected deer could transmit disease to sheep resulting in new scrapie strain properties. This work raises the concern that scrapie infected deer could serve as a confounding factor to scrapie eradication programs as the scrapie agent from deer is transmissible to sheep by the oronasal route.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=405929" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=405929</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item Cassmann, Eric item QI, XU - Case Western Reserve University (CWRU) item KONG, QINGZHONG - Case Western Reserve University (CWRU) item Greenlee, Justin</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 3/15/2023 Publication Date: 5/30/2023 Citation: Cassmann, E.D., Qi, X., Kong, Q., Greenlee, J.J. 2023. The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons (abstract). Meeting Abstract. 4th International Chronic Wasting Disease Symposium, May 30-June 3, 2023, Denver, Colorado. Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Technical Abstract: The aim of this study was to evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer (WTD) host. Pooled brain (GG96) from CWD positive deer was used to intracranially inoculate two WTD and one raccoon. Brain homogenates (10% w/v) from the raccoon and the WTD were used to intracranially inoculate transgenic mice (Tg40h) expressing the methionine 109 human prion protein. Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue. Tg40h mice inoculated with the raccoon passaged CWD agent from WTD exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPSc was detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPSc also was detected in brain tissue by western blot and immunohistochemistry. No PrPSc was detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from WTD did not have detectable PrPSc using conventional immunoassay techniques. These results demonstrated that the host range of the CWD agent from WTD was expanded in our experimental model after one passage through raccoons.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=400777</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">2023</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">34 Scientific Commission/September 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. Atypical BSE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*Corresponding and Presenting Author: <span dir="ltr" style="outline: currentcolor;">waqas.tahir@inspection.gc.ca</span></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Presentation Type: Oral Presentation</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant Number: ALMA/APRI: <span dir="ltr" style="outline: currentcolor;">201400006</span>, HC 414250</div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="font-family: arial; outline: currentcolor;" /></div></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Approximately 16.6 months post-inoculation, Steer 6 (<span dir="ltr" style="outline: currentcolor;">EK211</span> L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (<span dir="ltr" style="outline: currentcolor;">EK211</span> L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Further study of L-BSE in <span dir="ltr" style="outline: currentcolor;">EK211</span> cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases</div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">=====end</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Affiliations expand</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PMID: <span dir="ltr" style="outline: currentcolor;">21266763</span></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see full text;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">2023 PRION CONFERENCE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Experimental transmission of the chronic wasting disease agent to swine after oral or intracranial inoculation</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Running Title: The chronic wasting disease agent transmits to swine</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">S. Jo Moore1,2 , M. Heather West Greenlee3 , Naveen Kondru3 , Sireesha Manne3 , Jodi D. Smith1,# , Robert A. Kunkle1 , Anumantha Kanthasamy3 , Justin J. Greenlee1*</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, Iowa, United States of America</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, United States of America</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, Iowa, United States of America</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Current Address: Department of Veterinary Pathology, Iowa State University College of Veterinary Medicine, Ames, Iowa, United States of America * Corresponding author Email: justin.greenlee@ars.usda.gov</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This is a work of the U.S. Government and is not subject to copyright protection in the United States. Foreign copyrights may apply.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> on July 27, 2017 by guest http://jvi.asm.org/ Downloaded from</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is a naturally occurring, fatal neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation . Crossbred piglets were assigned to one of three groups: intracranially inoculated (n=20), orally inoculated (n=19), or non -inoculated (n=9). At approximately the age at which commercial pigs reach market weight, half of the pigs in each group were culled (‘market weight’ groups). The remaining pigs (‘aged’ groups) were allowed to incubate for up to 73 months post inoculation (MPI ). Tissues collected at necropsy were examined for disease -associated prion protein (PrPSc) by western blotting (WB), antigen -capture immunoassay (EIA), immunohistochemistry (IHC) and in vitro real -time quaking induced conversion (RT -QuIC). Brain samples from selected pigs were also bioassayed in mice expressing porcine prion protein. Four intracranially inoculated aged pigs and one orally inoculated aged pig were positive by EIA, IHC and/or WB. Using RT -QuIC, PrPSc was detected in lymphoid and/or brain tissue from one or more pigs in each inoculated group. Bioassay was positive in 4 out of 5 pigs assayed.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This study demonstrates that pigs can support low-level amplification of CWD prions, although the species barrier to CWD infection is relatively high. However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the case of feral pigs, exposure to the agent of CWD through scavenging of CWD-affected cervid carcasses or through consumption of prion contaminated plants or soil could allow feral pigs to serve as reservoirs of CWD infectivity. The range and numbers of feral pigs is predicted to continue to increase due to the ability of pigs to adapt to many climates, reproduce year-round, and survive on a varied diet (55 ). The range of CWD-affected cervids also continues to spread, increasing the likelihood of overlap of ranges of feral pigs and CWD -affected environments.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We demonstrate here that PrPSc accumulates in lymphoid tissues from pigs inoculated intracranially or orally with the CWD agent, and can be detected as early as 6 months after inoculation. Clinical disease suggestive of prion disease developed only in a single pig after a long (64 months) incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. However, the low amounts of PrPSc detected in the study pigs combined with the low attack rates in Tg002 mice suggest that there is a relatively strong species barrier to CWD prions in pigs.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://journals.asm.org/doi/10.1128/jvi.00926-17" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://journals.asm.org/doi/10.1128/jvi.00926-17</a></div></div><br style="outline: currentcolor;" /></div></div></div></div></div></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">cwd scrapie pigs oral routes </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CONFIDENTIAL</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">LINE TO TAKE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a> </div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: Tue, 9 Jan 2001 16:49:00 -0800</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: "Terry S. Singeltary Sr."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Reply-To: Bovine Spongiform Encephalopathy</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: BSE-L@uni-karlsruhe.de </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div></div></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (<span dir="ltr" style="outline: currentcolor;">Tg338</span> and <span dir="ltr" style="outline: currentcolor;">Tg501</span>). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: No evidence of transmission was found on a first passage in <span dir="ltr" style="outline: currentcolor;">Tg338</span> nor Tg501ovinized mice, but on second passage, <span dir="ltr" style="outline: currentcolor;">4/10</span> <span dir="ltr" style="outline: currentcolor;">Tg338</span> mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and <span dir="ltr" style="outline: currentcolor;">1/12</span> <span dir="ltr" style="outline: currentcolor;">Tg501</span> mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***thus questioning the origin of human sporadic cases. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2015 CONFERENCE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION <span dir="ltr" style="outline: currentcolor;">2016 TOKYO</span></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Saturday, April 23, 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SCRAPIE <span dir="ltr" style="outline: currentcolor;">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr" style="outline: currentcolor;">1933-6896</span> 1933-690X </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span dir="ltr" style="outline: currentcolor;">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Tuesday, December 16, 2014 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Evidence for zoonotic potential of ovine scrapie prions </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,<span dir="ltr" style="outline: currentcolor;">1, Naima Aron</span>,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the humanprion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject terms: Biological sciences• Medical research At a glance</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">why do we not want to do TSE transmission studies on chimpanzees $ 5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip... R. BRADLEY </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1: J Infect Dis 1980 Aug;142(2):205-8 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: <span dir="ltr" style="outline: currentcolor;">6997404</span></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Nature. 1972 Mar 10;236(5341):73-4. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://scrapie-usa.blogspot.com/</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://nor-98.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://nor-98.blogspot.com/</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://scrapie-usa.blogspot.com/</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://nor-98.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://nor-98.blogspot.com/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH and USDA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">end... <br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Fortuitous generation of a zoonotic cervid prion strain </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (<span dir="ltr" style="outline: currentcolor;">Tg12</span>), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the <span dir="ltr" style="outline: currentcolor;">Tg12</span> mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (<span dir="ltr" style="outline: currentcolor;">Tg12</span>) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the <span dir="ltr" style="outline: currentcolor;">Tg12</span> mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively</div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">"Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time."<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A probable diagnostic marker for CWD infection in humans </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Xu Qi, Liuting Qing, Manuel Camacho, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Multiple in vitro CWD-seeded human PrP conversion experiments and some animal model studies indicate that the species barrier for CWD to human transmission can be overcome, but whether CWD prion can infect humans in real life remains controversial. The very limited understanding on the likely features of CWD infection in humans and the lack of a reliable diagnostic marker for identification of acquired human CWD cases contribute to this uncertainty. We aim to stablish such a reliable diagnostic marker for CWD infections in humans should they occur. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: A couple of PrPSc-positive spleens were identified from humanized transgenic mice inoculated with either CWD or sCJDMM1. Prions in these spleens were compared by bioassays in cervidized or humanized transgenic mice. A couple of PrPSc-positive spleens from UK sCJDMM1 patients were also examined similarly as controls with no exposure to CWD. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: We have detected two prion-positive spleens in humanized transgenic mice inoculated with some CWD isolates. Such experimentally generated splenic “humanized” CWD prions (termed eHuCWDsp) appear indistinguishable from prions in the brain of sCJDMM1 patients on Western blot. We compared eHuCWDsp with prions in the spleen from humanized mice infected with sCJDMM1 (termed sCJDMM1sp) by bioassays in cervidized or humanized transgenic mice. Significantly, we found that eHuCWDsp can efficiently infect not only the humanized mice but also cervidized transgenic mice, and cervidized mice infected by eHuCWDsp produced PrPSc and brain pathology that are practically identical to those of CWD-infected cervidized mice. In contrast, sCJDMM1sp, similar to prions from sCJDMM1 patient brains, is poorly transmissible in the cervidized mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data demonstrate that high transmissibility with CWD features of splenic prions in cervidized transgenic mice is unique to acquired human CWD prions, and it may serve as a reliable marker to identify the first acquired human CWD cases. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">=====end </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">The detection and decontamination of chronic wasting disease prions during venison processing </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Marissa S. Milstein1,2, Marc D. Schwabenlander1,2, Sarah C. Gresch1,2, Manci Li1,2, Stuart Lichtenberg1,2, Rachel Shoemaker1,2, Gage R. Rowden1,2, Jason C. Bartz2,3 , Tiffany M. Wolf2,4, Peter A. Larsen1,2 Presenting author: Tiffany M. Wolf 1 Department of Veterinary and Biomedical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 2 Minnesota Center for Prion Research and Outreach, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 3 Department of Medical Microbiology and Immunology, School of Medicine, Creighton University, Omaha, Nebraska, USA 4 Department of Veterinary Population Medicine, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: There is a growing concern that chronic wasting disease (CWD) prions in venison pose a risk to human health. CWD prions accumulate in infected deer tissues that commonly enter the human food chain through meat processing and consumption. The United States (US) Food and Drug Administration and US Department of Agriculture now formally consider CWD-positive venison unfit for human and animal consumption. Yet, the degree to which prion contamination occurs during routine venison processing is unknown. Here, we use environmental surface swab methods to: a) experimentally test meat processing equipment (i.e., stainless steel knives and polyethylene cutting boards) before and after processing CWD-positive venison and b) test the efficacy of five different disinfectant types (i.e., Dawn dish soap, Virkon-S, Briotech, 10% bleach, and 40% bleach) to determine prion decontamination efficacy. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: We used a real-time quaking-induced conversion (RT-QuIC) assay to determine CWD infection status of venison and to detect CWD prions in the swabs. We collected three swabs per surface and ran eight technical replicates on RT-QuIC. <br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: CWD prions were detected on all cutting boards (n= 3; replicates= 8/8, 8/8, 8/8 and knives (n= 3; replicates= 8/8, 8/8, 8/8) used in processing CWD-positive venison, but not on those used for CWD-negative venison. After processing CWD-positive venison, allowing the surfaces to dry, and washing the cutting board with Dawn dish soap, we detected CWD prions on the cutting board surface (n= 3; replicates= 8/8, 8/8, 8/8) but not on the knife (n= 3, replicates = 0/8, 0/8, 0/8). Similar patterns were observed with Briotech (cutting board: n= 3; replicates= 7/8, 1/8, 0/8; knife: n= 3; replicates = 0/8, 0/8, 0/8). We did not detect CWD prions on the knives or cutting boards after disinfecting with Virkon-S, 10% bleach, and 40% bleach. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These preliminary results suggest that Dawn dish soap and Briotech do not reliably decontaminate CWD prions from these surfaces. Our data suggest that Virkon-S and various bleach concentrations are more effective in reducing prion contamination of meat processing surfaces; however, surface type may also influence the ability of prions to adsorb to surfaces, preventing complete decontamination. Our results will directly inform best practices to prevent the introduction of CWD prions into the human food chain during venison processing. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: Funding was provided by the Minnesota Environment and Natural Resources Trust Fund as recommended by the Legislative-Citizen Commission on Minnesota Resources (LCCMR), the Rapid Agriculture Response Fund (#95385/RR257), and the Michigan Department of Natural Resources. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Theme: Animal prion diseases</div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">=====end</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://https//prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div></div></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Prion 2023 Detection of CWD prions in plants collected from white-tailed deer farms </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Francisca Bravo Risi1,3, Paulina Soto1,3, Yumeng Huang1 , Tracy A. Nichols4 & Rodrigo Morales1,2* Affiliations: 1 Department of Neurology, The University of Texas Health Science Center at Houston, <span dir="ltr" style="outline: currentcolor;">6431 Fannin St.,Houston, TX 77030, USA</span>. 2 Centro Integrativo de Biologia y Quimica Aplicada (CIBQA), Universidad Bernardo O’Higgins, Santiago, Chile. 3 Universidad Bernardo O’Higgins, Doctorado en Ciencias con Mención en Materiales Funcionales, <span dir="ltr" style="outline: currentcolor;">General Gana 1702, Santiago, Chile</span>. 4 Veterinary Services Cervid Health Program, United States Department of Agriculture, Animal and Plant Health Inspection Service, Fort Collins, Colorado, </div><div style="outline: currentcolor;">USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) affects both farmed and free-ranging cervids. Transmission of CWD is thought to occur by direct animal-to-animal contact and by exposure to contaminated environmental fomites. CWD-prion seeding activity has been detected in natural and experimentally-contaminated environmental samples including mineral licks, water sources, dust, manmade surfaces, soils, and plants. Importantly, prion infectivity in some of these samples has been proven. However, whether CWD exposed plants carry prion levels relevant to sustain infectivity has not been tested. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The aim of this study is to explore if plants collected in a CWD contaminated facility are able to spread prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: In this study, we optimized the detection of CWD-prions in plants using the protein misfolding cyclic amplification (PMCA). We compared NaPTA pretreatment and direct spiking of the sample into the PMCA reactions. After achieving technical optimizations, we screened multiple plant specimens collected from white-tailed deer breeding facilities displaying variable CWD prevalence. Plants from a site displaying the highest CWD prevalence were tested for infectivity in meadow voles, a co-existing animal species that feed from grass plants. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results demonstrated that CWD-prion detection in plants was optimal when samples were pre-treated with a NaPTA-based protocol. Our screening results showed positive PMCA activity for specimens collected from the farm with the highest CWD prevalence. Although meadow voles were highly susceptible to CWD-prions by intra-cranial administration, consumption of contaminated grass did not induce prion infection in these rodents. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Pre-PMCA treatment with NaPTA increase the detection of CWD-prionsin vitro in plant specimens. Although the detection of CWD in naturally contaminated vegetation was possible, the amount of prion was apparently low. This was demonstrated by the lack of transmission to meadow voles exposed to these plants. These findings further contribute to understand how CWD prions interact with multiple environmental elements. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH and USDA Grant number: 1R01AI132695 and AP20VSSPRS00C143<br style="outline: currentcolor;" /></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">=====end</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="font-family: arial; outline: currentcolor;"><div style="outline: currentcolor;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, <span dir="ltr" style="outline: currentcolor;">18 Route du Panorama, 92265 Fontenay-aux-Roses, France</span>.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: currentcolor;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: currentcolor;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: currentcolor;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: currentcolor;">tg650</span> with fecal homogenates. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Published</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">22 August 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Accepted: 7 August 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full text;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH and USDA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">end... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2023 CONTINUED; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="font-family: arial; outline: currentcolor;"><div style="outline: currentcolor;">''Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">31 TAC §§65.82, 65.85, 65.88</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Our results show positive prion detection in all products.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">FRIDAY, JANUARY 05, 2024 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Texas CWD Cases Mount, 624 documented cases statewide, with 181 cases reported in 2023 alone<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2024/01/texas-cwd-cases-mount-624-documented.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2024/01/texas-cwd-cases-mount-624-documented.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">THURSDAY, DECEMBER 7, 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic Wasting Disease CWD TSE Prion Cervid Update By State December 2023 (Long Version)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(Short Version)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">spontaneous TSE Prion in any species documented?<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">not a documented species to date, has had a naturally occurring spontaneous TSE Prion disease been documented. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">It is interesting to consider the potential virulence in common domestic species in which spontaneous prion diseases have never been reported. <br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3710336/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3710336/</a><span style="color: #212121; font-family: Cambria, stixgeneral, serif; font-size: 20px; outline: currentcolor;"><br style="outline: currentcolor;" /></span></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div dir="ltr" style="outline: currentcolor;">However, the crucial prediction that a disease-associated PrP mutation can, in fact, spontaneously generate infectivity has never been experimentally demonstrated.<br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2775465/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2775465/</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">NOW, considering all the factors, IF there was ever a documented case of atypical TSE Prion in any species, especially cattle, cervid, sheep, and so on, that would be the industries worst nightmare i.e. BSE feed ban, surveillance, regulations, and SRM removals etc., would have to therefore be indefinite. </div></div><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="color: #26282a; outline: currentcolor;"><span face="Helvetica, Arial, sans-serif" style="outline: currentcolor;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd, PLUS, SPORADIC CJD HAS NOW BEEN LINKED TO ATYPICAL AND TYPICAL BSE, SCRAPIE, AND NOW CWD. ...terry</span><br clear="none" style="outline: currentcolor;" /></div><div dir="ltr" style="color: #26282a; outline: currentcolor;"><span face="Helvetica, Arial, sans-serif" style="outline: currentcolor;"><br clear="none" style="outline: currentcolor;" /></span></div><div dir="ltr" style="color: #26282a; outline: currentcolor;"><span face="Helvetica Neue, Helvetica, Arial, sans-serif" style="outline: currentcolor;">NOW, again, think cwd, zoonosis to humans as some strain of sporadic cjd exposure, or what about hunters field dressing their deer, cervid, knives, utensils, cutting boards, etc., iatrogenic spread, what if?</span></div></div></div></div></div></div></div></div></div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">terry</div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-66255741526494684352023-12-24T16:15:00.001-06:002023-12-24T16:15:16.013-06:00France Sporadic CJD Rising, Typical and Atypical, BSE, Scrapie, and CWD, Zoonosis, what if? <p><span style="background-color: white; font-family: arial; font-size: 16px;">France Sporadic CJD Rising, Typical and Atypical, BSE, Scrapie, and CWD, Zoonosis, what if? </span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;">France CJD cases continue to rise, what about zoonotic TSE to humans?</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">France is having a serious problem with continued spontaneous atypical BSE cases?</span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">France Chronic Wasting Disease CWD TSE Prion in cervid?</span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">France, what about their typical and atypical Scrapie cases?</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">France, what about TSE Prion tainted feed for livestock?</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">France, sCJD, Iatrogenic CJD cases, misdiagnosis, what if?</span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">Seems to me, if atypical BSE was as spontaneous and sporadic as they claim it to be, it would be the same across the board, however, this hypothesis is crumbling, imo.</span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">European Food Safety Authority (EFSA)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">First published: 28 November 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.2903/j.efsa.2023.8384" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.2903/j.efsa.2023.8384</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 19 October 2023 Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">with one case of H-BSE in France. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2023.8384" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2023.8384</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annual Report of the Scientific Network on BSE-TSE 2022 European Food Safety Authority (EFSA) First published: 10 November 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">with two cases of H-BSE in France and Spain, and four L-BSE in France (2), Germany and Spain</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/abs/10.2903/sp.efsa.2022.EN-7656" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/abs/10.2903/sp.efsa.2022.EN-7656</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/7655" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/7655</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: 30 November 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 25 October 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">with three cases of H‐BSE in France, Ireland and Spain, and two L‐BSE in France and Switzerland.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/6934" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/6934</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: 17 November 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 16 October 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Six cases of H‐BSE were reported by France (4) and Spain (2), and 1 L‐BSE by Poland. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/6303" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/6303</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2018</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: 3 December 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 15 November 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Three atypical BSE cases (2 L‐type/1 H‐type) were reported by France. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/5925" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/5925</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSEs) in 2017</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: 29 November 2018</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 6 November 2018</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Six atypical BSE cases were reported by three different MSs: Spain 1 H‐BSE/2 L‐BSE; France 1 H‐BSE/1 L‐BSE; and Ireland 1 L‐BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/5492" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/5492</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published: 30 November 2017</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approved: 30 October 2017</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For the first time, the United Kingdom did not report any case of bovine spongiform encephalopathy (BSE), whereas France reported one classical and three atypical cases (H), and Spain one atypical case (H). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.efsa.europa.eu/en/efsajournal/pub/5069" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.efsa.europa.eu/en/efsajournal/pub/5069</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">H-BSE was first reported in France7, and has been subsequently detected in several European countries and North America5,6.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep22753" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep22753</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Atypical Bovine Spongiform Encephalopathies, France, 2001–2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In France, through exhaustive active surveillance, ≈17.1 million adult cattle were tested for bovine spongiform encephalopathy from July 2001 through July 2007; ≈3.6 million were >8 years of age. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our retrospective Western blot study of all 645 confirmed cases found that 7 were H-type and 6 were L-type.</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2600212/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2600212/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Thursday, March 24, 2016 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://bovineprp.blogspot.com/2016/03/france-confirms-bovine-spongiform.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***atypical spontaneous BSE in France LOL***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">If you Compare France to other Countries with atypical BSE, in my opinion, you cannot explain this with ‘spontaneous’.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 1: Number of Atypical BSE cases reported by EU Member States in the period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE databases on 1 July 2014). By 2015, these data might be more comprehensive following a request from the European Commission to Member States for re-testing and retrospective classification of all positive bovine isolates in the EU in the years 2003–2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Country 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a) 2014(a) Total</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">H-BSE Austria 1 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> France(b) 1 2 3 1 2 2 2 2 15</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Germany 1 1 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland 1 1 2 1 5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Netherlands 1 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Poland 1 1 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Portugal 1 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Spain 1 1 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sweden 1 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">United Kingdom 1 1 1 1 1 5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">L-BSE Austria 1 1 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Denmark 1 1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France(b) 1 1 1 1 2 1 3 2 1 1 14</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Germany 1 1 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Italy 1 1 1 1 1 5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Netherlands 1 1 1 3</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Poland 1 2 2 1 2 1 2 1 12</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Spain 2 2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">United Kingdom 1 1 1 1 4</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total Atypical cases (H + L)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2 8 6 5 4 5 8 5 7 8 8 7 5 2 80</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(a): Data for 2013-2014 are incomplete and may not include all cases/countries reported.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(b): France has performed extensive retrospective testing to classify BSE cases, which is probably the explanation for the higher number of Atypical BSE cases reported in this country.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The number of Atypical BSE cases detected in countries that have already identified them seems to be similar from year to year. In France, a retrospective study of all TSE-positive cattle identified through the compulsory EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively, which increased to 1.9 and 1.7 cases per million, respectively, in tested animals over eight years old (Biacabe et al., 2008). No comprehensive study on the prevalence of Atypical BSE cases has yet been carried out in other EU Member States. All cases of Atypical BSE reported in the EU BSE databases have been identified by active surveillance testing (59 % in fallen stock, 38 % in healthy slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported in animals over eight years of age, with the exception of two cases (one H-BSE and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et al., 2012).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.efsa.europa.eu/sites/default/files/scientific_output/files/main_documents/3798.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.efsa.europa.eu/sites/default/files/scientific_output/files/main_documents/3798.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html</a> </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, OCTOBER 5, 2014 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France stops BSE testing for Mad Cow Disease France stops BSE testing 0 79 Process Management</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The France government has decided to stop testing cattle on mad cow disease (BSE). The measure applies to all animals born after 2002. This is what the French Minister of Agriculture: Stéphane Le Foll communicated during the Sommet de 'Elevage in Cournon, near Clermont-Ferrand.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research indicates that the first probable infections of BSE in cows occurred during the 1970's with two cases of BSE being identified in 1986. BSE possibly originated as a result of feeding cattle meat-and-bone meal that contained BSE-infected products from a spontaneously occurring case of BSE or scrapie-infected sheep products. The major epidemic in the UK in the early 90s led to the decision to introduce compulsory BSE testing of cattle in 2001.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The remaining tests cost € 40 million annually, but show little or no more positive results for BSE. Moreover, the organs that can be infected with BSE are already standardly removed. 'Consumption of beef is therefore without any risk,' said the minister.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">by Ruud Peijs 2 Oct 2014</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.allaboutfeed.net/animal-feed/feed-processing/france-stops-bse-testing/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.allaboutfeed.net/animal-feed/feed-processing/france-stops-bse-testing/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2014/10/france-stops-bse-testing-for-mad-cow.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted May 2, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A cow in northern France has been confirmed to have bovine spongiform encephalopathy, according to the World Organisation for Animal Health (OIE).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The cow had developed partial paralysis and was euthanized March 1, a March 25 OIE report states.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE is a fatal neurologic prion disease with a typical incubation period of four to five years. The cow in France was almost 5 years old.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The affected cow had the classic form of BSE, which is most often associated with feed containing neurologic tissue from infected animals. It is distinct from atypical BSE, which may develop spontaneously, according to information from the U.S. Centers for Disease Control and Prevention.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Investigators were trying to identify the source of infection and other animals at risk for BSE at the time the report was published.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.avma.org/News/JAVMANews/Pages/160515n.aspx" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.avma.org/News/JAVMANews/Pages/160515n.aspx</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The affected bovine, a Salers female born on April, 8th 2011, showed paresis and was euthanized on March, 1st 2016. Samples made on March, 4th 2016 during rendering were analyzed at the Department Laboratory of La Somme. The rapid test proved positive on March, 8th 2016 and the samples were then sent for further analysis to the National Reference Laboratory, ANSES, which confirmed a case of classical BSE on March, 21st 2016. The European Union Reference Laboratory confirmed those results on the basis of documentation on March, 23rd 2016.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.oie.int/wahis_2/public/wahid.php/Reviewreport/Review?page_refer=MapFullEventReport&reportid=19974" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.oie.int/wahis_2/public/wahid.php/Reviewreport/Review?page_refer=MapFullEventReport&reportid=19974</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide*(excluding the United Kingdom) Country/Year</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1989 - 2012</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">France 0 0 5 0 1 4 3 12 6 18 31a 162d 274e 239f 137g 54h 31 8 9 8 10 5 3 </span><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">* Cases are shown by year of confirmation. ... Not available</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">a</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Canada: 1 case diagnosed in Canada in May 2003 + 1 case diagnosed in the United States of America in December 2003 and confirmed as having been imported from Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Finland: date of confirmation of the case: 7 December 2001.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France: includes 1 imported case (confirmed on 13 August 1999).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland: includes imported cases: 5 in 1989, 1 in 1990, 2 in 1991 and 1992, 1 in 1994 and 1995.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Italy: includes 2 imported cases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Liechtenstein : date of the last confirmation of a case: 30 September 1998.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Portugal: includes 1 imported case.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Slovenia: includes 1 imported case.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">b</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Imported case(s).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">c</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Czech Republic - Data as of 30 June 2012. Ireland - Data as of 30 June 2012. Liechtenstein - Data as of 30 June 2012. Luxembourg - Data as of 30 September 2012. Poland - Includes 1 atypical BSE case (L-type). Data as of 6 August 2012. Slovenia - Data as of 30 June 2012. Sweden - Data as of 30 June 2012. Switzerland - Atypical imported BSE case. Data as of 12 March 2012. United States of America - Atypical BSE case. Data as of 26 April 2012.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">d</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France year 2000 - Clinical cases = 102. Cases detected within the framework of the research programme launched on 8 June 2000 = 60. Ireland year 2000 - Clinical cases = 138. Cases identified by active surveillance of at risk cattle populations = 7. Cases identified by examination of depopulated BSE positive herds, birth cohorts and progeny animals = 4. Switzerland year 2000 - Clinical cases = 17. Cases detected within the framework of the investigation programme = 16.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">e</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France year 2001 - Clinical cases = 91. Cases detected at rendering (bovines at risk) = 100 (out of 139,500 bovines tested). Cases detected as result of routine screening at the abattoir = 83 (out of 2,373,000 bovines tested). Ireland year 2001 - Clinical cases = 123. Cases identified by systematic active surveillance of all adult bovines = 119. Cases identified by examination of depopulated BSE positive herds, birth cohorts and progeny animals = 4. Japan year 2001 - Clinical cases = 1. Cases detected as result of screening at the abattoir = 2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">f</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France year 2002 - Clinical cases = 41. Cases detected at rendering (bovines at risk) = 124 (out of 274,143 bovines tested). Cases detected as result of systematic screening at the abattoir = 74 (out of 2,915,103 bovines tested). The active BSE surveillance programmes implemented in France in 2002 led to routine examination of cattle aged over 24 months, which were slaughtered for consumption purposes, were euthanised or died due to other reasons. Ireland year 2002 - Clinical cases = 108. Cases detected by the active surveillance programme = 221. Cases identified by examination of depopulated BSE positive herds, birth cohorts and progeny animals = 4. Poland year 2002 - Clinical cases = 1. Cases detected as result of routine screening at the abattoir (cattle over 30 months) = 3.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">g</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France year 2003 - Clinical cases = 13. Cases detected at rendering (bovines at risk) = 87. Cases detected as result of systematic screening at the abattoir = 37. Japan year 2003 - The 9th case was a bullock aged 21 months. Ireland year 2003 - Clinical cases = 41. Cases detected by the active surveillance programme = 140. Switzerland year 2003 - Clinical cases: 8. Cases detected within the framework of the official surveillance programme: 11. Cases detected through voluntary testing following routine slaughter: 2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">h</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France year 2004 - Clinical cases = 8. Cases detected at rendering (bovines at risk) = 29. Cases detected as result of systematic screening at the abattoir = 17. Ireland year 2004 - Clinical cases = 31. Cases detected by the active surveillance programme = 94. Cases identified by examination of depopulated BSE positive herds, birth cohorts and progeny animals = 1.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">i</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland year 2005 - Cases detected by the passive surveillance programme = 13. Cases detected by the active surveillance programme = 56. Switzerland year 2005 - Cases detected by the passive surveillance programme = 1. Cases detected within the framework of the official surveillance programme: 1. Cases detected through voluntary testing following routine slaughter = 1.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">j</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland year 2006 - Cases detected by the passive surveillance programme = 5. Cases detected by the active surveillance programme = 36.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">k</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland year 2007 - Cases detected by the passive surveillance programme = 5. Cases detected by the active surveillance programme = 20.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">l</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland year 2008 - Cases detected by the passive surveillance programme = 3. Cases detected by the active surveillance programme = 20.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">m</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Netherlands - Atypical BSE case (L-type) Switzerland - Atypical BSE cases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20121022075241/http://www.oie.int/animal-health-in-the-world/bse-specific-data/number-of-reported-cases-worldwide-excluding-the-united-kingdom/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20121022075241/http://www.oie.int/animal-health-in-the-world/bse-specific-data/number-of-reported-cases-worldwide-excluding-the-united-kingdom/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20110612124319/http://esbinfo.agriculture.gouv.fr/index.php3?forcer_lang=true&lang=fr" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20110612124319/http://esbinfo.agriculture.gouv.fr/index.php3?forcer_lang=true&lang=fr</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Emerg Infect Dis. 2005 Aug; 11(8): 1274–1279. doi: 10.3201/eid1108.041223</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMCID: PMC3320489PMID: 16102318</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sheep Feed and Scrapie, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sandrine Philippe,* Christian Ducrot,† Pascal Roy,‡ Laurent Remontet,‡ Nathalie Jarrige,* and Didier Calavas corresponding author*</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Proprietary concentrates and milk replacers were linked to risk for scrapie.<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords: Scrapie, Sheep, Transmission, Epidemiology, Case-Control studies, Risk Factors, France, Transmissible Spongiform Encephalopathy Go to:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Scrapie is a small ruminant, transmissible spongiform encephalopathy (TSE). Although in the past scrapie has not been considered a zoonosis, the emergence of bovine spongiform encephalopathy, transmissible to humans and experimentally to sheep, indicates that risk exists for small ruminant TSEs in humans. To identify the risk factors for introducing scrapie into sheep flocks, a case-control study was conducted in France from 1999 to 2000. Ninety-four case and 350 control flocks were matched by location and main breed. Three main hypotheses were tested: direct contact between flocks, indirect environmental contact, and foodborne risk. Statistical analysis was performed by using adjusted generalized linear models with the complementary log-log link function, considering flock size as an offset. A notable effect of using proprietary concentrates and milk replacers was observed. The risk was heterogeneous among feed factories. Contacts between flocks were not shown to be a risk factor.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords: Scrapie, Sheep, Transmission, Epidemiology, Case-Control studies, Risk Factors, France, Transmissible Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Scrapie is a transmissible spongiform encephalopathy (TSE) affecting sheep and goats (1), as is Creutzfeldt-Jakob disease (CJD) in humans or bovine spongiform encephalopathy (BSE) in cattle. Moreover, scrapie is contagious in natural conditions (2). Though genetic determinism is a major feature of scrapie, the infectious agent is nonetheless needed for the disease to develop (3,4).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Known to exist for centuries, scrapie was thought to be a possible origin of BSE, although this hypothesis has not yet been verified. Sheep and goats can be experimentally infected with BSE, resulting in a disease that is impossible to distinguish from natural scrapie (5). Since BSE is implicated in the emergence of variant CJD (6,7), the existence of BSE in small ruminants poses a further risk for human health. Scrapie has become a public health challenge, and its propagation must be stopped; therefore, the risk factors for the introduction of scrapie in sheep must be understood.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In sheep infected with scrapie, the infectious agent is widely distributed in the organism. In particular, the gut-associated lymphoid tissues and the placenta are considered highly important in spreading the disease (8) and can contaminate the environment (9). Because feed is considered to be the main, if not the only, contamination source of BSE in cattle (10,11), it can also be presumed to be a potential risk factor for scrapie in sheep.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A case-control study of infected and scrapie-free flocks was conducted to identify risk factors for introducing scrapie into sheep flocks in France. Various risk factors hypotheses were tested from the most plausible to the weakest.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Study Design</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A case-control study of infected and scrapie–free flocks was designed (Appendix). A flock was defined as having at least 20 adult ewes. To consider the heterogeneity of exposure to scrapie risk, cases and controls were matched according to main sheep breed and location. A "case" was any flock having ≥1 animal that had been shown as scrapie-positive by the French surveillance network from January 1996 to July 2000 (12). Four frequency-matched control flocks were randomly selected from the sheep flocks in which scrapie had never been reported. Flocks that did not meet this criterion were excluded.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The suspected risk factors were grouped into 3 categories corresponding to the main working hypotheses of scrapie dissemination. The first category covered risks for transmission by direct contact between flocks and indirectly through the environment. The second category covered foodborne risks. The third category covered other environmental dissemination risks such as equipment sharing between farms or transmission through hay mites. Table 1describes the 22 potential risk factors studied.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Data Collection</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Information was collected by using a preestablished questionnaire to interview farmers and analyzing farm records. Questions related to potential risk factors covered the 4-year period preceding detection of the first clinical case of scrapie in case flocks and the 4-year period preceding the interview for controls. Additionally, information regarding potential confounding factors including flock size, production type (dairy, meat, or mixed), and intensification level of the flock production was recorded (Table 2). Interviews were conducted from May 1999 to July 2000 with 453 flock owners (98 cases and 355 controls). Nine flocks were excluded because they did not meet the inclusion criteria. A total of 444 flocks (94 cases and 350 controls) were included in the study. Data were encoded and then stored in an Access database (Microsoft Access 97 SR-2, Microsoft Corporation, Redmond, WA, USA).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Study Sample</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The flocks were mainly located in 2 departments (Pyrénées Atlantiques, n = 267/444, Aveyron n = 51/444). The others were widely distributed throughout metropolitan France. Ten mixed breeds and 23 pure breeds were included in the study. The flocks were mainly specialized in 1 type of production (66% in dairy production, 32% in meat production) (Table 2). The flock size ranged from 21 to 1,787 ewes (mean 274, SD 198).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Data analysis was conducted in 2 steps by using statistical models adjusted for the 2 matching factors through the corresponding cross-variable "strata" (main breed and location) treated as a stratification variable (13). First, to identify the confounding factors to be further analyzed (14), a log-linear model considered 5 factors, including flock size (number of ewes), production type, intensification level of the flock production as potential confounding factors, flock status, and strata. The model introduced the main effect of these 5 factors with all second interaction terms. Flock size was the only potential confounding factor notably associated with the flock status (Table 2). Second, to assess associations between flock status and risk factors, a generalized linear model for binary outcome was set up with the complementary log-log link function (Clog-log model) (14) (Appendix). This model considered the flock size by using the logarithm of the flock size as an offset (15,16). All exposures were considered as binary, and the absence of exposition was the reference modality for each risk factor. Factors notably associated with the flock status at 20% level through univariate analysis (Table 1) were selected for subsequent multivariate analyses. The univariate analysis consisted of the construction of a Clog-log model for each risk factor; strata were systematically introduced as covariate. Furthermore, 2 distinct multivariate models were applied to consider colinearity between feed type and feed factories in the foodborne risk study. The first model (multivariate Clog-log 1) analyzed feed types without regard to factories, whereas the second one (multivariate Clog-log 2) evaluated the risk according to the feed factories that produced milk replacers and proprietary concentrates. Regarding the proprietary feed factories, only the purchase of milk replacers and proprietary concentrates at factory 1 and the purchase of proprietary concentrates at factory 2 occurred frequently enough to be studied separately. Statistical software Splus (S-Plus 2000 Professional Release 2, Mathsoft, Inc., Seattle, WA, USA) was used to analyze the data.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">According to the univariate analysis, 8 potential risk factors were selected (Table 1). Six risk factors were related to foodborne risk; the other 2 were related to purchasing ewes, and cesarean sections performed by the veterinarian. The subsequent multivariate model (multivariate Clog-log 1) (Table 3) showed a significant association between the flock status and using milk replacers. In addition, using the multivariate Clog-log 2 model milk replacers and proprietary concentrates from factory 1 were significantly associated with the flock status (Table 3).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The main finding of the study was the role of feed as a risk factor for scrapie. This is consistent with what has been shown for BSE in cattle. The use of proprietary concentrates, and more precisely the use of feeds containing meat and bone meal (MBM), was shown to have a major role in BSE infection of cattle (11). The agent of BSE is not inactivated by MBM processing methods, which were put into place by the industry in the late 1970s (17).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In France, MBM was authorized for small ruminants until July 1994. Moreover, the MBM ban proved to be <100% efficient; hundreds of BSE cases were observed in cattle in France born after the MBM ban of feeds for cattle. The exposure period that was investigated in the current study was from 1991 to June 2000, depending on the case. It occurred before the French MBM ban in feeds for all farmed animals in November 2000; furthermore, the period investigated was before the MBM ban for small ruminants in France for more than half of the cases. It is, therefore, plausible that sheep may have been contaminated by MBM in feeds throughout the 1990s, despite control measures. The results showed that 1 feed company was at risk for proprietary concentrates when others were not. This finding is in agreement with the fact that risk might depend on the type of raw materials used in the factory, as well as the way they were processed and used.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The risk attributable to milk replacers is the first evidence of such a TSE risk in animals. Milk replacers for all farmed species are made of skimmed cow milk enriched with vegetable or animal fats. Milk has not been shown to be at risk for scrapie transmission (18–20). Even if animal fat is not infectious, the animal fats that were incorporated in milk replacers may have been contaminated. Contamination could have occurred during collection at the slaughterhouse by contact with infectious material such as central nervous system or paravertebral ganglia. In France, these fats were prohibited for use in farm animal feeds in November 2000.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The same factory was identified as selling both the milk replacers and the proprietary concentrates at risk for scrapie. Most farmers buy both their feeds concentrates and milk replacers from the same wholesaler (which, in turn, buys from the same factory). Even if the effect of the 2 factors remained in the multivariate analysis, a confounding effect between these 2 factors cannot be excluded.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The main concern raised by this study is the nature of the infectious agent that was transmitted to sheep by means of feeds. It might be scrapie, but it could be also BSE, since cattle were infected by feeds during the same period in France. In 2005, BSE in a goat was first reported in France (21); in the United Kingdom, a goat that was thought to have scrapie in 1990 is being reexamined because it is now suspected to have had BSE (http://www.defra.gov.uk/news/2005/050208a.htm). In France, every index case animal from infected small ruminant flocks that has been reported since the surveillance began in 1990 has been biochemically tested to distinguish natural scrapie isolates from isolates sharing common biochemical features with experimental ovine BSE (validated by the TSEs Community Reference Laboratory of Weybridge, UK [unpub. data]). Among >400 small ruminant field isolates tested in France, only 1 isolate from a goat was indistinguishable from BSE. These arguments suggest that the agent transmitted to sheep by food was scrapie rather than BSE. Moreover, BSE is thought to have been transmitted and amplified by recycling contaminated carcasses into MBM on a regional basis (22). It follows that if the sheep identified as having scrapie did in fact have BSE, this misconception would have occurred in the same regions as BSE in cattle. That the areas of France most at risk for BSE in cattle (23) were different from those where scrapie occurred during the study does not suggest that the infectious agent for sheep was BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unexpectedly, the other hypotheses concerning the contamination of flocks with scrapie were not confirmed by the present study. In Norway, a matched case-control study showed 3 risk factors, though at a 10% a level: purchasing females, sharing rams, and sharing pastures between flocks (24). However, in a recent Irish study, purchasing breeding sheep through markets was not a risk factor for scrapie at a 5% a level (25). In the Norwegian study, feed did not appear to be a risk factor, whereas in the Irish study, feeding proprietary concentrates to lambs appeared to be protective. In the present study, purchasing ewes may not have emerged as a risk factor merely because of the lack of power of the study. The link between cesarean sections and scrapie occurrence that was observed in the univariate analysis was likely due to a confounding effect with the real risk factors and so became nonsignificant in the multivariate analyses.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Beyond the limits of the study, our results clearly show that in France, and more precisely in southwest France where most of the studied farms were located, the major risk for the introduction of scrapie in a flock during the 1990s was feeding certain proprietary concentrates and, possibly, milk replacers to sheep. Exposing sheep to TSE risk by feeding has certainly decreased since that time because of the complementary control measures taken in 1996 (ban on specified risk materials and cadavers in the processing of MBM) and 2000 (complete ban of MBM and certain animal fats for all farmed animals). However, it is essential to monitor these risk factors over time in France and to extend this kind of study to other countries in which the disease occurs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The study results show strong evidence that TSEs can spread to sheep through feeding in field conditions, as is the case for cattle. Given the potential risk for humans, the possibility of BSE spreading to sheep must be taken seriously, even though the horizontal transmission of BSE in sheep would occur and stay at a low level (26), should such contamination occur (27). In any case, such findings support the need for a more comprehensive surveillance of TSEs in sheep, as well as the need to systematically examine all scrapie cases for their resemblance to BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Appendix</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Description of the Clog-log model</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This appendix provides a description of the statistic model, a generalized linear model for binary outcome with the complementary log-log link function, used to assess the associations between flock status and risk factors. The construction is based on the probability (P) for a sheep flock to be qualified as an "infected scrapie flock," assuming independence and equiprobability for animals of a same flock to be infected by scrapie. This probability is equal to</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An external file that holds a picture, illustration, etc.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Object name is 04-1223-M1.jpg where k is the flock size and p the probability for an animal of the flock to be diagnosed infected by scrapie. Then, applying the complementary log-log function (Clog-log) on P, the quantity below was obtained.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An external file that holds a picture, illustration, etc. Object name is 04-1223-M2.jpg Therefore, the use of Clog-log as the link function (28) leads to model the probability to be infected at the animal level instead of at the flock level. In this model, flock size is introduced through the offset "Log(k)".</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An external file that holds a picture, illustration, etc. Object name is 04-1223-M3.jpg where Xj, j∈{1, …, q} is the vector of covariates. Moreover, if X1 and X2 are two exposure variables, then</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An external file that holds a picture, illustration, etc. Object name is 04-1223-M4.jpg For values of p smaller than 10%, An external file that holds a picture, illustration, etc. Object name is 04-1223-M5.jpg and An external file that holds a picture, illustration, etc. Object name is 04-1223-M6.jpg are very close. Hence, for small values of p,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An external file that holds a picture, illustration, etc. Object name is 04-1223-M7.jpg. The parameters estimated from the complementary log-log model can be interpreted as those from a logistic model. The appropriate coding of exposure (X = 1) and nonexposure (X = 0) provides an easy interpretation of the parameters with OR = exp(β) and IC95% = [exp(β) +/- zα/2 s.e.(β)], zα/2 being issued from the cumulative distribution function of the standard normal distribution, and s.e. (β) being the standard error of parameter β (29,30).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgments</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3320489/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3320489/</a></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;">2023 ORAL TRANSMISSION OF TYPICAL BSE AND ATYPICAL BSE L-TYPE AND H-TYPE TO CATTLE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">''Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease.''</span><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Abstract for Prion 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presentation Type: Oral Presentation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: ALMA/APRI: 201400006, HC 414250</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">2023 ORAL TRANSMISSION OF CWD TO CATTLE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Transmission of the Chronic Wastng Disease agent from elk to cattle after oronasal exposure</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (<span dir="ltr" style="outline: none !important;">EK211</span>) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack ratesin mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the <span dir="ltr" style="outline: none !important;">EK211</span> genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: NA</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: The authors wish to thank Quazetta Brown, Ami Frank, and Kevin Hassall for their technical contributions.</div></div><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Nuria Jerez-Garrido1, Sara Canoyra1, Natalia Fernández-Borges1, Alba Marín Moreno1, Sylvie L. Benestad2, Olivier Andreoletti3, Gordon Mitchell4, Aru Balachandran4, Juan María Torres1 and Juan Carlos Espinosa1.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">1 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">2 Norwegian Veterinary Institute, Ås, Norway.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">3 UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">4 Canadian Food Inspection Agency, Ottawa, Canada.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD) is an infectious prion disease that affects cervids. Various CWD prion strains have been identified in different cervid species from North America and Europe. The properties of the infectious prion strains are influenced by amino acid changes and polymorphisms in the PrP sequences of different cervid species. This study, aimed to assess the ability of a panel of CWD prion isolates from diverse cervid species from North America and Europe to infect bovine species, as well as to investigate the properties of the prion strains following the adaptation to the bovine-PrP context.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: BoPrP-Tg110 mice overexpressing the bovine-PrP sequence were inoculated by intracranial route with a panel of CWD prion isolates from both North America (two white-tailed deer and two elk) and Europe (one reindeer, one moose and one red deer).</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show distinct behaviours in the transmission of the CWD isolates to the BoPrP-Tg110 mouse model. Some of these isolates did not transmit even after the second passage. Those able to transmit displayed differences in terms of attack rate, survival times, biochemical properties of brain PrPres, and histopathology.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: MCIN/AEI /10.13039/501100011033 and by European Union Next Generation EU/PRTR</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: PCI2020-120680-2 ICRAD </div></div><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;">2023 ATYPICAL SCRAPIE TRANSMIT TO CATTLE</div><div dir="ltr" style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;">Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie</div><div dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Marina Betancor1, Belén Marín1, Alicia Otero1#, Carlos Hedman1, Antonio Romero2, Tomás Barrio3, Eloisa Sevilla1, Jean Yves Douet3, Alvina Huor3, Juan José Badiola1, Olivier Andréoletti3, Rosa Bolea1.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">1Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza, Instituto Agroalimentario de Aragón - IA2, 50013, Zaragoza, Spain.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">2 Servicio de Cirugía y Medicina Equina, Hospital Veterinario, <span dir="ltr" style="outline: none !important;">Universidad de Zaragoza, 50013, Zaragoza, Spain</span></div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">3 UMR École Nationale Vétérinaire de Toulouse (ENVT) - Institut National pour l’Agriculture, l’Alimentation et l’Environnement (INRAE) - 1225 Interactions Hôtes Agents Pathogènes (IHAP), 31300 Toulouse, France.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently proved in rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease <span dir="ltr" style="outline: none !important;">between 7.2 and 11.3</span> years post-inoculation and tested for the accumulation of prions by conventional techniques and protein misfolding cyclic amplification (PMCA).</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Results: None of the bovines showed signs compatible with prion disease. In addition, all tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prions was detected during in vitro propagation of brain samples from the inoculated animals.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This work was supported financially by the following Spanish and European Interreg grants: Ministerio de Ciencia, Innovación y Universidades (Spanish Government), cofunded by Agencia Estatal de Investigación and the European Union and POCTEFA, which was 65% co-financed by the European Regional Development Fund (ERDF) through the Interreg V-A Spain-France-Andorra program (POCTEFA 2014– 2020).</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: n° PID2021-125398OB-I00, EFA148/16 REDPRION</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: The authors would like to thank Sandra Felices and Daniel Romanos for their excellent technical assistance. Authors would also like to acknowledge the use of Servicio General de Apoyo a la Investigación-SAI, Universidad de Zaragoza</div></div><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Emerg Infect Dis. 2011 May; 17(5): 848–854.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">doi: 10.3201/eid1705.101654</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Experimental Oral Transmission of Atypical Scrapie to Sheep<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Marion M. Simmons, corresponding author S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specific prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These findings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although we do not have epidemiologic evidence that supports the efficient spread of disease in the field, these data imply that disease is potentially transmissible under field situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing finding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown.</div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/17/5/10-1654_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/17/5/10-1654_article</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;">CWD, SCRAPIE, ORAL TRANSMISSION TO PIGS, OH MY!</div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Experimental transmission of the chronic wasting disease agent to swine after oral or intracranial inoculation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Running Title: The chronic wasting disease agent transmits to swine</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">S. Jo Moore1,2 , M. Heather West Greenlee3 , Naveen Kondru3 , Sireesha Manne3 , Jodi D. Smith1,# , Robert A. Kunkle1 , Anumantha Kanthasamy3 , Justin J. Greenlee1*</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, Iowa, United States of America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, United States of America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, Iowa, United States of America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Current Address: Department of Veterinary Pathology, Iowa State University College of Veterinary Medicine, Ames, Iowa, United States of America * Corresponding author Email: <span dir="ltr" style="outline: none !important;">justin.greenlee@ars.usda.gov</span></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JVI Accepted Manuscript Posted Online 12 July 2017 J. Virol. doi:10.1128/JVI.00926-17</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This is a work of the U.S. Government and is not subject to copyright protection in the United States. Foreign copyrights may apply.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> on July 27, 2017 by guest <span dir="ltr" style="outline: none !important;">http://jvi.asm.org/</span> Downloaded from</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic wasting disease (CWD) is a naturally occurring, fatal neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation . Crossbred piglets were assigned to one of three groups: intracranially inoculated (n=20), orally inoculated (n=19), or non -inoculated (n=9). At approximately the age at which commercial pigs reach market weight, half of the pigs in each group were culled (‘market weight’ groups). The remaining pigs (‘aged’ groups) were allowed to incubate for up to 73 months post inoculation (MPI ). Tissues collected at necropsy were examined for disease -associated prion protein (PrPSc) by western blotting (WB), antigen -capture immunoassay (EIA), immunohistochemistry (IHC) and in vitro real -time quaking induced conversion (RT -QuIC). Brain samples from selected pigs were also bioassayed in mice expressing porcine prion protein. Four intracranially inoculated aged pigs and one orally inoculated aged pig were positive by EIA, IHC and/or WB. Using RT -QuIC, PrPSc was detected in lymphoid and/or brain tissue from one or more pigs in each inoculated group. Bioassay was positive in 4 out of 5 pigs assayed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study demonstrates that pigs can support low-level amplification of CWD prions, although the species barrier to CWD infection is relatively high. However, detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the case of feral pigs, exposure to the agent of CWD through scavenging of CWD-affected cervid carcasses or through consumption of prion contaminated plants or soil could allow feral pigs to serve as reservoirs of CWD infectivity. The range and numbers of feral pigs is predicted to continue to increase due to the ability of pigs to adapt to many climates, reproduce year-round, and survive on a varied diet (55 ). The range of CWD-affected cervids also continues to spread, increasing the likelihood of overlap of ranges of feral pigs and CWD -affected environments.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We demonstrate here that PrPSc accumulates in lymphoid tissues from pigs inoculated intracranially or orally with the CWD agent, and can be detected as early as 6 months after inoculation. Clinical disease suggestive of prion disease developed only in a single pig after a long (64 months) incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. However, the low amounts of PrPSc detected in the study pigs combined with the low attack rates in <span dir="ltr" style="outline: none !important;">Tg002</span> mice suggest that there is a relatively strong species barrier to CWD prions in pigs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.asm.org/doi/10.1128/jvi.00926-17" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.asm.org/doi/10.1128/jvi.00926-17</a></div></div><br style="outline: none !important;" /></div></div></div></div></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">cwd scrapie pigs oral routes </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in <span dir="ltr" style="outline: none !important;">8/18</span> (44%), and the tonsil in 10/25 (40%). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> CONFIDENTIAL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">LINE TO TAKE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a> </div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">***> Singeltary Hacks in to USDA 50 State Emergency BSE Conference Call <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Tue, 9 Jan 2001 16:49:00 -0800</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: <span dir="ltr" style="outline: none !important;">BSE-L@uni-karlsruhe.de</span> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;">CWD ORAL TRANSMISSION TO WTD</div><div dir="ltr" style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Sarah Zurbuchena,b , S. Jo Moorea,b , Jifeng Biana , Eric D. Cassmanna , and Justin J. Greenleea .</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">a. Virus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, US</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">b. Oak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, United States</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (<span dir="ltr" style="outline: none !important;">Tg12</span>; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10).</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection, analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Ami Frank and Kevin Hassall for their technical contributions to this project.</div></div><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #26282a; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">The detection and decontamination of chronic wasting disease prions during venison processing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Marissa S. Milstein1,2, Marc D. Schwabenlander1,2, Sarah C. Gresch1,2, Manci Li1,2, Stuart Lichtenberg1,2, Rachel Shoemaker1,2, Gage R. Rowden1,2, Jason C. Bartz2,3 , Tiffany M. Wolf2,4, Peter A. Larsen1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presenting author: Tiffany M. Wolf 1 Department of Veterinary and Biomedical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 2 Minnesota Center for Prion Research and Outreach, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 3 Department of Medical Microbiology and Immunology, School of Medicine, Creighton University, Omaha, Nebraska, USA 4 Department of Veterinary Population Medicine, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: There is a growing concern that chronic wasting disease (CWD) prions in venison pose a risk to human health. CWD prions accumulate in infected deer tissues that commonly enter the human food chain through meat processing and consumption. The United States (US) Food and Drug Administration and US Department of Agriculture now formally consider CWD-positive venison unfit for human and animal consumption. Yet, the degree to which prion contamination occurs during routine venison processing is unknown. Here, we use environmental surface swab methods to: a) experimentally test meat processing equipment (i.e., stainless steel knives and polyethylene cutting boards) before and after processing CWD-positive venison and b) test the efficacy of five different disinfectant types (i.e., Dawn dish soap, Virkon-S, Briotech, 10% bleach, and 40% bleach) to determine prion decontamination efficacy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: We used a real-time quaking-induced conversion (RT-QuIC) assay to determine CWD infection status of venison and to detect CWD prions in the swabs. We collected three swabs per surface and ran eight technical replicates on RT-QuIC.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: CWD prions were detected on all cutting boards (n= 3; replicates= 8/8, 8/8, 8/8 and knives (n= 3; replicates= 8/8, 8/8, 8/8) used in processing CWD-positive venison, but not on those used for CWD-negative venison. After processing CWD-positive venison, allowing the surfaces to dry, and washing the cutting board with Dawn dish soap, we detected CWD prions on the cutting board surface (n= 3; replicates= 8/8, 8/8, 8/8) but not on the knife (n= 3, replicates = 0/8, 0/8, 0/8). Similar patterns were observed with Briotech (cutting board: n= 3; replicates= 7/8, 1/8, 0/8; knife: n= 3; replicates = 0/8, 0/8, 0/8). We did not detect CWD prions on the knives or cutting boards after disinfecting with Virkon-S, 10% bleach, and 40% bleach.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: These preliminary results suggest that Dawn dish soap and Briotech do not reliably decontaminate CWD prions from these surfaces. Our data suggest that Virkon-S and various bleach concentrations are more effective in reducing prion contamination of meat processing surfaces; however, surface type may also influence the ability of prions to adsorb to surfaces, preventing complete decontamination. Our results will directly inform best practices to prevent the introduction of CWD prions into the human food chain during venison processing.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: Funding was provided by the Minnesota Environment and Natural Resources Trust Fund as recommended by the Legislative-Citizen Commission on Minnesota Resources (LCCMR), the Rapid Agriculture Response Fund (#95385/RR257), and the Michigan Department of Natural Resources.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Theme: Animal prion diseases</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====end</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;">Prion 2023 Abstracts</div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results show positive prion detection in all products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none !important;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none !important;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none !important;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none !important;">tg650</span> with fecal homogenates. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> <a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a> </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acta Neuropathol 144, 767–784 (2022). https://doi.org/10.1007/s00401-022-02482-9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">© The Author(s) 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords Chronic wasting disease · CWD · Zoonotic potential · Prion strains · Zoonotic prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">================================</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In this study, we evaluated the zoonotic potential of CWD using a transgenic mouse model overexpressing human M129-PrPC (tg650 [12]). We inoculated tg650 mice intracerebrally with two deer CWD isolates, Wisc-1 and 116AG [22, 23, 27, 29]. We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions. Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage. Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute. The fact that its signature was not typical raises the questions whether CWD would manifest in humans as a subclinical infection, whether it would arise through direct or indirect transmission including an intermediate host, or a silent to uncovered human-to-human transmission, and whether current detection techniques will be suffcient to unveil its presence.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=================================</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">CWD transmits to cervid by oral routes with as little as 300NG! </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">PLoS One. 2020; 15(8): e0237410.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Published online 2020 <span dir="ltr" style="outline: none !important;">Aug 20.</span> doi: 10.1371/journal.pone.0237410</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">PMCID: PMC7446902</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">PMID: <span dir="ltr" style="outline: none !important;">32817706</span></div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/</a><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR <span dir="ltr" style="outline: none !important;">589.2000</span>) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">The infamous 1997 mad cow feed ban i.e. Food and Drug Administration's BSE Feed Regulation (21 CFR <span dir="ltr" style="outline: none !important;">589.2000</span>) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">***>However, this recommendation is guidance and not a requirement by law.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">WITH GREAT URGENCY, THE Food and Drug Administration's BSE Feed Regulation (21 CFR <span dir="ltr" style="outline: none !important;">589.2000</span>) MUST BE ENHANCED AND UPDATED TO INCLUDE CERVID, PIGS, AND SHEEP, SINCE RECENT SCIENCE AND TRANSMISSION STUDIES ALL, INCLUDING CATTLE, HAVE SHOWN ORAL TSE PrP TRANSMISSIONS BETWEEN THE SPECIES, AND THIS SHOULD BE DONE WITH THE UTMOST URGENCY, REASONS AS FOLLOW.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">First off I will start with a single BSE feed breach 10 years after 1997 partial ban. If you got to the archived link, all the way down to bottom…THE NEXT YEAR I RECALL ONE WITH 10,000,000+ banned products recall…see this records at the bottom…terry </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">REASON The feed was manufactured from materials that may have been contaminated with mammalian protein. </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">VOLUME OF PRODUCT IN COMMERCE 27,694,240 lbs DISTRIBUTION MI </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">snip..... end</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">***>However, this recommendation is guidance and not a requirement by law.</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">THIS MUST CHANGE ASAP!</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">“For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.”…</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Snip…please see my full submission with reference materials…</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Monday, November 13, 2023</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR <span dir="ltr" style="outline: none !important;">589.2000</span>) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a> </div><div style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;">FRIDAY, JULY 07, 2023 </div><div dir="ltr" style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">***> TME, <span dir="ltr" style="outline: none !important;">589.2000</span> (21 C.F.R. <span dir="ltr" style="outline: none !important;">589.2000</span>), atypical L-BSE, who’s testing MINK for TSE? </div><div dir="ltr" style="outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/07/tme-5892000-21-cfr-5892000-atypical-l.html</a></div></div></div></div><div style="outline: none !important;"> </div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ... See all authors </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">First published: 17 January 2018 <a href="https://doi.org/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.2903/j.efsa.2018.5132</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">also, see; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers.. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div></div></div></div><br style="outline: none !important;" /></div></div><div style="outline: none !important;">OIE Conclusions on transmissibility of atypical BSE among cattle<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">34 Scientific Commission/September 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. Atypical BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nadine Mestre-Francés, Simon Nicot, Sylvie Rouland, Anne-Gaëlle Biacabe, Isabelle Quadrio, Armand Perret-Liaudet, Thierry Baron, and Jean-Michel Verdier</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We report transmission of atypical L-type bovine spongiform encephalopathy to mouse lemurs after oral or intracerebral inoculation with infected bovine brain tissue. After neurologic symptoms appeared, transmissibility of the disease by both inoculation routes was con firmed by detection of disease-associated prion protein in samples of brain tissue.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study clearly con firms, experimentally, the potential risk for interspecies oral transmission of the agent of L-BSE. In our model, this risk appears higher than that for the agent of classical BSE, which could only be transmitted to mouse lemurs after a first passage in macaques (14). We report oral transmission of the L-BSE agent in young and adult primates. Transmission by the IC route has also been reported in young macaques ( 6,7). A previous study of L-BSE in transgenic mice expressing human PrP suggested an absence of any transmission barrier between cattle and humans for this particular strain of the agent of BSE, in contrast to findings for the agent of classical BSE (9). Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://stacks.cdc.gov/view/cdc/23645" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://stacks.cdc.gov/view/cdc/23645</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presentation Type: Oral Presentation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: ALMA/APRI: 201400006, HC 414250</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada. *Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presentation Type: Oral Presentation Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: ALMA/APRI: 201400006, HC 414250</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. "</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2023 CONTINUED; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Molecular phenotype shift after passage of low-type bovine spongiform encephalopathy (L-BSE). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Zoe J. Lambert, M. Heather West Greenlee, Jifeng Bian, Justin J. Greenlee Ames, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this study is to compare the molecular phenotypes of L-BSE in wild type cattle and cattle with the E211K polymorphism to samples of other cattle TSEs, such as classical BSE (C-BSE), hightype BSE (H-BSE), and transmissible mink encephalopathy (TME). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: Two wild type cattle (EE211 PRNP) and one steer with the E211K polymorphism (EK211) were intracranially inoculated with 1 mL of brain homogenate that originated from a 2005 French L-BSE case. Multiple assays were used to compare and differentiate tissues, including enzyme immunoassay, western blot (Sha31, 12B2, SAF84), stability (Sha31), and immunohistochemistry (F99/97). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Approximately 16.6 months post-inoculation, Steer 6 (EK211 L-BSE) developed neurologic signs, including agitation, difficulty eating accompanied by weight loss, head tremor, ataxia, and fasciculations in the forelimbs, and was necropsied. Enzyme immunoassays demonstrated misfolded prion protein in the brainstem (4.0 O.D) but not in peripheral tissues, such as the retropharyngeal lymph node and palatine tonsil. When compared by western blot, the molecular phenotype of the brainstem of Steer 6 (EK211 L-BSE) is higher than that of wildtype cattle inoculated with L-BSE, requiring careful differentiation from C-BSE. Ongoing mouse studies in bovinized mice (K211 and TgBov) will provide data to compare to all other BSE strains available, including L-BSE, C-BSE, H-BSE, E211K H-BSE, and TME. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Further study of L-BSE in EK211 cattle with a higher molecular phenotype in the brainstem may give more insight into the origin of C-BSE. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript. This research was supported in part by an appointment to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DE-SC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: DOE contract number DE-SC0014664 Acknowledgements: NA Theme: Animal prion diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2023 CONTINUED; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="color: #26282a; outline: none !important;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***thus questioning the origin of human sporadic cases. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">============== </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">PRION 2015 CONFERENCE</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">PRION <span dir="ltr" style="outline: none !important;">2016 TOKYO</span></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Saturday, April 23, 2016</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">SCRAPIE <span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr" style="outline: none !important;">1933-6896</span> 1933-690X </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Tuesday, December 16, 2014 </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Evidence for zoonotic potential of ovine scrapie prions </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,<span dir="ltr" style="outline: none !important;">1, Naima Aron</span>,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Abstract </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the humanprion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Subject terms: Biological sciences• Medical research At a glance</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">why do we not want to do TSE transmission studies on chimpanzees $ 5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">snip... R. BRADLEY </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">1: J Infect Dis 1980 Aug;142(2):205-8 </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">snip... </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: <span dir="ltr" style="outline: none !important;">6997404</span></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a></div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"> Nature. 1972 Mar 10;236(5341):73-4. </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis) </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://nor-98.blogspot.com/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://nor-98.blogspot.com/</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/</a> </div><div style="color: #26282a; outline: none !important;"><br clear="none" style="outline: none !important;" /></div><div style="color: #26282a; outline: none !important;"><a href="http://nor-98.blogspot.com/" rel="nofollow" shape="rect" style="color: #196ad4; outline: none !important;" target="_blank">http://nor-98.blogspot.com/</a></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations expand</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMID: 21266763</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 PRION CONFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms separator commenting unavailable </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Angéline Denouel1 , Jean-Philippe Brandel1,2 , Laurène Peckeu-Abboud3 , Danielle Seilhean1 , Elodie Bouaziz-Amar4,5 , Isabelle Quadrio6,7 , Jean-Baptiste Oudart8,9,10 , Sylvain Lehmann11 , Pantxika Bellecave12 , Jean-Louis Laplanche4,5 , Stéphane Haik1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion diseases are rare, fatal disorders that have repeatedly raised public health concerns since the early 1990s. An active prion disease surveillance network providing national level data was implemented in France in 1992.AimWe aimed to describe the epidemiology of sporadic, genetic and infectious forms of prion diseases in France since surveillance implementation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We included all suspected cases notified from January 1992 to December 2016, and cases who died during the period with a definite or probable prion disease diagnosis according to EuroCJD criteria. Demographic, clinical, genetic, neuropathological and biochemical data were collected.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In total, 25,676 suspected cases were notified and 2,907 were diagnosed as prion diseases, including 2,510 (86%) with sporadic Creutzfeldt-Jakob disease (sCJD), 240 (8%) genetic and 157 (6%) with infectious prion disease. Suspected cases and sCJD cases increased over time. Younger sCJD patients (≤ 50 years) showed phenotypes related to a distinct molecular subtype distribution vs those above 50 years. Compared to other European countries, France has had a higher number of cases with iatrogenic CJD after growth hormone treatment and variant CJD (vCJD) linked to bovine spongiform encephalopathy (second after the United Kingdom), but numbers slowly decreased over time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords: Creutzfeldt-Jakob diseases; diagnosis; prion diseases; surveillance system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Key public health message</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">What did you want to address in this study?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion diseases are rare, devastating brain diseases that are transmissible and always fatal. They include genetic, sporadic and infectious forms, e.g. from consumption of contaminated beef (i.e. mad cow disease) or treatment using human-derived medical products. Using the French prion surveillance data, we analysed the data collected since 1992 to understand the appearance of different forms of prion diseases over time within the general population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">What have we learnt from this study?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Infectious forms, and notably cases after treatment with growth hormone of human origin or from consumption of contaminated beef, decreased over time. On the contrary, the number of sporadic cases, for which the cause remains unknown, tended to increase without clear explanation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">What are the implications of your findings for public health?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The decreasing trend of infectious forms we observed in France demonstrates the effectiveness of measures taken to limit prion diseases in the general population. The tendency toward an increase of sporadic forms, also noted in other countries, as well as their unclear origin and the emergence of new prion diseases in animals consumed by humans, underline the need of sustaining an active surveillance.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Introduction Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are rare transmissible neurodegenerative disorders that are invariably fatal. They are caused by a non-conventional agent called a prion, short for ‘proteinaceous infectious particle’ [1], formed by assemblies of a misfolded isoform (PrPsc) of the host-encoded cellular prion protein (PrPc) [2,3]. In humans, TSEs are observed in different forms: sporadic Creutzfeldt–Jakob disease (sCJD), infectious forms including iatrogenic Creutzfeldt–Jakob disease (iCJD), variant Creutzfeldt–Jakob disease (vCJD) and kuru, and genetic forms with genetic Creutzfeldt–Jakob disease (gCJD), Gerstmann–Sträussler–Scheinker syndrome (GSS) and fatal familial insomnia (FFI), which have autosomal dominant transmission with variable penetrance [4]. The incidence of TSEs in humans is around 1 to 2 cases per million person-years in Europe, with sCJD being the most frequent form, accounting for 85% of cases in countries where an active surveillance was implemented in the early 1990s [5-8].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic CJD occurs in late middle age (ca 60–80 years) and is typically characterised by specific neurological symptoms including rapidly progressive dementia associated with ataxia, pyramidal and extrapyramidal signs, myoclonus and visual disorders [9]. Genetic forms of prion disease are caused by different pathogenic point mutations or nucleotide insertions in the prion protein gene (PRNP). Clinical features, which are similar to sCJD, can differ according to the mutation/insertion. Infectious vCJD was first observed in the United Kingdom (UK) [10,11] and France [12] in 1995, linked to cross-species contamination by the agent of classical bovine spongiform encephalopathy (BSE) [13-16]. Variant CJD has mainly impacted younger individuals (< 40 years); the mean age of vCJD cases is 26.5 years in UK [5] and 36 years in France [17], which are the two most affected countries worldwide. Cases with vCJD show a peculiar clinical presentation with early psychiatric disorders and sensory symptoms including atypical pains that affect the face or the limbs and are often drug-resistant. Cases of iCJD appeared from the late 1970s and were associated with corneal transplantations or neurosurgery, primarily with human dura mater grafts or cadaver-sourced human pituitary growth hormone (hGH-iCJD) and more rarely with gonadotrophin treatment [18]. In contrast to sCJD, hGH-iCJD typically affects younger individuals (< 50 years) and is characterised by ataxia and motor disorders followed by myoclonus and dementia [19]. The highest numbers of hGH-iCJD cases have been observed in France followed by the UK, where the first cases occurred in 1989 and 1985, respectively [20].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Because TSEs have led to several public health crises [21,22] given intra-species and inter-species transmissibility of prions and their resistance to conventional procedures of inactivation, many countries implemented nationwide surveillance networks, mostly in 1990s, that are still active. We describe the TSE data from national surveillance between 1992 to 2016 in France, with a particular focus on infectious forms and young patients with sCJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Surveillance system and data collection</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A national surveillance network of TSEs was established in France in 1992 and included in the European CJD surveillance network (EuroCJD) [23]. The French network is coordinated by the French Institute on Health and Medical Research (INSERM) and Santé Publique France, and involves biochemistry laboratories performing 14-3-3 detection in cerebrospinal fluid (CSF), the neuropathology department of the Salpetriere hospital (Paris) that coordinates the neuropathological CJD network, the French national reference centre for prion diseases and the French national unit for CJD care.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD is a notifiable disease since 19 September 1996. Suspected cases are mostly notified to the surveillance network by the biochemistry laboratories that systematically send the results of 14-3-3 detection weekly or monthly, but also by physicians through direct contact with the expert neurologists of the network (JPB and SH) and rarely by neuropathology laboratories.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The network collects demographic and clinical data, including clinical signs, results of 14-3-3 protein detection in the cerebrospinal fluid, electroencephalogram (EEG) and magnetic resonance imaging (MRI)), family history, genetic data (PRNP analysis [24]), results from neuropathological examination and molecular typing of brain PrPsc protease-resistant core (PrPres) by Western blot visualisation after proteinase K (PK) treatment [25].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">All cases registered are investigated until obtaining a final diagnosis (at death for TSE diagnosis). Clinical data are transmitted by the treating physician who is asked to fill in a questionnaire and to send a hospitalisation summary to the French TSE coordinator in the network. Only cases of vCJD are systematically examined by the expert neurologists of the French surveillance network. For autopsied cases, neuropathological laboratories organised in the CJD network transmit neuropathological data. Data on blood donors and recipients were provided by the ‘Etablissement Français du Sang’. The French Ministry of Health requested the national surveillance network to follow up the recipients through a yearly interview with their general practitioner since 2005.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Case definition and diagnosis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Each individual notified to the French surveillance network with a progressive neurological syndrome and at least one clinical sign included in case definitions of CJD was considered as a suspected case of CJD. The case definition for possible, probable or definite TSEs was established by EuroCJD [26]. Diagnostic criteria have been modified since the creation of the French surveillance network, notably for the classification of sCJD. The results from additional paraclinical tests were gradually included, such as those from EEG in 1992, CSF 14-3-3 protein detection in 1998 and striatal high signals from MRI in 2010. The combination of typical clinical symptoms (including cognitive disorders, myoclonus, visual or cerebellar disorders, pyramidal or extrapyramidal features and akinetic mutism) with results from paraclinical testing, enables the classification of a case as possible or probable (evolution of diagnostic criteria can be found in Supplementary Figure S1). The methionine (M)/valine (V) polymorphism at codon 129 of PRNP gene that is known to influence susceptibility to prion disease, age at onset and clinicopathological phenotype was regularly analysed [27-29].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The diagnosis of definite TSE is based on a neuropathological examination showing typical lesions including neuronal loss, spongiosis, astrogliosis and in some cases amyloid plaques, PrPsc deposits as detected by immunohistochemistry and, when frozen brain samples are available, molecular PrPres typing. Type 1 and type 2A, observed in sCJD, are indicated by an electrophoretic mobility of the PK-resistant fragment of the unglycosylated form of PrPres at 21 kDa and 19 kDa, respectively [30]. Type 2B, observed in vCJD, is defined by a molecular weight of 19 kDa of unglycosylated forms associated to predominant biglycosylated forms of PrPres [13,31].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results Between 1992 and 2016, 25,676 suspected cases of prion disease were notified to the French surveillance network. A diagnosis of probable or definite prion disease was retained for 2,907 (11%) of the 25,676 suspected cases. Among those, 2,510 (86%) were classified with a diagnosis of sCJD, 240 (8%) as genetic prion diseases, and 157 (5%) as infectious prion diseases (Figure 1).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Figure 1.Number of suspicions and cases with a diagnosis of probable or definite prion disease by form, France, 1992–2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For the 2,907 prion disease cases, the age at disease onset ranged from 10 to 93 years (median: 68 years; IQR: 60-75); 1,562 females and 1,345 males were observed. The youngest patients were observed among hGH-iCJD cases (n = 116) with a median age at onset of 27 years (IQR: 22–31). Disease duration was the longest in GSS and the shortest in iCJD after dura mater graft and in sCJD cases. Gene analysis of PRNP was performed for 2,077 (71%) cases for whom consent was obtained. An autopsy was performed in 2,114 suspected patients and 1,476 (70%) were confirmed as definite cases of TSE. Molecular type was studied in 926 cases (63%; 926/1,476). Patient characteristics are presented for each form in Table 1. Clinical and diagnostic test characteristics of patients are described for each form in Table 2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 1.Case characteristics for each form of prion disease, France, 1992–2016 (n = 2,907)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 2.Clinical characteristics at the terminal stage of disease by form of prion disease, France, 1992–2016 (n = 2,907)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic Creutzfeldt–Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Median age at disease onset for the 2,510 sCJD cases was 69 years (IQR: 63–76). A genetic analysis was performed on 1,704 cases, and 1,347 were homozygotes at codon 129 (79%) including 1,014 (60%) MM. Age at onset was not significantly different between genotypes at codon 129 (p = 0.057) and no sex difference was observed (p = 0.509). However, cases with MM showed a significantly shorter disease duration (p = 0.001) than those with VV and MV genotypes (Table 3). A total of 842 sCJD cases were pathologically confirmed with information on the molecular type. The most frequent molecular subtype was MM/MV1 and the less frequent VV1. Age at disease onset and disease duration were significantly different between cases with different molecular subtypes (p < 0.001). Cases with VV1 subtypes were younger at disease onset and the highest median disease duration was observed in MV2. Detailed characteristics of each codon 129 polymorphism and molecular subtypes are shown in Table 3 and the repartition of molecular subtypes over time are provided in Supplementary Figure S2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 3.Characteristics of PRNP codon 129 genotypes and molecular subtypes of probable and definite sporadic Creutzfeldt–Jakob disease cases, France, 1992–2016 (n = 1,704)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The sensitivity of the diagnostic tests was respectively 52% for the EEG, 82% for the detection of the 14-3-3 protein, and 46% for the MRI. The specificity was equal to 85%, 58% and 93% respectively. Moreover, sensitivity and specificity of diagnostic criteria over time are described in Supplementary Material S2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Patients aged 90 years and above represented 0.3% (8/2,510) of our sCJD population and had a significantly shorter disease duration (median: 2 months) than sCJD cases aged less than 90 years (2,502/2,510; median: 4 months; p = 0.04). In contrast, patients aged 50 years and under (n = 85) had a longer disease duration compared with patients aged more than 50 years (p < 0.001). Supplementary Table S1 provides a comparison of disease duration between vCJD and sCJD cases, which showed a significant longer disease duration for vCJD compared with sCJD aged 50 years and under (p = 0.002) or above than 50 years (p = 0.028).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Younger sCJD patients (≤ 50 years) had a significantly different distribution of molecular subtypes compared with cases aged above 50 years (p = 0.03) with a higher proportion of VV1 (23%) and MM2 (11%), which are rare in older patients (0.3% and 4%, respectively). The proportion of MM1/MV1 was 59% for cases above 50 years and 39% for 50 years and under (Table 4). Supplementary Table S2 provides the diagnostic test characteristics of patients aged 50 years and under and we observed a significant difference only for the presence of PSWCs on EEG with 29% in younger sCJD cases and 37% in older cases (p = 0.027).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 4.Molecular subtypes of cases of sporadic Creutzfeldt–Jakob disease by age groups, France, 1992–2016 (n = 2,510)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Genetic prion diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Between 1992 and 2016, 8% (240/2,907) of cases were diagnosed with a genetic prion disease; half of them were confirmed as definite cases (120/240). The median age at onset of genetic prion diseases was 58 years (IQR: 50–68) and the median disease duration was 7 months (IQR: 4–12). Among the 240 cases, 118 were females and 122 males. The most frequent mutation was E200K with a phenotype of gCJD, except for one autopsied case who had a phenotype of FFI. The second most frequent mutation was D178N-129M in cases with FFI phenotype, followed by the mutations V201I and D178N-129V in cases with a CJD phenotype. The most frequent insertion was the 192 bp insertion with a phenotype of GSS. Characteristics by form of observed cases in France with genetic prion diseases are presented in Table 1 and Table 2, and additional information on each mutation and insertion are given in Supplementary Table S3.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A diagnosis of vCJD was made in 27 patients (18 definite and 9 probable) including 15 females and 12 males. The median age at onset was 35 years (IQR: 23–47) and the median disease duration was 14 months (IQR: 11–19). All patients were MM at codon 129 of PRNP gene (Table 1). Of 22 vCJD cases who had a tonsil biopsy, 21 were positive for the detection of abnormal prion protein. The patient with a negative tonsil biopsy was classified as definite vCJD after autopsy. Thirteen cases had both a tonsil biopsy and an autopsy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Three vCJD patients with disease onset in 2004 were blood donors, and 42 individuals received labile blood products (i.e. red blood cells, platelets, plasma) from these donors. Of these 42, 31 died from another cause, 22 during the year of the impacted blood transfusion and nine between 4 and 21 years following the transfusion. Of the remaining 11 patients, four were not followed up because they were transfused in 1984 before the BSE outbreaks. The other seven patients, transfused between 1994 and 2004, were still alive with no symptoms of CJD at the time of the surveillance. One of these living individuals received a blood transfusion in 2004 from a donor whose plasma retrospectively tested positive by protein misfolding cyclic amplification (PMCA) assay during the incubation period [32].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Iatrogenic Creutzfeldt–Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From 1992 to 2016, 130 iCJD cases have been reported. Of these, 14 were due to a Lyodura brand dura mater grafted between the mid-1980s and 1994 [6]. The other 116 iCJD were linked to a treatment with growth hormone of human cadaveric origin from at-risk batches during the at-risk treatment period in France (between 1983 and 1985) [33]. Median age at onset was 56 years (IQR: 42–70) in cases related to a dura mater graft and 27 years (IQR: 22–31) in hGH-iCJD, with a median disease duration of 4 and 15 months, respectively. More men than women experienced hGH-iCJD disease (sex ratio: 3.8). Half of the cases were homozygous MM at codon 129 of the PRNP gene (Table 1).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The temporal distribution and incubation period of cases by codon 129 genotype are shown in Figure 2. From 1992 to 1995, all cases were MM or VV. The first heterozygous cases were reported in 1996 and no VV case was observed after 2000 [20] except one in 2015. This case had an incubation period of 31 years, whereas no other homozygous VV cases had an incubation period over 16 years. The incubation period of MM and MV cases was up to 25 years.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Figure 2.Temporal distribution and incubation perioda of cases with iatrogenic Creutzfeldt–Jakob disease linked to human growth hormone treatment, reported by genotype at codon 129 of the prion protein gene, France, 1992–2016 (n = 116)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion Following the emergence of hGH-iCJD cases and the increasing number of BSE cases in the UK, several European countries, including France, implemented an epidemiological surveillance of human TSEs [23]. This enabled the rapid identification of a new form of CJD linked to a cross-species contamination with the BSE agent (vCJD) [10,11,13-16]. The active CJD surveillance network initiated at the national level in France in 1992 has provided long-term data on this rare group of diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Consistent with series collected by other surveillance systems, we report the same distribution of sporadic CJD (around 85%) [5-8]. Genetic CJD represent 10–15% of cases worldwide [4,34,35]. The occurrence of infectious forms varies between countries: vCJD is primarily reported in UK (n = 178 cases) and France (n = 27 cases), while in some other countries, only 1 to 5 cases of vCJD have been observed since the initiation of CJD surveillance [8,36]. With respect to hGH-iCJD cases, the most affected countries in Europe are the UK [5] and France (n = 79 and 116, respectively); fewer hGH-iCJD cases are observed in the other European countries [37].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The number of TSE suspicions increased progressively over time in France, as reported in other countries with a similar surveillance system, such as the UK and Italy [7,38]. This can be explained by network implementation, improvement in case identification [39] (especially for the first years of surveillance) and by population ageing, with an increasing number of older people (≥ 65 years) (‘The National Institute of Statistics and Economic Studies’ (Insee, https://www.insee.fr/en/accueil) who are the most affected by dementias including CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We observed yearly variations in the number of sCJD cases, as reported in other countries by the EuroCJD network [38]. An annual variation of almost 50% in the sCJD mortality is not unusual and not necessarily worrying. However, in the last decades, an increasing trend of sCJD mortality over time occurred in France as well as in other countries [38,40]. Our data show that the evolution of sCJD diagnostic criteria increased the sensitivity contributing to better case detection. More recently, detection of cortical high signals on MRI sequences in at least two different regions of the brain were introduced on criteria in 2017 as well as the results of real-time quaking-induced conversion (RT-QuIC), an amplification method used to detect low amount of PrPsc in cerebrospinal fluid (CSF). The impact of improved diagnosis criteria on measured sCJD mortality should be evaluated in large series. However, even if an intense surveillance system can explain better case ascertainment [39], it cannot be excluded that an actual concurrent increase of sCJD cases occurred over time because of unknown factors [40].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Analyses of PRNP codon 129 of sCJD patients showed an excess proportion of homozygotes at codon 129 (79%, including 60% of MM) in comparison with the French general population (50% of homozygotes) [41]. This observation supports that methionine homozygosity is a susceptibility factor for sCJD occurrence [27,42]. Of the different subtypes, MM1/MV1 was the commonest sCJD subtype with the shortest disease duration (median: 3 months), as previously described [30]. In our study, we merged data from MM1 and MV1 into one subtype, as performed in previous studies, since these molecular subtypes share common clinicopathological characteristics and are both associated with the M1 sCJD prion strain as shown by strain typing in experimental models [9,43,44]. It is worth noting, however, that disease duration was longer in French MV1 than in MM1 cases. More precisely, a subgroup of MV1 cases showed a longer disease duration (data not shown) suggesting that MM1/MV1 subtype might be divided into two subtypes. Gelpi et al. [45] recently identified a new subtype of sCJD in patients carrying MV at PRNP codon 129 with PrPres type 1. These patients presented distinctive clinicopathological features and a long duration (mean: 20.5 months). Further investigations are needed to assess whether the French MV1 patients with longer duration we studied are consistent with this recent observation from Spanish and Italian patients.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The subtype distribution was different for younger sCJD cases with a more frequent proportion of VV1 and MM2 subtypes. The shorter frequency of typical cases (MM1/MV1) in young people compared with patients with sCJD over 50 years old remains to be explained. Indeed, taking the hypothesis of a stochastic conversion of PrP as the event causing sCJD occurrence, the subtype distribution should be the same regardless of the age of the individuals. The specific strain distribution we observed in younger patients compared with older ones might be related to a specific strain selection pressure modulated by age-related endogenous factors (such as the proteostasis system) or to a distinct causative event in some younger patients such as an exposure to exogenous factors.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The largest series of hGH-iCJD cases has been observed in France and the present study confirms a specific time distribution of cases according to the codon 129 genotype, as suggested by Brandel et al. in 2003 [20]. The first French hGH-iCJD cases were all homozygous and the first MV patient was reported 5 years after the onset of the epidemic. More precisely, all valine homozygous hGH-iCJD cases occurred in 2000 and before except for one case that was reported in 2015. A neuropathological examination was not performed and even if the case was actually treated with at-risk batches of cadaverous human pituitary growth hormone [33], we cannot exclude the possibility of a misclassification of a sCJD into a hGH-iCJD case because of his medical history. Clinical characteristics resembled those of sCJD VV2 subtype: 3-month survival time, early ataxia and no dementia at onset, no typical EEG, positive CSF 14-3-3 detection and high signals in basal ganglia on MRI. Of note, our study from 2020 showed that the incubation period was significantly shorter in valine homozygotes than heterozygotes [46], whereas this last hGH-iCJD case showed an extreme incubation period (31 years) in comparison with the other homozygous valine (16 years or less) and with MM and MV cases (25 years or less).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France was the second most affected country by vCJD after the UK. In France, 27 cases of primary vCJD were reported during our study period and the last one occurred in 2014. Two additional cases were observed in 2020 and in 2021 that occurred after occupational exposure to the BSE agent in research laboratories. In 2020, we reported a definite vCJD case in a research technician who experienced an accidental occupational exposure to the classical BSE agent in a prion research laboratory 7.5 years before the disease onset [47]. Even if oral transmission related to contaminated cattle product consumption cannot be formally excluded, the hypothesis of an occupational contamination was reinforced in 2021 with the occurrence of a case of probable vCJD in a retired laboratory worker who also experienced an accidental occupational exposure to the BSE agent 15 years before clinical onset. Both patients were homozygous methionine at codon 129. In 2016, a heterozygous vCJD case was reported in the UK, raising fears of the emergence of a second wave of MV individuals related to a longer incubation period [48]. However, to date, no further heterozygous cases have been reported worldwide, which does not support this hypothesis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">One of the remaining concerns is the risk of secondary contamination in individuals that received blood transfusion from donors incubating vCJD. In the UK, all transfusion-transmitted vCJD cases occurred within 10 years following the transfusion with non-leuco-depleted blood (measure used to prevent bloodborne transmission). In France, last vCJD donors died in 2004 and, up to 2023, no patient who has received labile leuco-depleted blood products from these donors developed symptoms of CJD, not even the patient that received red blood cells prepared from blood donation that retrospectively tested positive by PMCA in plasma [32].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Another concern comes from chronic wasting disease (CWD), a contagious form of prion diseases responsible for epidemics in cervids, the zoonotic potential of which is still debated. The emergence of new CWD strains in European countries was recently demonstrated [49-51].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">During our 25-year surveillance, genetic analyses of the PRNP gene revealed 240 cases of TSE caused by a genetic mutation, and identified several new mutations [24,52]. The commonest observed one was the E200K responsible for a gCJD phenotype [34]. The mutation D178N associated with two distinct phenotypes depending on the genotype at codon 129 on the allele carrying the mutation constitutes the second most frequent mutation in France. Among patients with a FFI phenotype, one had E200K mutation. This genotype/phenotype combination, which was neuropathologically confirmed, has been very rarely reported [53-55].</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study has some limitations. Firstly, the two neurologists in charge of the French surveillance network did not systematically consult all CJD patients (except for vCJD suspected cases) meaning that clinical signs at disease onset are not known with certainty for each patient. They do not have access to all EEGs and MRI data for all patients (only medical reports) and some data might be missed or misinterpreted. Secondly, genetic analyses of PRNP gene and neuropathological examination of suspected cases are not systematically performed, which may lead to some form misclassifications, especially cases considered as sporadic instead of genetic because of missing genetic information. However, this limitation is encountered in the majority of surveillance systems. Finally, in our sCJD population, few patients were aged more than 89 years at disease onset (n = 8). This population had very short disease duration (median: 2 months) that might make the diagnosis more difficult, and we cannot exclude the fact that some cases in the oldest age group were missed by the surveillance system or misdiagnosed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion An active nationwide surveillance was implemented in France in 1992 providing 25 years of data. This enabled us to describe the epidemiology and subtypes of sCJD, including those cases observed in unexpected age groups, and on the epidemic profiles of infectious human prion diseases notably those acquired after peripheral contamination. Sustaining an active surveillance is needed regarding uncertainties about future primary or secondary vCJD cases, the recent occurrence of chronic wasting disease in European cervids with possible zoonotic potential and the tendency towards a regular increase of sCJD mortality observed in various countries.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><span style="outline: none !important;">Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms separator commenting unavailable </span><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><a href="https://www.eurosurveillance.org/content/10.2807/1560-7917.ES.2023.28.50.2300101#html_fulltext" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.eurosurveillance.org/content/10.2807/1560-7917.ES.2023.28.50.2300101#html_fulltext</a></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Total Cases of Sporadic CJD (Deaths)<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic CJD: Definite and probable cases<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Country 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 Total</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France 35 45 59 68 80 81 92 88 109 107 108 97 83 124 138 105 114 146 76 1755</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.eurocjd.ed.ac.uk/surveillance%20data%203.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.eurocjd.ed.ac.uk/surveillance%20data%203.htm</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total Cases of CJD/GSS (Deaths)<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">All Definite And Probable Cases:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic, Familial/Genetic, FFI, GSS and Iatrogenic Deaths (excluding vCJD)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France 55 60 74 88 92 103 105 105 129 124 127 114 98 137 154 122 132 155 80 2054</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.eurocjd.ed.ac.uk/surveillance%20data%202.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.eurocjd.ed.ac.uk/surveillance%20data%202.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.eurocjd.ed.ac.uk/surveillancedata.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.eurocjd.ed.ac.uk/surveillancedata.htm</a> </div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;">France iatrogenic CJD TSE Prion</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision CORRESPONDENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jacqueline Mikol1 · Jérôme Delmotte1 · Dolorès Jouy1 · Elodie Vaysset1 · Charmaine Bastian1 · Jean‑Philippe Deslys1 ·</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel Comoy1 Received: 10 July 2020 / Revised: 8 September 2020 / Accepted: 25 September 2020 / Published online: 6 October 2020 © The Author(s) 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Non-human primates appeared as the closest model to study human iatrogenic prion diseases [14]: we report here the consequences of variant Creutzfeldt–Jakob disease/bovine spongiform encephalopathy (vCJD/BSE) inoculation in a cynomolgus macaque finger, with the demonstration of an original mode of propagation and the practical risk for professional exposure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The distal right middle finger handpad of a 4-year-old macaque was incised on both lateral sides to induce local inflammation, and then injected with the equivalent of 10 mg of a BSE, orally challenged macaque brain [18]. After an 18 months period of finger clumsiness, the clinical disease (behaviour abnormalities, fear, hyperesthesia, gait disturbances, shaking) began 7.5 years after inoculation and euthanasia took place 2 months later for welfare reasons. Motor conduction velocity of the right median nerve was reduced to one-third of the left counterpart and sensory potential was not detected.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Histological and biochemical studies were performed as previously described. All the elements of the triad were present [7–9]: spongiform change was moderate in neocortex, striatum, brain stem, mild in spinal cord but severe in thalamus and cerebellum; neuronal loss was globally moderate, but severe in cerebellum and sacral spinal cord (vacuolated neurons); gliosis was severe in thalamus, cerebellum and brain stem and moderate elsewhere (Supplementary Fig. 1). ELISA and western blot (WB) showed the expected accumulation of PrPres with BSE glycophoretic pattern at all levels of brain and spinal cord (Supplementary Fig. 2).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the brain, PrPd deposits were laminar into the cortical deep layers, massive into thalamus, basal ganglia, cerebellum, and brain stem. In spinal cord, PrPd was symmetrically distributed, intense in the Substantia gelatinosa and nucleus dorsal of Clarke while decreased at sacral level. Deposits were diverse into the whole CNS: synaptic, perineuronal, reticular aggregates, mini-plaques, plaques, and incomplete florid plaques. The retinal plexiform layers were labelled (Supplementary Fig. 1i). There were no amyloid or tau deposits.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unusual PrPd deposits were observed along dendrites, short and long axons, neuritic threads tracing fne networks of straight lines or like strings of pearls (Supplementary Fig. 3). They were present into deep neocortex, basal ganglia, and motoneurons. Such long processes are not frequent but have been reported in human [13] and experimental studies [10, 22]. PrPd deposits were also noted as very mild into striato-pallidal projections, both limbs of internal capsule and fornix (Supplementary Fig. 3). The presence of PrPd in white matter has been reported (Supplementary text 4).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Peripherally, the expected PrPd was undetectable in lymphoid organs, including spleen, through biochemical or immunohistochemical analyses, while prion replication was detected in the peripheral nervous system (PNS): PrPd staining was visualized in many dorsal root ganglia (DRG) but only in nerves innervating the forelimb site of injection (median and ulnar nerves). At the cellular level, PrPd was limited to ganglia and satellite cells in DRG and Schwann cells (Scs) all along nerves whereas axons were never labelled (Fig. 1). Previously, using postmortem immunohistochemical studies (listed in Supplementary text 5), PrPd has been shown in peripheral nervous system in all forms of human neuropathies, albeit more frequently in vCJD, mostly in posterior root nerve fbres at adaxonal location and/or in ganglion and satellite cells. The restricted amount of PrPd was repeatedly underlined but, recently, prion RTQuiC was positive in all nerves examined [2]. PrPd has also been described, frst in scrapie [17] then in BSE, as limited “adaxonal deposits” or/and Sc deposits, with or without DRG cell involvement (review in [4] and Supplementary text 6). Previous studies of the mode of propagation of PrPd have reported variable observations and analyses depending on strains, host species and genotype (Supplementary text 6); the authors discussed the role of the sensory route of trafficking of prions, the modifications of axonal transport, the centrifugal versus centripetal spread of PrPd .</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">After peripheral infection, accumulation of infectious agent is reputed to occur in lymphoid tissues before direct neuroinvasion [18, 19], even with very little apparent peripheral lymphoreticular deposition [6, 20]. Here, there is no apparent replication/amplification of vCJD/BSE agent in the lymphoid tissues of the exposed macaque. In this model, the neural contamination occurred directly in the highly innervated finger while neuroinvasion appears to occur in Scs along the median nerve to the DRG, with the appearance of the classical labelling of ganglion cells which indicates the onset of the first level of neuronal infection. This model provides direct evidence of the hypothesis of a sequential infection of Scs from the periphery to the CNS, followed by a secondary diffusion into the spinal cord, as already considered by our group [15] and others [1, 3, 11, 12, 21]. It is to note that studies based on intra-sciatic nerve injections in hamsters [16] and transgenic mice [12] had established a rate of transport of infectivity of, respectively, 0.5–2 mm and 0.7 mm per day. This key role of Scs could explain both the low speed of propagation and the discrepancy between the paucity of PrPd into the distal part of the sensory nerves followed by the positivity of DRG, satellite cells and proximal roots.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In conclusion, we have observed that the exposure of a primate to vCJD/BSE through a distal finger lesion induces, after more than 7.5 years of silent incubation, a massive deposit of PrPd , strictly restricted to the nervous system and the eye.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our data suggest a new type of pure unique peripheral nervous contamination in which the Scs would have a major role in the mode of centripetal progression of PrPd in the peripheral nervous system. Moreover, considering the fact that, recently, “a variant CJD diagnosed 7.5 years after occupational exposure” (cryomicrotomy) in a technician was observed [5], this experimental case report supports the risk linked to professional exposure and reinforces the necessity of adequate measures of prevention. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Second death in France in a laboratory working on prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease has killed a person who handled this infectious agent at Inrae in Toulouse. After a first death in 2019, a moratorium on work on this pathogen has been extended.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">By Hervé Morin</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working with these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of mice infected with prions, in another unit of INRAE, in Jouy-en-Josas.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Computer representation of part of a prion protein on a light micrograph of pyramidal nerve cells (neurons, in black) in the cerebellum of the brain. ALFRED PASIEKA / SCIENCE PHOTO LIBRARY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Temporary suspension of work on prions in French public research laboratories</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRESS RELEASE - The general directorates of ANSES, CEA, CNRS, INRAE and Inserm, have decided jointly and in agreement with the Ministry of Higher Education, Research and Innovation to suspend as a precaution all their research and experimentation work relating to prion diseases, for a period of three months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This precautionary measure is motivated by the knowledge of a possible new case of a person suffering from Creutzfeldt-Jakob disease and who worked in a laboratory for research on prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted on July 27, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The suspension period put in place as of this day will make it possible to study the possibility of a link between the observed case and the person's former professional activity and to adapt, if necessary, the preventive measures in force in the research laboratories. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The person with Creutzfeldt-Jakob disease (CJD)1, whose form is not yet known, is a retired INRAE agent. This could be the second case of infectious CJD affecting a scientist who worked on prions, after that of an assistant engineer who died of the disease in 2019, and who was injured in 2010 during of an experiment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Following this death, a general inspection mission was launched in July 2019 by the ministries of research and agriculture with French laboratories handling prions. Submitted in October 2020, the report concluded on the regulatory compliance of the laboratories visited as well as the presence of a risk control culture within the research teams.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research around prion proteins, with high public health issues, allows major advances in the understanding of the functioning of these infectious pathogens, and contributes to results that are transferable to other related degenerative diseases such as Alzheimer's and Alzheimer's diseases. Parkinson's.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At the level of each establishment, regular and transparent information will be provided to all the working communities concerned by this measure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 The disease Creutzfeldt-Jakob disease (CJD) is one of prion diseases - still called encephalopathies subacute spongiform transmitted(TSE) - of diseases rare, characterized by a degeneration rapid and fatal the system nervous central. They are caused by the accumulation in the brain of a normally expressed protein but poorly conformed - the prion protein - which leads to the formation of deleterious aggregates for neurons. For now , no treatment will allow to change the course of these diseases. It can be of origin sporadic , form the most frequent , original genetic or finally to form infectious following a contamination. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">By Barbara CasassusJul. 28, 2021 , 4:35 AM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">With reporting by Martin Enserink.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted in: EuropeHealthScientific Community</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">doi:10.1126/science.abl6587</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TO THE EDITOR:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Figure 1.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">5 References</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">July 2, 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">N Engl J Med 2020; 383:83-85</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DOI: 10.1056/NEJMc2000687</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Metrics</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The threat from iatrogenic TSE Prion disease, is a real threat today, one that should be taken seriously across the medical/surgical arenas, considering the recent potential TSE iatrogenic events in Spain, and confirmed iatrogenic TSE, France several years back, where lab workers were exposed to sheep BSE, and died from vCJD. The USA must be very cautious with CWD TSE Prion in Cervid, consumption, exposure of the Cwd, and friendly fire there from, This is my greatest fear now, and this is why all this matters.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Iatrogenic TSE Prion </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/</a></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">FRIDAY, DECEMBER 22, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Mad Cow That Stole Christmas, 20 Years Later</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Mad Cow That Stole Christmas, 20 Years Later, What Has Changed, Nothing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THE USA has systematically covered up mad cow disease, in my honest opinion, the USA mad cow disease today, is Chronic Wasting Disease CWD TSE Prion disease in Cervid, they can't cover that up. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2023/12/the-mad-cow-that-stole-christmas-23.html</a></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">TUESDAY, NOVEMBER 28, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EFSA TSE Report 2022 First published 28 November 2023</div><div style="outline: none !important;">The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-tse-report-2022-first-published-28.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-tse-report-2022-first-published-28.html</a></div></div></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">THURSDAY, DECEMBER 7, 2023 <br style="outline: none !important;" /></div></div></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic Wasting Disease CWD TSE Prion Cervid Update By State December 2023 (Long Version) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(Short Version) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, DECEMBER 08, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TEXAS CWD TSE PRION DIRE CONSEQUENCES ARE HERE! </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Why is USDA "only" testing 25,000 samples a year?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USDA's surveillance strategy is to focus on the targeted populations where we are most likely to find disease if it is present. This is the most effective way to meet both OIE and our domestic surveillance standards. After completing our enhanced surveillance in 2006 and confirming that our BSE prevalence was very low, an evaluation of the program showed that reducing the number of samples collected to 40,000 samples per year from these targeted, high risk populations would allow us to continue to exceed these standards. In fact, the sampling was ten times greater than OIE standards. A subsequent evaluation of the program in 2016 using data collected over the past 10 years showed that the surveillance standards could still be met with a further reduction in the number of samples collected by renderers and 3D/4D establishments which have a very low OIE point value because the medical history of these animals is usually unknown. Therefore, in 2016, the number of samples to be tested was reduced to 25,000 where it remains today.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.usda.gov/topics/animals/bse-surveillance-information-center" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.usda.gov/topics/animals/bse-surveillance-information-center</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Bottom line, you don’t test, you don’t find$ FRIDAY, MAY 19, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, MAY 20, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, May 24, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Docket No. APHIS–2023–0027 Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full submission;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WEDNESDAY, NOVEMBER 08, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE confirmed November 3 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 14, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE case, 3 progeny of case cow to be culled </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, JULY 16, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Atypical BSE detected in a cow in the canton of St. Gallen </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monday, March 20, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE START DATE 2023/01/18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE CONFIRMATION DATE 2023/02/22</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE END DATE 2023/03/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE START DATE 2023/01/21</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE CONFIRMATION DATE 2023/02/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE END DATE 2023/02/06</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE START DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE CONFIRMATION DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE END DATE 2023/03/13</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">EFSA atypical Scrapie</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...SEE;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THURSDAY, JULY 8, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EFSA Scientific report on the analysis of the 2‐year compulsory intensified monitoring of atypical scrapie</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/10.2903/j.efsa.2021.6686</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2021.6686</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2021/07/efsa-scientific-report-on-analysis-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2021/07/efsa-scientific-report-on-analysis-of.html</a> </div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Monday, November 13, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor John Collinge on tackling prion diseases, sCJD accounts for around 1 in 5000 deaths worldwide<br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">MONDAY, SEPTEMBER 11, 2023 <br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor John Collinge on tackling prion diseases </span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</span></div></div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.<br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp96b8352fyiv6513226400ydp7cf26efayiv5525069040ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></span></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">MONDAY, DECEMBER 18, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/change-in-epidemiology-of-creutzfeldt.html</a></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">TUESDAY, DECEMBER 12, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">SUNDAY, NOVEMBER 26, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/the-role-of-environmental-factors-on.html</a></div></div></div></div></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">FRANCE HISTORY MAD COW DISEASE</div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://www.google.com/search?q=france+mad+cow+feed&sca_esv=593473706&hl=en&source=hp&ei=7KSIZfPcJaizqtsPutKpoAI&iflsig=AO6bgOgAAAAAZYiy_GvIT53Mjtoah9gBpWIdK0v-n0a-&ved=0ahUKEwjzgq60hKmDAxWomWoFHTppCiQQ4dUDCAw&uact=5&oq=france+mad+cow+feed&gs_lp=Egdnd3Mtd2l6IhNmcmFuY2UgbWFkIGNvdyBmZWVkMgUQIRigATIFECEYoAFI6yJQAFiCHXAAeACQAQCYAV-gAbILqgECMTm4AQPIAQD4AQHCAhEQLhiABBixAxiDARjHARjRA8ICCxAAGIAEGLEDGIMBwgIIEAAYgAQYsQPCAgUQABiABMICDhAuGIAEGLEDGMcBGNEDwgIOEC4YxwEYsQMY0QMYgATCAg4QABiABBiKBRixAxiDAcICFBAuGIAEGIoFGLEDGIMBGMcBGNEDwgILEC4YgAQYsQMYgwHCAgsQLhiABBjHARjRA8ICCxAuGIAEGLEDGNQCwgIIEC4YgAQYsQPCAgUQLhiABMICCxAuGIAEGMcBGK8BwgIEEAAYA8ICBxAAGIAEGArCAgYQABgWGB7CAggQABgWGB4YD8ICCBAAGBYYHhgKwgILEAAYgAQYigUYhgPCAgUQIRirAsICBRAhGJ8F&sclient=gws-wiz#ip=1" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.google.com/search?q=france+mad+cow+feed&sca_esv=593473706&hl=en&source=hp&ei=7KSIZfPcJaizqtsPutKpoAI&iflsig=AO6bgOgAAAAAZYiy_GvIT53Mjtoah9gBpWIdK0v-n0a-&ved=0ahUKEwjzgq60hKmDAxWomWoFHTppCiQQ4dUDCAw&uact=5&oq=france+mad+cow+feed&gs_lp=Egdnd3Mtd2l6IhNmcmFuY2UgbWFkIGNvdyBmZWVkMgUQIRigATIFECEYoAFI6yJQAFiCHXAAeACQAQCYAV-gAbILqgECMTm4AQPIAQD4AQHCAhEQLhiABBixAxiDARjHARjRA8ICCxAAGIAEGLEDGIMBwgIIEAAYgAQYsQPCAgUQABiABMICDhAuGIAEGLEDGMcBGNEDwgIOEC4YxwEYsQMY0QMYgATCAg4QABiABBiKBRixAxiDAcICFBAuGIAEGIoFGLEDGIMBGMcBGNEDwgILEC4YgAQYsQMYgwHCAgsQLhiABBjHARjRA8ICCxAuGIAEGLEDGNQCwgIIEC4YgAQYsQPCAgUQLhiABMICCxAuGIAEGMcBGK8BwgIEEAAYA8ICBxAAGIAEGArCAgYQABgWGB7CAggQABgWGB4YD8ICCBAAGBYYHhgKwgILEAAYgAQYigUYhgPCAgUQIRirAsICBRAhGJ8F&sclient=gws-wiz#ip=1</a><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr.</div><div><br /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-39069786391548004692023-12-18T17:07:00.000-06:002023-12-18T17:07:10.859-06:00Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020<div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</span><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">crane@jhu.edu</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Letter</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">December 11, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et alJohn A. Romley, PhD4; John C. Probasco, MD5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations Article Information</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA Neurol. Published online December 11, 2023. doi:10.1001/jamaneurol.2023.4678</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This cross-sectional study used data from the Wide-Ranging Online Data for Epidemiologic Research multiple cause of death database.4 Data were retrieved on September 7, 2023, and analyzed between September 7 and September 23, 2023. The Johns Hopkins Medicine institutional review board determined that this study did not constitute human participant research; thus, informed consent was not sought. We followed STROBE reporting guidelines.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We examined death certificates with ICD-10 code A81.0 from 2007 to 2020 for CJD incidence and age and sex distribution.2 Patient-level demographic information could not be collected due to limitations of the mortality surveillance database. Joinpoint Regression Program, version 4.9.1.0 (National Cancer Institute) was used to characterize inflection points and average annual percent change (AAPC) (eMethods in Supplement 1). We used Stata, version 18.0 (StataCorp LLC) to conduct t tests for cohort changes across the study period, with a 2-sided P < .05 considered significant.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reported CJD incidence rose consistently in 2007-2020 (5882 total cases; 3009 female [51.2%] and 2873 male [48.8%]). Males saw an increase from 155 deaths and incidence of 1.05 (95% CI, 0.88-1.21) per million in 2007 to 238 deaths and incidence of 1.47 (95% CI, 1.28-1.65) per million (P = .001) in 2020. Females saw a greater increase from 162 deaths and incidence of 1.06 (95% CI, 0.89-1.22) per million to 264 deaths and incidence of 1.58 (95% CI, 1.39-1.77) per million (P < .001) across the same period (Figure). After age adjustment, the increase in incidence was not significant for males (1.06 [95% CI, 0.89-1.23] to 1.19 [95% CI, 1.04-1.35] per million; P = .25), but remained significant for females (0.92 [95% CI, 0.78-1.06] to 1.12 [95% CI, 0.99-1.26] per million; P = .045). In individual age groups, the largest increase in incidence was in those aged 75 to 84 years, though this finding was not significant in males (3.50 [95% CI, 2.65-4.55] to 7.48 [95% CI, 5.62-9.75] per million; P = .18) or females (3.38 [95% CI, 2.21-4.95] to 5.20 [95% CI, 3.84-6.90] per million; P = .07).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Joinpoint analysis revealed a significant increase in crude incidence for males (AAPC, 2.9; 95% CI, 2.3-3.6; P < .001) and females (AAPC, 3.5; 95% CI, 2.2-4.7; P < .001) overall. The crude incidence decreased after age adjustment but remained significant for both sexes (AAPC: males, 0.8 [95% CI, 0.1-1.5; P = .02]; females, 1.9 [95% CI, 0.6-3.2; P = .005]). Males aged 55 to 64 years were the only male age group with a significant increase, but females showed a significant increase in all age cohorts (Table).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Accepted for Publication: October 9, 2023.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published Online: December 11, 2023. doi:10.1001/jamaneurol.2023.4678</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Corresponding Author: Matthew A. Crane, BS, Johns Hopkins University School of Medicine, 733 N Broadway, Edward D. Miller Research Bldg, Ste 137, Baltimore, MD 21205-2196 (crane@jhu.edu).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Contributions: Mr Crane had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Concept and design: Crane, Nair-Desai, Probasco.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acquisition, analysis, or interpretation of data: All authors.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Drafting of the manuscript: Crane, Nair-Desai, Probasco.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Critical review of the manuscript for important intellectual content: All authors.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Statistical analysis: Crane, Nair-Desai, Romley.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supervision: Nair-Desai, Probasco.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conflict of Interest Disclosures: None reported.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disclaimer: This article reflects the views of the authors and should not be construed to represent the views or policies of the US Food and Drug Administration, the Department of Health and Human Services, or the US government.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Data Sharing Statement: See Supplement 2.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">References</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1. Watson N , Brandel JP , Green A , et al. The importance of ongoing international surveillance for Creutzfeldt-Jakob disease. Nat Rev Neurol. 2021;17(6):362-379. doi:10.1038/s41582-021-00488-7 PubMedGoogle ScholarCrossref</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2. Holman RC , Belay ED , Christensen KY , et al. Human prion diseases in the United States. PLoS One. 2010;5(1):e8521. doi:10.1371/journal.pone.0008521 Google Scholar 3. Nishimura Y , Harada K , Koyama T , Hagiya H , Otsuka F . A nationwide trend analysis in the incidence and mortality of Creutzfeldt-Jakob disease in Japan between 2005 and 2014. Sci Rep. 2020;10(1):15509. doi:10.1038/s41598-020-72519-0 PubMedGoogle ScholarCrossref</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4. About multiple cause of death 1999-2020. Centers for Disease Control and Prevention; 2021. Accessed September 6, 2023. https://wonder.cdc.gov/mcd-icd10.html 5. Davanipour Z , Smoak C , Bohr T , Sobel E , Liwnicz B , Chang S . Death certificates: an efficient source for ascertainment of Creutzfeldt-Jakob disease cases. Neuroepidemiology. 1995;14(1):1-6. doi:10.1159/000109771 PubMedGoogle ScholarCrossref</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">6. Maddox RA , Person MK , Blevins JE , et al. Prion disease incidence in the United States: 2003-2015. Neurology. 2020;94(2):e153-e157. doi:10.1212/WNL.0000000000008680 PubMedGoogle ScholarCrossref</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jamaneurology/fullarticle/2812784</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Greetings JAMA et al, Officials, Authors et al, </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">Many Thanks for this study!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would like to address this study, and my concerns of why I propose, under present CJD TSE Prion surveillance methods, that I believe the true count of deaths are woefully undercounted, and the reasons there from being ignored, and passed off as sporadic/spontaneous. i believe that the increase of CJD and human TSE is partly due to the real risk from recent studies that these sporadic, spontaneous, CJD, BSE, Scrapie and CWD, may not be so spontaneous/sporadic, and in fact zoonosis from typical and atypical BSE, Scrapie, CWD, and or iatrogenic events from all of the above. Other recent studies have proposed this same thing, I questioned it back in 2001, in JAMA, and other Journals. With the recent findings of another case of mad cow disease in the USA, Atypical L-Type BSE, the most virulent to date, the fact that laboratory studies, studies in Macaque, shows transmission of this strain will not look like nvCJD, but like some strain of sporadic CJD, the fact that CWD of cervid will transmit to Macaque, Cattle, Sheep, Cervid, by oral routes. transmission studies now show sheep scrapie in humans will look like sporadic CJD. we just can't keep kicking the zoonosis/iatrogenic can down the sporadic/spontaneous road. Iatrogenic transmission from all of these TSE prion disease in all these species, are a solid risk factor now for transmission to humans, and should be addressed as such. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">My name is Terry S. Singeltary Sr. and I wrote a short piece way back in 2001 on Creutzfeldt Jakob Disease, in which JAMA published, this after losing my Mother to the Heidenhain Variant of Creutzfeldt Jakob disease hvCJD. I was very grateful for that. see;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, Sr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NOW, 2023, we finally see sporadic CJD cases rising, documented in print, but there was a great deal of important information left out, WHY IS IT RISING? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> Due to aging populations worldwide, the epidemiology of CJD is evolving.3 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I wish to submit to you my concerns again as a layperson, one that has followed the TSE Prion saga every day, since that fateful day December 14, 1997. 2023, the United States had another mad cow case, atypical L-type BSE, the most virulent strain to date, and scientific studies have linked sporadic CJD to atypical BSE, to typical BSE, to atypical and typical Scrapie, and to CWD. 2023 Prion Conference and recent studies beforehand, shows that CWD will transmit by ORAL ROUTES TO, Macaques, CATTLE, SHEEP, PIGS, AND TO CERVID. we know the USA BSE surveillance and testing is woefully inadequate (<25k A YEAR) especially with Cervid and Pigs being allowed for cattle feed still. many mad cow cases have been documented in 2023 from many different countries, and the old cow spontaneous theory is bogus IMO, for 85%+ of all human TSE Prion i.e. sporadic CJD, atypical BSE, the old spontaneous BSE old cow disease excuse that theory is starting to crumbling with recent transmission studies. TSE prion Science is evolving and showing that sporadic CJD and atypical BSE might no be so spontaneous after all. now from all that, think iatrogenic ramifications from all the above, in Hospitals across the USA. CWD in cervid is out of control in the USA, with no stopping it in sight, what about consumption, exposure, and friendly fire there from i.e. iatrogenic. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">with great urgency, i present my facts. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> Due to aging populations worldwide, the epidemiology of CJD is evolving.3 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would kindly like to submit with great urgency, the science of what is really going on now with CJD, and the science and transmission studies to back them up. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">to date, there has been no proof of spontaneous TSE prion in any species, that's just the facts, to date. the nvCJD or what is called vCJD today, they keep claiming that is over, yet, sporadic CJD is growing, and environmental factors are pointing to sporadic CJD now.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I know this is not the proper channels to publish anything with JAMA, but i had to at least send it to you all, so that at least you would be aware of the ramifications of the future ahead with TSE Prion disease in the USA. i just also finished my end of year reports on BSE and CWD. I will add them in links. this is very serious, i have wasted every day for 26 years, please study this, i'm getting old, times getting short, please take heed, sporadic CJD just might be the nvCJD nightmare epidemic everyone missed, and with the recent potential cjd occupational exposure in Spain now, and the recent documented 2 deaths of iatrogenic sheep BSE transmission to lab workers as nvCJD, now think CWD exposure, and iatrogenic transmission there from. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Today, there is more science showing that CWD will transmit to humans, yet no call has been made, than there was with nvCJD back in 1995, some decade passed that infamous day back in 1984ish, Carol Richard, kind of documented something, the next year 1995, Mad Cow was confirmed, typical c-type BSE. what are we waiting for, who makes that call, how many do we expose, and or, how many have to die? Who will bare that Burdon of ignorance for not sounding the alarm that sCJD was zoonotic zoonosis from all of the above, when the evidence had been staring us in the face for decades? I wish to submit to you my concerns again as a layperson, one that has followed the TSE Prion saga every day, since that fateful day December 14, 1997. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023, the United States had another mad cow case, atypical L-type BSE, the most virulent strain to date, and scientific studies have linked sporadic CJD to atypical BSE, to typical BSE, to atypical and typical Scrapie, and to CWD. 2023 Prion Conference and recent studies beforehand, shows that CWD will transmit by ORAL ROUTES TO, Macaques, CATTLE, SHEEP, PIGS, AND TO CERVID. we know the USA BSE surveillance and testing is woefully inadequate (<25k A YEAR) especially with Cervid and Pigs being allowed for cattle feed still. many mad cow cases have been documented in 2023 from many different countries, and the old cow spontaneous theory is bogus, studies show this now. TSE prion Science is evolving and showing that sporadic CJD and atypical BSE might no be so spontaneous after all. now from all that, think iatrogenic ramifications from all the above, in Hospitals across the USA. CWD in cervid is out of control in the USA, with no stopping it in sight, what about consumption, exposure, and friendly fire there from i.e. iatrogenic. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">with great urgency, i present my facts. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> Due to aging populations worldwide, the epidemiology of CJD is evolving.3 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would kindly like to submit with great urgency, the science of what is really going on now with CJD, and the science and transmission studies to back them up. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">to date, there has been no proof of spontaneous TSE prion in any species, that's just the facts, to date. the nvCJD or what is called vCJD today, they keep claiming that is over, yet, sporadic CJD is growing, and environmental factors are pointing to sporadic CJD now.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I know this is not the proper channels to publish anything with JAMA, but i had to at least send it to you all, so that at least you would be aware of the ramifications of the future ahead with TSE Prion disease in the USA. i just also finished my end of year reports on BSE and CWD. I will add them in links. this is very serious, i have wasted every day for 26 years, please study this, i'm getting old, times getting short, please take heed, sporadic CJD just might be the nvCJD nightmare epidemic everyone missed, and with the recent potential cjd occupational exposure in Spain now, and the recent documented 2 deaths of iatrogenic sheep BSE transmission to lab workers as nvCJD, now think CWD exposure, and iatrogenic transmission there from. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Today, there is more science showing that CWD will transmit to humans, yet no call has been made, than there was with nvCJD back in 1995, some decade passed that infamous day back in 1984ish, Carol Richard, kinda documented something, the next year 1995, Mad Cow was confirmed, typical c-type BSE. what are we waiting for, who makes that call, how many do we expose, and or, how many have to die? Who will bare that Burdon of ignorance for not sounding the alarm that sCJD was zoonotic zoonosis from all of the above, when the evidence had been staring us in the face for decades?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, DECEMBER 12, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THURSDAY, DECEMBER 7, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Chronic Wasting Disease CWD TSE Prion Cervid Update By State December 2023 (Long Version) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">***> Chronic Wasting Disease CWD TSE Prion Cervid Update By State December 2023 (Short Version) </div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, DECEMBER 08, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TEXAS CWD TSE PRION DIRE CONSEQUENCES ARE HERE! </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Why is USDA "only" testing 25,000 samples a year?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USDA's surveillance strategy is to focus on the targeted populations where we are most likely to find disease if it is present. This is the most effective way to meet both OIE and our domestic surveillance standards. After completing our enhanced surveillance in 2006 and confirming that our BSE prevalence was very low, an evaluation of the program showed that reducing the number of samples collected to 40,000 samples per year from these targeted, high risk populations would allow us to continue to exceed these standards. In fact, the sampling was ten times greater than OIE standards. A subsequent evaluation of the program in 2016 using data collected over the past 10 years showed that the surveillance standards could still be met with a further reduction in the number of samples collected by renderers and 3D/4D establishments which have a very low OIE point value because the medical history of these animals is usually unknown. Therefore, in 2016, the number of samples to be tested was reduced to 25,000 where it remains today.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.usda.gov/topics/animals/bse-surveillance-information-center" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.usda.gov/topics/animals/bse-surveillance-information-center</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Bottom line, you don’t test, you don’t find$ FRIDAY, MAY 19, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, MAY 20, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, May 24, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Docket No. APHIS–2023–0027 Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full submission;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WEDNESDAY, NOVEMBER 08, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE confirmed November 3 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 14, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ireland Atypical BSE case, 3 progeny of case cow to be culled </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, JULY 16, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Atypical BSE detected in a cow in the canton of St. Gallen </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2020/02/switzerland-oie-bovine-spongiform.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monday, March 20, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE START DATE 2023/01/18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE CONFIRMATION DATE 2023/02/22</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE END DATE 2023/03/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE START DATE 2023/01/21</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE CONFIRMATION DATE 2023/02/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE END DATE 2023/02/06</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/02/spain-bovine-spongiform-encephalopathy.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE START DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE CONFIRMATION DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE END DATE 2023/03/13</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/02/netherlands-bovine-spongiform.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...please see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WEDNESDAY, AUGUST 23, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CWD TSE PRION OF CERVID, ZOONOSIS, don't let history repeat itself!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/08/cwd-tse-prion-of-cervid-zoonosis-dont.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/08/cwd-tse-prion-of-cervid-zoonosis-dont.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 28, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EFSA TSE Report 2022 First published 28 November 2023 The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-tse-report-2022-first-published-28.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-tse-report-2022-first-published-28.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">***> Professor John Collinge on tackling prion diseases 2023 UPDATE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is accumulating evidence also for iatrogenic AD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">2023 TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE IN THE USA ZOONOSIS</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Marina Betancor, Belén Marín, Alicia Otero, Carlos Hedman, Antonio Romero, Tomás Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan José Badiola, Olivier Andréoletti & Rosa Bolea * Veterinary Research volume 54, Article number: 89 (2023) Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle. Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease and tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prion seeding activity was detected during in vitro propagation of brain samples from the inoculated animals. These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Snip…</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Further in vivo experiments challenging different mouse lines have been started in order to confirm the infectivity of the PMCA products obtained in this study. However, in conclusion, our findings show that the propagation of atypical scrapie in cattle leads to the emergence of BSE-like seeding activity.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This is a concerning issue with far-reaching implications for public health and food safety. The possibility of interspecies transmission of prion diseases and the emergence of new prion strains highlight the critical need for continued surveillance and monitoring of these diseases in both animal and human populations. Early detection of prion diseases is crucial, and highly sensitive detection techniques such as PMCA can play an important role in this regard.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;">FRIDAY, MAY 19, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"> </div></div><a href="https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">SATURDAY, MAY 20, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;">May 2, 2023, i submitted this to the USDA et al;</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Docket No. APHIS–<span dir="ltr" style="outline: none !important;">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div></div><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full submission;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div></div></div></div><div dir="ltr" style="outline: none !important;"><br style="font-family: arial; font-size: 16px; outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;">Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">=====end</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">=====end</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">Experimental transmission of ovine atypical scrapie to cattle Experimental transmission of ovine atypical scrapie to cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy McKenna, Lauren Read, Sara Canoyra, Alba Marín-Moreno & Juan María Torres </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Veterinary Research volume 54, Article number: 98 (2023) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Classical bovine spongiform encephalopathy (BSE) in cattle was caused by the recycling and feeding of meat and bone meal contaminated with a transmissible spongiform encephalopathy (TSE) agent but its origin remains unknown. This study aimed to determine whether atypical scrapie could cause disease in cattle and to compare it with other known TSEs in cattle. Two groups of calves (five and two) were intracerebrally inoculated with atypical scrapie brain homogenate from two sheep with atypical scrapie. Controls were five calves intracerebrally inoculated with saline solution and one non-inoculated animal. Cattle were clinically monitored until clinical end-stage or at least 96 months post-inoculation (mpi). After euthanasia, tissues were collected for TSE diagnosis and potential transgenic mouse bioassay. One animal was culled with BSE-like clinical signs at 48 mpi. The other cattle either developed intercurrent diseases leading to cull or remained clinical unremarkable at study endpoint, including control cattle. None of the animals tested positive for TSEs by Western immunoblot and immunohistochemistry. Bioassay of brain samples from the clinical suspect in Ov-Tg338 and Bov-Tg110 mice was also negative. By contrast, protein misfolding cyclic amplification detected prions in the examined brains from atypical scrapie-challenged cattle, which had a classical BSE-like phenotype. This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This is the first study in cattle inoculated with naturally occurring scrapie isolates that found the presence of prions resembling classical BSE in bovine brain although this was limited to detection by the ultrasensitive PMCA. The results from thermostability assay confirmed that the isolates were as thermoresistant as the BSE agent as proven in other studies [36, 48]. Previous PMCA studies with various British atypical scrapie isolates did not find any evidence of amplification [49, 50]. This may be explained by the use of ovine brain as substrate rather than brain from Bov-Tg110 mice, which may facilitate conversion to classical BSE prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Two hypotheses for prion strain propagation in cross-species transmission experiments have been proposed: conformational selection favours a particular strain conformation out of a mixture of conformations in a scrapie isolate whilst mutation results in the conformational shift of one conformation into another [51]. Following on from the study in mice [17], it has been subsequently suggested that classical BSE properties that arise in atypical scrapie isolates transmitted to cattle may be due to conformational mutation in a new host [52]. It does not confirm that the atypical scrapie agent is the origin of the classical BSE epidemic and further transmission studies would be required to see whether classical BSE can be generated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Would PMCA applied to brains from cattle exposed to TSE agents other than classical BSE and atypical scrapie also produce a classical BSE-like molecular phenotype? The PMCA product obtained in the thermostability test using a thermosensitive classical scrapie control showed a profile unlike classical BSE. Atypical BSE has been linked to the origin of classical BSE because of its conversion into classical BSE following serial passages in wild-type mice (L-type BSE [11]) and bovine transgenic mice (H-type BSE [53]). Although we have not tested PMCA products of atypical BSE isolates as part of this study, there is no evidence that PMCA products from atypical BSE convert into classical BSE, at least for H-type BSE using bovine brain as substrate [54]. In fact, we were unable to propagate H-type BSE using the same methodology (S Canoyra, A Marín-Moreno, JM Torres, unpublished observation).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The study results support the decision to maintain the current ban on animal meal in feedstuffs for ruminants, particularly as atypical scrapie occurs world-wide, and eradication is unlikely for a sporadic disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In summary, experimental inoculation of cattle with the atypical scrapie agent may produce clinical disease indistinguishable from classical BSE, which cannot be diagnosed by conventional diagnostic tests, but prions can be amplified by ultrasensitive tests in both clinically affected and clinically unremarkable cattle, which reveal classical BSE-like characteristics. Further studies are required to assess whether a BSE-like disease can be confirmed by conventional tests, which may initially include a second passage in cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3</a></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presentation Type: Oral Presentation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: ALMA/APRI: 201400006, HC 414250</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">34 Scientific Commission/September 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. Atypical BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations expand</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMID: 21266763</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full text;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div><div dir="ltr" style="outline: none !important;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) <br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (<span dir="ltr" style="outline: none !important;">Tg338</span> and <span dir="ltr" style="outline: none !important;">Tg501</span>). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: No evidence of transmission was found on a first passage in <span dir="ltr" style="outline: none !important;">Tg338</span> nor <span dir="ltr" style="outline: none !important;">Tg501</span>ovinized mice, but on second passage, <span dir="ltr" style="outline: none !important;">4/10</span> <span dir="ltr" style="outline: none !important;">Tg338</span> mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and <span dir="ltr" style="outline: none !important;">1/12</span> <span dir="ltr" style="outline: none !important;">Tg501</span> mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed. </div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***thus questioning the origin of human sporadic cases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2015 CONFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"></div><div style="outline: none !important;">PRION <span dir="ltr" style="outline: none !important;">2016 TOKYO</span></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, April 23, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SCRAPIE <span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr" style="outline: none !important;">1933-6896</span> 1933-690X </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Tuesday, December 16, 2014</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Evidence for zoonotic potential of ovine scrapie prions</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the humanprion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. ***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. ***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Subject terms: Biological sciences• Medical research At a glance</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">why do we not want to do TSE transmission studies on chimpanzees $</div><div style="outline: none !important;"> </div><div style="outline: none !important;">5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">R. BRADLEY</div><div style="outline: none !important;"> </div><div style="outline: none !important;"> <a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">1: J Infect Dis 1980 Aug;142(2):205-8</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">PMID: 6997404</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">76/10.12/4.6</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">Nature. 1972 Mar 10;236(5341):73-4.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Gibbs CJ Jr, Gajdusek DC.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)</div><div style="outline: none !important;"> </div><div style="outline: none !important;">C. J. GIBBS jun. & D. C. GAJDUSEK</div><div style="outline: none !important;"> </div><div style="outline: none !important;">National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland</div><div style="outline: none !important;"> </div><div style="outline: none !important;">SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://nor-98.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://nor-98.blogspot.com/</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">2001</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Suspect symptoms</div><div style="outline: none !important;"> </div><div style="outline: none !important;">What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?</div><div style="outline: none !important;"> </div><div style="outline: none !important;">28 Mar 01</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Like lambs to the slaughter</div><div style="outline: none !important;"> </div><div style="outline: none !important;">31 March 2001</div><div style="outline: none !important;"> </div><div style="outline: none !important;">by Debora MacKenzie Magazine issue 2284.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."</div><div style="outline: none !important;"> </div><div style="outline: none !important;">There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">REPORT OF THE ADVISORY COMMITTEE ON SCRAPIE 1976</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">IN CONFIDENCE</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Such considerations suggest first that those responsible for work with scrapie should be selected with as much care as are astronauts. </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">THURSDAY, NOVEMBER 9, 2023 <br style="outline: none !important;" /></div></div></div></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">European Food Safety Authority (EFSA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">APPROVED: 25 October 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="font-family: arial; font-size: 16px; outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Monday, November 13, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none !important;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none !important;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none !important;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none !important;">tg650</span> with fecal homogenates. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Accepted: 7 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><p class="ydpafe6dc81yiv6594540084ydp858d20ecyiv0662081683ydp7fad6b93yiv2780175211p1" style="font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydpafe6dc81yiv6594540084ydp858d20ecyiv0662081683ydp7fad6b93yiv2780175211s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</span></p></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH and USDA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">end... <br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Fortuitous generation of a zoonotic cervid prion strain </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (<span dir="ltr" style="outline: none !important;">Tg12</span>), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the <span dir="ltr" style="outline: none !important;">Tg12</span> mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (<span dir="ltr" style="outline: none !important;">Tg12</span>) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the <span dir="ltr" style="outline: none !important;">Tg12</span> mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively</div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time."<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A probable diagnostic marker for CWD infection in humans </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xu Qi, Liuting Qing, Manuel Camacho, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Multiple in vitro CWD-seeded human PrP conversion experiments and some animal model studies indicate that the species barrier for CWD to human transmission can be overcome, but whether CWD prion can infect humans in real life remains controversial. The very limited understanding on the likely features of CWD infection in humans and the lack of a reliable diagnostic marker for identification of acquired human CWD cases contribute to this uncertainty. We aim to stablish such a reliable diagnostic marker for CWD infections in humans should they occur. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: A couple of PrPSc-positive spleens were identified from humanized transgenic mice inoculated with either CWD or sCJDMM1. Prions in these spleens were compared by bioassays in cervidized or humanized transgenic mice. A couple of PrPSc-positive spleens from UK sCJDMM1 patients were also examined similarly as controls with no exposure to CWD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: We have detected two prion-positive spleens in humanized transgenic mice inoculated with some CWD isolates. Such experimentally generated splenic “humanized” CWD prions (termed eHuCWDsp) appear indistinguishable from prions in the brain of sCJDMM1 patients on Western blot. We compared eHuCWDsp with prions in the spleen from humanized mice infected with sCJDMM1 (termed sCJDMM1sp) by bioassays in cervidized or humanized transgenic mice. Significantly, we found that eHuCWDsp can efficiently infect not only the humanized mice but also cervidized transgenic mice, and cervidized mice infected by eHuCWDsp produced PrPSc and brain pathology that are practically identical to those of CWD-infected cervidized mice. In contrast, sCJDMM1sp, similar to prions from sCJDMM1 patient brains, is poorly transmissible in the cervidized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data demonstrate that high transmissibility with CWD features of splenic prions in cervidized transgenic mice is unique to acquired human CWD prions, and it may serve as a reliable marker to identify the first acquired human CWD cases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====end </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Prion 2023 Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Sarah Zurbuchena,b , S. Jo Moorea,b , Jifeng Biana , Eric D. Cassmanna , and Justin J. Greenleea . a. Virus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, US b. Oak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, United States </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (<span dir="ltr" style="outline: none !important;">Tg12</span>; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection, analysis, decision to publish, or preparation of the manuscript. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Ami Frank and Kevin Hassall for their technical contributions to this project.</div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====end </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">''Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">31 TAC §§65.82, 65.85, 65.88</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results show positive prion detection in all products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none !important;"><br style="font-family: arial; font-size: 16px; outline: none !important;" /></div></div></div><div style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none !important;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none !important;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none !important;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none !important;">tg650</span> with fecal homogenates. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management. </div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;"> <a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">WEDNESDAY, NOVEMBER 01, 2023 </div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">TEXAS CHRONIC WASTING DISEASE RISES SUBSTANTIALLY TO 575 CONFIRMED CWD CASES TO DATE<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">FRIDAY, DECEMBER 08, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">TEXAS CWD TSE PRION DIRE CONSEQUENCES ARE HERE! </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div></div></div></div></div></div></div><div style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">NOW, THINK CONSUMPTION, EXPOSURE, FRIENDLY FIRE, PASS IT FORWARD, IATROGENIC CJD!</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Friday, October 20, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An investigation has been opened into the death of a scientist who was studying a transmissible and deadly disease CJD in Spain DEGENERATIVE DISEASES</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An investigation has been opened into the death of a scientist who was studying a transmissible and deadly disease in Spain </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Three institutions are trying to ascertain the origin of the infectious Creutzfeldt-Jakob disease samples discovered in the biochemist’s laboratory. The 45-year-old investigator died in 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The University of Barcelona’s School of Medicine, in L’Hospitalet de Llobregat, where laboratory 4141 is located.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MASSILIANO MINOCRI</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Manuel Ansede</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MANUEL ANSEDE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Madrid - OCT 19, 2023 - 16:15 EDT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A prestigious Spanish researcher of Creutzfeldt-Jakob disease died last year after experiencing symptoms consistent with this deadly ailment, as EL PAÍS has learned from multiple sources at the three institutions involved. Three months ago, the University of Barcelona opened an internal investigation to ascertain the origin of thousands of unauthorized samples, some of them infectious, discovered in a freezer in its laboratory 4141, where the deceased biochemist worked. He was a member of the Bellvitge Biomedical Research Institute (IDIBELL) and the CIBER public consortium. These two institutions have joined the internal investigation, after noting concern among colleagues at the facility, who did not know the level of risk to which they were exposed without their knowledge. This neurodegenerative disease incubates silently for years, but when symptoms appear — rapid dementia and muscle stiffness — it is fatal. Life expectancy after diagnosis is barely six months. Its best-known animal equivalent is mad cow disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The biochemist joined the 4141 lab at the University of Barcelona in January 2018 as a principal investigator with a group of his own; his wife joined shortly after. Together, they identified characteristic substances in human cerebrospinal fluid, useful for the diagnosis of rapid dementia. In November 2020, the now deceased scientist began to feel unwell and asked to leave. After his colleagues found out that his symptoms were consistent with Creutzfeldt-Jakob disease, he demanded absolute privacy and decided to hide his diagnosis, according to the sources consulted for this article. He died at the age of 45.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">On December 18, 2020, the head of the 4141 laboratory, Isidre Ferrer, a professor of Pathology at the University of Barcelona and a member of IDIBELL, informed the directors of both institutions that suspicious samples of cerebrospinal fluid from people with Creutzfeldt-Jakob disease and other neurodegenerative types of dementia had been discovered by chance in a freezer at 80 degrees below zero, according to internal documentation to which EL PAÍS had access. The thousands of unauthorized samples from patients and animals were in a drawer reserved for the sick researcher’s group and lacked records indicating their presence. The University of Barcelona then ordered the immediate closure and decontamination of laboratory 4141, located in the School of Medicine at L’Hospitalet de Llobregat.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Doctor Gabriel Capellá, the director of IDIBELL, explains that they have identified “a maximum of eight people” who worked in the laboratory at that time, in addition to the deceased scientist and Isidre Ferrer. Some of these coworkers have required months of psychological care. The university’s safety office and IDIBELL’s prevention service determined that there was “an unacceptable risk,” although Capellá emphasizes that “there is no record of any occupational accident” in which a researcher could have been infected with contaminated material. Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies are caused by abnormal proteins called prions, which accumulate in the brain and cause a microscopic sponge-like appearance. There are only one or two cases per million inhabitants, the vast majority of which are of unknown cause, but cases of the disease have also been reported after contact with surgical instruments contaminated by these prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The three institutions involved took more than two years to send the suspect samples for analysis to a specialized center, the CIC bioGUNE, in Derio, Spain. A spokeswoman for the University of Barcelona says that they sent them in December 2022 and the three organizations received the results in March 2023. Four months later, in July, the legal departments at the three institutions finally informed the 4141 laboratory workers that the Creutzfeldt-Jakob disease samples were potentially infectious, as feared. “You can debate whether we have been quick [in our response] or not, but we have been transparent. We are [part of] three institutions that had to agree, and we have acted as guarantors,” says Capellá. A similar situation also occurred in France; following the death of a researcher from Creutzfeldt-Jakob in 2019 and the discovery of another suspected case, all public laboratories investigating prion diseases decided to temporarily close in July 2021 to review their protocols.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laboratory 4141 was not equipped to handle high biohazard samples. It did not even have a biosafety hood. At the end of 2018, the CIBER public consortium signed an agreement so that the group could work with these dangerous samples at the high-security laboratory of the Animal Health Research Center (CReSA) in Bellaterra, Spain, near Barcelona. According to the sources we consulted, there was no reason to have the contaminated material in laboratory 4141, beyond saving time during experiments, since the CReSA bunker is 30 kilometers (about 19 miles) away and required waiting one’s turn to use. Isidre Ferrer, the head of the facility at the time, who has since retired, prefers not to comment on the case until the internal investigation is completed, but he emphasizes that he was unaware of the existence of these dangerous samples.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The IDIBELL director recalls that the deceased scientist was “a promising and brilliant researcher.” From 2013 to 2017, he worked at the University Medical Center of Göttingen (Germany) under neurologist Inga Zerr, a leading international expert in Creutzfeldt-Jakob disease. Physician Margarita Blázquez, who manages the CIBER public consortium, notes that the disease’s incubation period can last several years, so, if the deceased researcher really had it, he also could have become infected with it in Germany or at another of his previous laboratories. This newspaper has tried to contact the scientist’s widow via email but has not received a response. She asked to be discharged shortly after her husband did. The three institutions are now investigating whether the couple handled the dangerous samples without authorization in lab 4141. A third person affiliated with CIBER, a member of the now-deceased biochemist’s research group, worked with potentially infectious Creutzfeldt-Jakob samples without being informed that they were infectious.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The security office of the University of Barcelona believes that the samples would only have been a problem in the case of accidental inoculation or ingestion while handling them. But internal documents confirm the alarm the situation has caused on campus. “The laboratory technicians and investigators express their enormous concern about the fact that, so far, it has not been possible to determine the origin of the doctor’s illness. They are left to worry about whether they may suffer the same fate in a few years’ time as a result of uncontrolled contamination that may have been created in the laboratory,” according to the minutes of a December 22, 2020, meeting between workers and Carles Solsona, the director of the Department of Pathology at the University of Barcelona. “This fear causes them to suffer a state of permanent anguish, causing insomnia and irritability.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The IDIBELL director sent a message to the center’s entire staff on the 11th, five days after EL PAÍS informed him that it was investigating the case. Gabriel Capellá then told his workers of “a very serious incident that became known on campus for the first time at the end of 2020.″ With “deep dismay,” Capellá announced the researcher’s death “due to a possible prion condition,” with “a possible iatrogenic [a disease acquired by contact with contaminated materials during a medical procedure].” The director also reported finding “potentially dangerous samples” in a freezer. “Our priority is to ensure that this situation is handled rigorously and transparently to limit the damage to the reputation of our institutions,” he said.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Do you have more information about this case or other similar ones? You can write to us at mansede@elpais.es.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sign up for our weekly newsletter to get more English-language news coverage from EL PAÍS USA Edition</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://english.elpais.com/science-tech/2023-10-20/an-investigation-has-been-opened-into-the-death-of-a-scientist-who-was-studying-a-transmissible-and-deadly-disease-in-spain.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://english.elpais.com/science-tech/2023-10-20/an-investigation-has-been-opened-into-the-death-of-a-scientist-who-was-studying-a-transmissible-and-deadly-disease-in-spain.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision CORRESPONDENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jacqueline Mikol1 · Jérôme Delmotte1 · Dolorès Jouy1 · Elodie Vaysset1 · Charmaine Bastian1 · Jean‑Philippe Deslys1 ·</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel Comoy1 Received: 10 July 2020 / Revised: 8 September 2020 / Accepted: 25 September 2020 / Published online: 6 October 2020 © The Author(s) 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Non-human primates appeared as the closest model to study human iatrogenic prion diseases [14]: we report here the consequences of variant Creutzfeldt–Jakob disease/bovine spongiform encephalopathy (vCJD/BSE) inoculation in a cynomolgus macaque finger, with the demonstration of an original mode of propagation and the practical risk for professional exposure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The distal right middle finger handpad of a 4-year-old macaque was incised on both lateral sides to induce local inflammation, and then injected with the equivalent of 10 mg of a BSE, orally challenged macaque brain [18]. After an 18 months period of finger clumsiness, the clinical disease (behaviour abnormalities, fear, hyperesthesia, gait disturbances, shaking) began 7.5 years after inoculation and euthanasia took place 2 months later for welfare reasons. Motor conduction velocity of the right median nerve was reduced to one-third of the left counterpart and sensory potential was not detected.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Histological and biochemical studies were performed as previously described. All the elements of the triad were present [7–9]: spongiform change was moderate in neocortex, striatum, brain stem, mild in spinal cord but severe in thalamus and cerebellum; neuronal loss was globally moderate, but severe in cerebellum and sacral spinal cord (vacuolated neurons); gliosis was severe in thalamus, cerebellum and brain stem and moderate elsewhere (Supplementary Fig. 1). ELISA and western blot (WB) showed the expected accumulation of PrPres with BSE glycophoretic pattern at all levels of brain and spinal cord (Supplementary Fig. 2).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the brain, PrPd deposits were laminar into the cortical deep layers, massive into thalamus, basal ganglia, cerebellum, and brain stem. In spinal cord, PrPd was symmetrically distributed, intense in the Substantia gelatinosa and nucleus dorsal of Clarke while decreased at sacral level. Deposits were diverse into the whole CNS: synaptic, perineuronal, reticular aggregates, mini-plaques, plaques, and incomplete florid plaques. The retinal plexiform layers were labelled (Supplementary Fig. 1i). There were no amyloid or tau deposits.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unusual PrPd deposits were observed along dendrites, short and long axons, neuritic threads tracing fne networks of straight lines or like strings of pearls (Supplementary Fig. 3). They were present into deep neocortex, basal ganglia, and motoneurons. Such long processes are not frequent but have been reported in human [13] and experimental studies [10, 22]. PrPd deposits were also noted as very mild into striato-pallidal projections, both limbs of internal capsule and fornix (Supplementary Fig. 3). The presence of PrPd in white matter has been reported (Supplementary text 4).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Peripherally, the expected PrPd was undetectable in lymphoid organs, including spleen, through biochemical or immunohistochemical analyses, while prion replication was detected in the peripheral nervous system (PNS): PrPd staining was visualized in many dorsal root ganglia (DRG) but only in nerves innervating the forelimb site of injection (median and ulnar nerves). At the cellular level, PrPd was limited to ganglia and satellite cells in DRG and Schwann cells (Scs) all along nerves whereas axons were never labelled (Fig. 1). Previously, using postmortem immunohistochemical studies (listed in Supplementary text 5), PrPd has been shown in peripheral nervous system in all forms of human neuropathies, albeit more frequently in vCJD, mostly in posterior root nerve fbres at adaxonal location and/or in ganglion and satellite cells. The restricted amount of PrPd was repeatedly underlined but, recently, prion RTQuiC was positive in all nerves examined [2]. PrPd has also been described, frst in scrapie [17] then in BSE, as limited “adaxonal deposits” or/and Sc deposits, with or without DRG cell involvement (review in [4] and Supplementary text 6). Previous studies of the mode of propagation of PrPd have reported variable observations and analyses depending on strains, host species and genotype (Supplementary text 6); the authors discussed the role of the sensory route of trafficking of prions, the modifications of axonal transport, the centrifugal versus centripetal spread of PrPd .</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">After peripheral infection, accumulation of infectious agent is reputed to occur in lymphoid tissues before direct neuroinvasion [18, 19], even with very little apparent peripheral lymphoreticular deposition [6, 20]. Here, there is no apparent replication/amplification of vCJD/BSE agent in the lymphoid tissues of the exposed macaque. In this model, the neural contamination occurred directly in the highly innervated finger while neuroinvasion appears to occur in Scs along the median nerve to the DRG, with the appearance of the classical labelling of ganglion cells which indicates the onset of the first level of neuronal infection. This model provides direct evidence of the hypothesis of a sequential infection of Scs from the periphery to the CNS, followed by a secondary diffusion into the spinal cord, as already considered by our group [15] and others [1, 3, 11, 12, 21]. It is to note that studies based on intra-sciatic nerve injections in hamsters [16] and transgenic mice [12] had established a rate of transport of infectivity of, respectively, 0.5–2 mm and 0.7 mm per day. This key role of Scs could explain both the low speed of propagation and the discrepancy between the paucity of PrPd into the distal part of the sensory nerves followed by the positivity of DRG, satellite cells and proximal roots.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In conclusion, we have observed that the exposure of a primate to vCJD/BSE through a distal finger lesion induces, after more than 7.5 years of silent incubation, a massive deposit of PrPd , strictly restricted to the nervous system and the eye.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our data suggest a new type of pure unique peripheral nervous contamination in which the Scs would have a major role in the mode of centripetal progression of PrPd in the peripheral nervous system. Moreover, considering the fact that, recently, “a variant CJD diagnosed 7.5 years after occupational exposure” (cryomicrotomy) in a technician was observed [5], this experimental case report supports the risk linked to professional exposure and reinforces the necessity of adequate measures of prevention. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Second death in France in a laboratory working on prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease has killed a person who handled this infectious agent at Inrae in Toulouse. After a first death in 2019, a moratorium on work on this pathogen has been extended.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">By Hervé Morin</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working with these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of mice infected with prions, in another unit of INRAE, in Jouy-en-Josas.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Computer representation of part of a prion protein on a light micrograph of pyramidal nerve cells (neurons, in black) in the cerebellum of the brain. ALFRED PASIEKA / SCIENCE PHOTO LIBRARY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Temporary suspension of work on prions in French public research laboratories</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRESS RELEASE - The general directorates of ANSES, CEA, CNRS, INRAE and Inserm, have decided jointly and in agreement with the Ministry of Higher Education, Research and Innovation to suspend as a precaution all their research and experimentation work relating to prion diseases, for a period of three months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This precautionary measure is motivated by the knowledge of a possible new case of a person suffering from Creutzfeldt-Jakob disease and who worked in a laboratory for research on prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted on July 27, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The suspension period put in place as of this day will make it possible to study the possibility of a link between the observed case and the person's former professional activity and to adapt, if necessary, the preventive measures in force in the research laboratories. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The person with Creutzfeldt-Jakob disease (CJD)1, whose form is not yet known, is a retired INRAE agent. This could be the second case of infectious CJD affecting a scientist who worked on prions, after that of an assistant engineer who died of the disease in 2019, and who was injured in 2010 during of an experiment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Following this death, a general inspection mission was launched in July 2019 by the ministries of research and agriculture with French laboratories handling prions. Submitted in October 2020, the report concluded on the regulatory compliance of the laboratories visited as well as the presence of a risk control culture within the research teams.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research around prion proteins, with high public health issues, allows major advances in the understanding of the functioning of these infectious pathogens, and contributes to results that are transferable to other related degenerative diseases such as Alzheimer's and Alzheimer's diseases. Parkinson's.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At the level of each establishment, regular and transparent information will be provided to all the working communities concerned by this measure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 The disease Creutzfeldt-Jakob disease (CJD) is one of prion diseases - still called encephalopathies subacute spongiform transmitted(TSE) - of diseases rare, characterized by a degeneration rapid and fatal the system nervous central. They are caused by the accumulation in the brain of a normally expressed protein but poorly conformed - the prion protein - which leads to the formation of deleterious aggregates for neurons. For now , no treatment will allow to change the course of these diseases. It can be of origin sporadic , form the most frequent , original genetic or finally to form infectious following a contamination. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">By Barbara CasassusJul. 28, 2021 , 4:35 AM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">With reporting by Martin Enserink.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted in: EuropeHealthScientific Community</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">doi:10.1126/science.abl6587</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TO THE EDITOR:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Figure 1.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">5 References</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">July 2, 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">N Engl J Med 2020; 383:83-85</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DOI: 10.1056/NEJMc2000687</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Metrics</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">34 year old Doctor Orthopedic Surgeon dies from CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dr. Adam Thomas Dialectos</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1987 - 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BORN</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">April 29, 1987</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DIED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">June 21, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FUNERAL HOME</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Bean Funeral Homes & Crematory Inc</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1605 Rockland St</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reading, PA 19604</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">UPCOMING SERVICE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Visitation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Jun, 24 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">9:00a.m. - 11:00a.m.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saints Constantine & Helen Greek Orthodox Church</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Send Flowers</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Share</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">On Monday June 21, 2021, Dr. Adam Thomas Dialectos, loving husband, father, son, brother, uncle, Nouno, friend at the age of 34. Adam was born on April 29, 1987 in Reading, PA to Athan and Gretchen Dialectos. Adam was a 2005 graduate of Governor Mifflin High School, before receiving his degree in Health Sciences from James Madison University in 2009. Adam attended Philadelphia College of Osteopathic Medicine for medical school and his subsequent residency in orthopedic surgery. Adam was completing his Spine Surgery Fellowship at New England Baptist Hospital in Boston, Massachusetts. On February 7, 2019 Adam married the love of his life and girlfriend of 12 years, Lindsey (Schuler) Dialectos. They brought a beautiful baby boy into this world on January 6, 2021, Athananosis Adam Dialectos. Adam’s passion in life was unceasingly seeking to help others, emphasized by his desire to be a surgeon— a decision he made in his early elementary years. Adam continued this love of medicine throughout his life, which led to his achieving of the Henrietta and Jack Avart Memorial Award in 2019, awarded to the Orthopedic surgery resident who exhibited unparalleled excellence in their field during the residency program. This passion to learn, teach and support was truly understood through the patients whose lives Adam touched. When it came to his patients and coworkers, there was never a job too small for Adam. Those who knew Adam saw his personality shine through in so many other aspects of his life. Adam loved traveling. Some of his most memorable trips were with his wife, and countless snowboard trips with his brother, family, and friends. Adam loved everyone he was around; he loved and was loved by so many. Adam was truly one in a million. Adam is survived by his loving wife, Lindsey, and their son, Athan Adam; His father and mother, Athan and Gretchen; His brother Jordan and sister-in-law Megan, and their daughter Livia, Adam’s Goddaughter. His sister, Rachel, and her significant other, Bo Wagner. Furthermore, Adam is survived by his Yiayia, Joanne Dialectos, wife of the late George Dialectos; his Pop Pop, Donald Harford, husband of the late Nancy Servent; his Aunt Angel and Uncle Scott Helm; his Aunt Kelly and Uncle Darrell Markley. Adam was preceded in death by his Aunt Maria and Uncle Bob Care. Funeral Service will be held at Saints Constantine & Helen Greek Orthodox Church, 1001 East Wyomissing Blvd. Reading on Thursday June 24th. Father Theodore Petrides and Father Thomas L. Pappalas will officiate. Interment will follow at Charles Evans Cemetery. The family will receive relatives and friends at Saints Constantine & Helen Greek Orthodox Church from 9:00am to 11:00am with services beginning at 11:00. In lieu of flowers, contributions may be made to the CJD Foundation at 3634 West Market Street Suite 110 Akron, Ohio 44333 or cjdfoundation.org in remembrance of Dr. Adam Dialectos. Donations may also be made to Saints Constantine & Helen Greek Orthodox Church. Bean Funeral Home, 1605 Rockland Street, Hampden Heights, is in charge of arrangements and online condolences may be made at www.beanfuneralhomes.com.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To plant trees in memory, please visit our Sympathy Store.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published by Reading Eagle from Jun. 22 to Jun. 24, 2021.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.legacy.com/us/obituaries/readingeagle/name/adam-dialectos-obituary?pid=199144663" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.legacy.com/us/obituaries/readingeagle/name/adam-dialectos-obituary?pid=199144663</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our sincere condolences to the Family and Friends of Dr. Adam Thomas Dialectos. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I can't help but ponder, as a Orthopedic Surgeon, Spine Surgery Fellowship, and what the good Doctors work curtailed, i can't help but think this is a potential case of iatrogenic CJD. surgery on humans, i would imagine cadavers as well.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, provern, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd. ...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">least we forget...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery *** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, December 18, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Direct neural transmission of vCJD/BSE in macaque after finger incision </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/12/direct-neural-transmission-of-vcjdbse.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/12/direct-neural-transmission-of-vcjdbse.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tuesday, November 30, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Second death in France in a laboratory working on prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Second lab worker with deadly prion disease prompts research pause in France</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A lab worker died of prion disease in 2019, nine years after a lab accident.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BETH MOLE - 7/29/2021, 5:16 PM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://arstechnica.com/science/2021/07/second-lab-worker-with-deadly-prion-disease-prompts-research-pause-in-france/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://arstechnica.com/science/2021/07/second-lab-worker-with-deadly-prion-disease-prompts-research-pause-in-france/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A 2020 paper published in the New England Journal of Medicine left little doubt that Jaumain had been infected on the job. She had variant CJD, but since Europe’s ‘mad cow’ outbreak ended after 2000 and the disease virtually disappeared, the paper said it was virtually impossible for someone her age in France to contract food-borne vCJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Science also said two independent reports – one by government inspectors – had found no safety violations at the lab where Jaumain worked. The press release also noted that the inspectors concluded there was “the presence of a risk control culture within the research teams”. The Jaumain family’s lawyer called the neutrality of the reports into question, however.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At the same time, the government inspectors’ report also revealed that there had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, raising concerns about how effective this risk control culture is. Five of these occurred when workers “stabbed or cut themselves with contaminated syringes or blades”.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://science.thewire.in/the-sciences/second-case-of-fatal-disease-prompts-french-moratorium-on-prion-research/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://science.thewire.in/the-sciences/second-case-of-fatal-disease-prompts-french-moratorium-on-prion-research/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, July 28, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wednesday, July 28, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, AUGUST 01, 2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/08/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/08/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, JULY 19, 2020 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Joseph J. Zubak Orthopaedic surgeon passed away Monday, July 6, 2020, Creutzfeldt-Jakob Disease (CJD)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nejm.org/doi/suppl/10.1056/NEJMc2000687/suppl_file/nejmc2000687_appendix.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nejm.org/doi/suppl/10.1056/NEJMc2000687/suppl_file/nejmc2000687_appendix.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THURSDAY, JULY 02, 2020 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://vcjd.blogspot.com/2020/07/variant-creutzfeldtjakob-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vcjd.blogspot.com/2020/07/variant-creutzfeldtjakob-disease.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thursday, July 29, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TSE PRION OCCUPATIONAL EXPOSURE VIA ANIMAL OR HUMAN, iatrogenic transmission, nvCJD or sCJD, what if? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Deep Throat to Singeltary BSE Mad Cow 2001 to 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I remember what “deep throat” told me about Scrapie back around 2001, I never forgot, and it seems it’s come to pass;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Confidential!!!!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">---end personal email---end...tss </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(I never knew who this person was, but got me into the U.S. BSE Emergency 50 State conference call back 2001, and we corresponded for years about BSE TSE Prion, have not heard from in over a decade, but they were on the inside looking out. You can believe this or not, but this was real, i don’t make this stuff up…plus my endeavors to get those 1 million cattle tested for BSE failed. There was an ENHANCED BSE SURVEILLANCE put forth after 2003, we pushed for it, but it was abruptly shut down after the atypical BSE cases were popping up…a bit of history for anyone interested…terry)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at..... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!! And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!" </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">U.S. 50 State Emergency BSE Conference Call 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary Comment Docket No: 2002N-0273 (formerly Docket No. 02N-0273)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MY comments/questions are as follows ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Suppressed peer review of Harvard study October 31, 2002 *** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today ? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full text; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary Full Comments Submissions;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FSIS, HARVARD, REPLY TO SINGELTARY </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full submission;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission Comment from Terry Singeltary Posted by the Animal and Plant Health Inspection Service on Jun 19, 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2018-0087-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2018-0087-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Control of Chronic Wasting Disease OMB Control Number: 0579-0189APHIS-2021-0004 Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2021-0004-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0004-0002</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/document/APHIS-2018-0011-0003" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/document/APHIS-2018-0011-0003</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Comment from Singeltary Sr., Terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PUBLIC SUBMISSION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Comment from Terry Singeltary Sr.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by the Food and Drug Administration on May 17, 2016 Comment</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.regulations.gov/comment/FDA-2003-D-0432-0011" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/FDA-2003-D-0432-0011</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">***> Monday, November 13, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, APRIL 07, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, APRIL 24, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">THE PATHOLOGICAL PROTEIN</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">June 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BY Philip Yam</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CHAPTER 14 LAYING ODDS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.thepathologicalprotein.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.thepathologicalprotein.com/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate infor-mation showed that “we miss about 14 percent,” said CDC epidemiolo-gist Lawrence Schonberger. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/chapter/10.1007/0-387-21755-x_14" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/chapter/10.1007/0-387-21755-x_14</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, retired (medically), CJD WATCH</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Submitted March 26, 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">August 10, 2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Greetings,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">But, while sub-clinical, how many can one exposed human infect? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">please see history, and the ever evolving TSE science to date ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, June 13, 2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary 2000</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BMJ 2000; 320 doi: <a href="https://doi.org/10.1136/bmj.320.7226.8/b" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1136/bmj.320.7226.8/b</a> (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">02 January 2000 Terry S Singeltary retired</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rapid Response: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Something else I find odd, page 16;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A few more factors to consider, page 15;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To be continued...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr. Bacliff, Texas USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Competing interests: No competing interests</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tracking spongiform encephalopathies in North America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xavier Bosch</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Available online 29 July 2003. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Volume 3, Issue 8, August 2003, Page 463 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Volume 3, Number 8 01 August 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Newsdesk</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tracking spongiform encephalopathies in North America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xavier Bosch</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a></div></div></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Singeltary 2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">by Philip Yam </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Revisiting Sporadic CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow.org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">223</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on www.vegsource.com and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">224 CHAPTER 14</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laying Odds 225</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">226 CHAPTER 14</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A Case for Undercounting</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laying Odds 227</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a one in-a-million lottery, it’s more like one-in-2.5-million for African Americans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...SEE FULL TEXT;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary Submission SEAC 2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tables of Cases Examined</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">National Prion Disease Pathology Surveillance Center Cases Examined¹</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Updated quarterly.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Last updated on: September 2nd, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1999 & earlier 383 232 202 27 3 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2000 145 102 90 12 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001 209 118 110 8 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2002 241 144 124 18 2 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2003 259 160 137 21 2 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2004 315 180 163 16 0 1³</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2005 330 179 157 21 1 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2006 365 179 159 17 1 2⁴</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2007 374 210 191 19 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2008 384 221 205 16 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2009 397 231 210 20 1 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2010 402 247 219 28 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2011 392 238 214 24 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">12/12/23, 11:05 AM Tables of Cases Examined | Pathology | School of Medicine | Case Western Reserve University</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined</a> 2/4</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2012 413 244 221 23 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2013 416 258 223 34 1 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2014 355 208 185 21 1 1⁵</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2015 401 262 243 19 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2016 395 277 248 29 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 375 266 247 18 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2018 308 221 202 18 1 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2019 434 281 259 22 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2020 367 253 228 24 1 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2021 344 248 224 22 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2022 337 228 206 21 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 193 114 101 8 0 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TOTAL 8534 5301 4720 507 14 4</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Year CSF Only and RT-QuIC Positive</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2015 140</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2016 183</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 227</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2018 266</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2019 310</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2020 309</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2021 331</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2022 347</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 240</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TOTAL 2357</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Listed based on the year of death or, if not available, on the year of referral;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cases with suspected prion disease for which brain tissue was submitted;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disease acquired in the United Kingdom;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Disease possibly acquired in a Middle Eastern or Eastern European country;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Includes 28 cases in which the diagnosis is pending (2 from 2022 and 31 from 2023), and 20 inconclusive cases;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Includes 8 (2 from 2021, 1 from 2022, and 5 from 2023) cases with type determination pending in which the diagnosis of vCJD has been excluded.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The sporadic cases include 4641 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 88 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 39 cases of sporadic Fatal Insomnia (sFI).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total does not include 312 Familial cases diagnosed by blood test only.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For a downloadable PDF version of our quarterly table, please click the link below:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2023-09/WebTable%20Legal.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2023-09/WebTable%20Legal.pdf</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><a href="https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">TEXAS CJD</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd/creutzfeldt-jakob-disease-cjd-data" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd/creutzfeldt-jakob-disease-cjd-data</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Letter</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">December 11, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et alJohn A. Romley, PhD4; John C. Probasco, MD5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Johns Hopkins University School of Medicine, Baltimore, Maryland</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2Department of Economics, Stanford University, Stanford, California</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3Department of Population, Family and Reproductive Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4Leonard D. Schaeffer Center for Health Policy and Economics, University of Southern California, Los Angeles, California</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">5Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA Neurol. Published online December 11, 2023. doi:10.1001/jamaneurol.2023.4678 Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><a href="https://jamanetwork.com/journals/jamaneurology/article-abstract/2812784" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jamaneurology/article-abstract/2812784</a><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">> Due to aging populations worldwide, the epidemiology of CJD is evolving.3 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div>let's examine the reality of zoonosis of the livestock TSE prion disease and iatrogenic CJD shall we...terry</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Angéline Denouel1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Jean-Philippe Brandel1,2 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Danielle Seilhean1 · Jean-Louis Laplanche3,4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Alexis Elbaz5 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Stéphane Haik1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We included all cases aged 45–89 years old who died with a probable/definite sCJD diagnosis based on the French national surveillance network. We used age-period-cohort (APC) Poisson regression models to study variation in mortality rates by sex, age, period, and time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A total of 2475 sCJD cases aged 45–89 years were included. Mortality rates increased with age, reached a peak between 75 and 79 years, and decreased thereafter. Mortality rates were higher in women than men at younger ages and lower at older ages. The full APC model with a sex×age interaction provided the best fit to the data, thus in favour of sex, age, period, and cohort effects on mortality rates. In particular, mortality rates increased progressively with successive birth cohorts.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords Age-period-cohort model · Prion · Temporal trend · Sporadic Creutzfeldt-Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Worldwide, the number of patients with sCJD appears to have progressively increased over time [13]. This increase can be partly explained by increasing life expectancy as well as by better case ascertainment due to improved diagnostic tests and awareness of the disease among clinicians. Indeed, a relationship between surveillance intensity and sCJD incidence has been shown [14]. It cannot be excluded, however, that an actual increase of sCJD cases has occurred, and this hypothesis can be examined using age-period-cohort (APC) models.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In this paper, we estimated mortality rates from sCJD in France over a 25-year period (1992–2016) based on data from the French national surveillance network.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s10654-023-01004-5" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s10654-023-01004-5</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: N/A</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: XXX</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 years ago;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001 Singeltary on CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 14, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, Sr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, JANUARY 15, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">if not, why not?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD FOUNDATION Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">UK CJD Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">cjd questionnaire 1979</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">RE: re-Human Prion Diseases in the United States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> routine passive mortality CJD surveillance USA ?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> THIS has been proven not to be very useful in the U.K.;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">IDENTIFICATION OF CASES</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD Questionnaire </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2007/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, SEPTEMBER 11, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary sCJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">CANADA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Definite and probable CJD, 1998-2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">As of November 30, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Year Sporadic Iatrogenic Genetic vCJD Total</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1998 22 1 1 0 24</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1999 27 2 3 0 32</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2000 32 0 3 0 35</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001 27 0 3 0 30</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2002 30 0 5 1 36</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2003 27 1 1 0 29</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2004 42 0 4 0 44</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2005 42 0 2 0 44</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2006 39 0 5 0 44</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2007 35 0 4 0 39</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2008 48 0 1 0 49</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2009 48 0 5 0 53</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2010 35 0 3 0 38</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2011 46 0 4 1 51</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2012 62 0 1 0 63</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2013 50 0 1 0 51</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2014 51 0 5 0 56</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2015 44 0 8 0 52</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2016 57 1 6 0 64</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 82 0 5 0 87</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2018 75 1 5 0 81</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2019 76 0 3 0 79</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2020 65 0 4 0 69</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2021 60 0 3 0 63</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2022 88 0 2 0 90</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 52 0 1 0 53</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total 1263 6 86 2 1357</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cases with definite and probable diagnosis to date</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023, STILL NO MENTION OF VPSPr TSE Prion in Canada statistics...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THIS problem must be addressed immediately imo.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">QUOTE;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;">WEDNESDAY, NOVEMBER 22, 2023 <br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt-Jakob Disease in Mexico (1990-2023)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/creutzfeldt-jakob-disease-in-mexico.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/creutzfeldt-jakob-disease-in-mexico.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">CREUTZFELDT-JAKOB DISEASE IN THE UK (By Calendar Year)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REFERRALS OF SUSPECT CJD DEATHS OF DEFINITE AND PROBABLE CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Year Referrals Year Sporadic1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Iatrogenic Genetic2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">vCJD Total Deaths</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1990 [53]† 1990 28 5 0 - 33</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1991 75 1991 31 1 4 - 36</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1992 96 1992 45 2 6 - 53</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1993 79 1993 36 4 7 - 47</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1994 119 1994 53 1 9 - 63</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1995 87 1995 35 4 5 3 47</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1996 132 1996 40 4 6 10 60</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1997 163 1997 59 6 7 10 82</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1998 155 1998 64 3 5 18 90</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1999 170 1999 62 6 2 15 85</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2000 178 2000 48 1 3 28 80</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2001 179 2001 58 4 6 20 88</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2002 164 2002 73 0 5 17 95</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2003 163 2003 79 5 7 18 109</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2004 114 2004 50 2 6 9 67</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2005 124 2005 67 4 13 5 89</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2006 112 2006 68 1 9 5 83</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2007 119 2007 63 2 11 5 81</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2008 150 2008 84 5 6 2 97</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2009 153 2009 78 2 8 3 91</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2010 150 2010 85 3 6 3 97</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2011 158 2011 91 4 14 5 114</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2012 127 2012 92 5 12 0 109</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2013 152 2013 108 2 10 1 121</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2014 130 2014 100 3 13 0 116</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2015 140 2015 105 0 4 0 109</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2016 148 2016 118 1 6 1 126</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 159 2017 122 0 12 0 134</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2018 167 2018 137 2 12 0 151</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2019 147 2019 128 1 7 0 136</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2020 172 2020 135 1 8 0 144</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2021 179 2021 133 0 6 0 139</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2022 182 2022 150 0 8 0 158</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023 145 2023 113 1 3 0 117</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Referrals 4741 Total</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Deaths</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2738 85 246 178 3247</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">† Referral figure for 1990 is from 1 May onwards...*As at 4th December 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Summary of vCJD cases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Deaths from definite vCJD (confirmed): 123</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Deaths from probable vCJD (without neuropathological confirmation): 55</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Number of deaths from definite or probable vCJD (as above): 178</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Number of definite/probable vCJD cases still alive: 0</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Total number of definite or probable vCJD (dead and alive): 178</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 There are, in addition, a total of 20 cases of vPSPr (death in 1997(1 case), 2004(1), 2006(1), 2008(3), 2010(1), 2012(4), 2013(1), 2016(3), 2017(1), 2018(1), 2019(1), 2020(2)) not included in the above figures.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2 includes all genetic prion disease, including GSS</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Case Report</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An autopsy case of variably protease-sensitive prionopathy with Met/Met homogeneity at codon 129</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Akiko Uchino, Yuko Saito, Saori Oonuma, Shigeo Murayama, Saburo Yagishita, Tetsuyuki Kitamoto, Kazuko Hasegawa</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">First published: 30 May 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1111/neup.12911" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1111/neup.12911</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The typical clinical manifestations of sporadic Creutzfeldt-Jakob disease (sCJD) are rapid-progressive dementia and myoclonus. However, the diagnosis of atypical sCJD can be challenging due to its wide phenotypic variations. We report an autopsy case of variably protease-sensitive prionopathy (VPSPr) with Met/Met homogeneity at codon 129. An 81-year-old woman presented with memory loss without motor symptoms. Seventeen months after the onset, her spontaneous language production almost disappeared. Diffusion-weighted images (DWI) showed hyperintensity in the cerebral cortex while electroencephalogram (EEG) showed nonspecific change. 14-3-3 protein and real-time qualing-induced conversion (RT-QuIC) of cerebrospinal fluid were negative. She died at age 85, 3.5 years after the onset. Pathological investigation revealed spongiform change, severe neuronal loss, and gliosis in the cerebral cortex. Mild to moderate neuronal loss and gliosis were observed in the basal ganglia. PrP immunostaining revealed plaque-like, dotlike, and synaptic structures in the cerebral cortex and small plaque-like structures in the molecular layer of the cerebellum. Analysis of PRNP showed no pathogenic mutations, and Western blot examination revealed the lack of a diglycosylated band consistent with VPSPr. The present case, which is the first report on a VPSPr case in Japan, supports previously published evidence that VPSPr cases can present variable and nonspecific clinical presentations. Because a small number of VPSPr cases can show typical magnetic resonance imaging (MRI) change in sCJD. We should investigate the possibility of VPSPr in a differential diagnosis with atypical dementia that presented DWIs of high intensity in the cortex, even though 14-3-3 proteins and RT-QuIC are both negative. In addition, VPSPr cases can take a longer clinical course compared to that of sCJD, and long-term follow-up is important.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://onlinelibrary.wiley.com/doi/abs/10.1111/neup.12911?campaign=woletoc" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://onlinelibrary.wiley.com/doi/abs/10.1111/neup.12911?campaign=woletoc</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, OCTOBER 27, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion disease features in Japan based on the national surveillance from 1999 to 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/10/prion-disease-features-in-japan-based.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/10/prion-disease-features-in-japan-based.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FRIDAY, OCTOBER 27, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Geographic Diversity in the Incidence of Human Prion Diseases — China, 2006–2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/10/geographic-diversity-in-incidence-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/10/geographic-diversity-in-incidence-of.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, February 2, 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CWD GSS TSE PRION SPINAL CORD, Confucius Ponders, What if?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REVIEW</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div></div></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">TUESDAY, DECEMBER 12, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/12/creutzfeldt-jakob-disease-tse-prion.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;">***> Professor John Collinge on tackling prion diseases 2023 UPDATE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is accumulating evidence also for iatrogenic AD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div style="outline: none !important;">wasted days and wasted nights...Freddy Fender<br style="outline: none !important;" /></div></div></div></div></div></div></div></div></div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">terry</div><div style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net</div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;" /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-12259982327225166032023-12-12T12:06:00.006-06:002023-12-12T14:42:05.115-06:00CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023<p><span style="background-color: white; font-family: arial; font-size: 16px;">CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023</span></p><div style="background-color: white; outline: none;"><div style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><span style="font-family: arial;">Tables of Cases Examined</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">National Prion Disease Pathology Surveillance Center Cases Examined¹</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Updated quarterly.</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Last updated on: September 2nd, 2023</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">1999 & earlier 383 232 202 27 3 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2000 145 102 90 12 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2001 209 118 110 8 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2002 241 144 124 18 2 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2003 259 160 137 21 2 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2004 315 180 163 16 0 1³</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2005 330 179 157 21 1 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2006 365 179 159 17 1 2⁴</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2007 374 210 191 19 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2008 384 221 205 16 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2009 397 231 210 20 1 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2010 402 247 219 28 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2011 392 238 214 24 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2012 413 244 221 23 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2013 416 258 223 34 1 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2014 355 208 185 21 1 1⁵</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2015 401 262 243 19 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2016 395 277 248 29 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2017 375 266 247 18 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2018 308 221 202 18 1 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2019 434 281 259 22 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2020 367 253 228 24 1 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2021 344 248 224 22 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2022 337 228 206 21 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2023 193 114 101 8 0 0</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">TOTAL 8534 5301 4720 507 14 4</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">=====</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Year CSF Only and RT-QuIC Positive</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2015 140</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2016 183</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2017 227</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2018 266</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2019 310</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2020 309</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2021 331</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2022 347</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">2023 240</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">TOTAL 2357</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Listed based on the year of death or, if not available, on the year of referral;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Cases with suspected prion disease for which brain tissue was submitted;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Disease acquired in the United Kingdom;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Disease possibly acquired in a Middle Eastern or Eastern European country;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Includes 28 cases in which the diagnosis is pending (2 from 2022 and 31 from 2023), and 20 inconclusive cases;</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Includes 8 (2 from 2021, 1 from 2022, and 5 from 2023) cases with type determination pending in which the diagnosis of vCJD has been excluded.</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">The sporadic cases include 4641 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 88 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 39 cases of sporadic Fatal Insomnia (sFI).</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Total does not include 312 Familial cases diagnosed by blood test only.</span></div><div style="outline: none;"><span style="font-family: arial;"><br /></span></div><div style="outline: none;"><span style="font-family: arial;">Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.</span></div><div style="outline: none;"><br /></div></div><div style="font-family: arial; font-size: 16px; outline: none;">For a downloadable PDF version of our quarterly table, please click the link below:</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;"><a href="https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2023-09/WebTable%20Legal.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2023-09/WebTable%20Legal.pdf</a></div></div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><a href="https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined</a></div></div></div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">TEXAS CJD STATISTICS WOEFULLY OUTDATED, 2012 to 2021...terry</div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">Creutzfeldt-Jakob Disease Case Counts and Percentages by Year and Age Category (2012-2021)</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">Year <55 Years of Age % <55 Years of Age ≥ 55 Years of Age % ≥ 55 Years of Age Total Cases</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2012 6 27% 16 73% 22</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2013 1 7% 13 93% 14</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2014 5 19% 22 81% 27</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2015 2 10% 18 90% 20</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2016 6 18% 27 82% 33</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2017 3 12% 22 88% 25</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2018 4 11% 32 89% 36</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2019 4 9% 42 91% 46</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2020 9 22% 32 78% 41</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">2021 1 2% 44 98% 45</span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;"><br /></span></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="font-family: arial;">Total 41 13% 268 87% 309 </span></div></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><a href="https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd/creutzfeldt-jakob-disease-cjd-data" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.dshs.texas.gov/creutzfeldt-jakob-disease-cjd/creutzfeldt-jakob-disease-cjd-data</a><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">TEXAS CJD MAP</div><div style="font-family: arial; font-size: 16px; outline: none;"><br /></div><div style="outline: none;"><a href="https://www.dshs.texas.gov/sites/default/files/IDCU/disease/creutzfeldt_jakob/CJDC21.png">https://www.dshs.texas.gov/sites/default/files/IDCU/disease/creutzfeldt_jakob/CJDC21.png</a><br /></div><div style="outline: none;"><br /></div><div style="outline: none;">TEXAS CJD HISTORY</div><div style="outline: none;"><br /></div><div style="outline: none;"><a href="https://cjdtexas.blogspot.com/">https://cjdtexas.blogspot.com/</a><br /></div><div style="outline: none;"><br /></div><div style="font-family: arial; font-size: 16px; outline: none;">Research Letter</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">December 11, 2023</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et alJohn A. Romley, PhD4; John C. Probasco, MD5</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">Author Affiliations</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">1Johns Hopkins University School of Medicine, Baltimore, Maryland</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">2Department of Economics, Stanford University, Stanford, California</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">3Department of Population, Family and Reproductive Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">4Leonard D. Schaeffer Center for Health Policy and Economics, University of Southern California, Los Angeles, California</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">5Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none;">JAMA Neurol. Published online December 11, 2023. doi:10.1001/jamaneurol.2023.4678 Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><a href="https://jamanetwork.com/journals/jamaneurology/article-abstract/2812784" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://jamanetwork.com/journals/jamaneurology/article-abstract/2812784</a><br style="outline: none;" /></div></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div style="outline: none;">> Due to aging populations worldwide, the epidemiology of CJD is evolving.3 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">> We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.</div><div style="outline: none;"><br style="outline: none;" /></div></div>let's examine the reality of zoonosis of the livestock TSE prion disease and iatrogenic CJD shall we...terry</div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div style="outline: none;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Angéline Denouel1</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">· Jean-Philippe Brandel1,2 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">· Danielle Seilhean1 · Jean-Louis Laplanche3,4 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">· Alexis Elbaz5 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">· Stéphane Haik1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We included all cases aged 45–89 years old who died with a probable/definite sCJD diagnosis based on the French national surveillance network. We used age-period-cohort (APC) Poisson regression models to study variation in mortality rates by sex, age, period, and time.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A total of 2475 sCJD cases aged 45–89 years were included. Mortality rates increased with age, reached a peak between 75 and 79 years, and decreased thereafter. Mortality rates were higher in women than men at younger ages and lower at older ages. The full APC model with a sex×age interaction provided the best fit to the data, thus in favour of sex, age, period, and cohort effects on mortality rates. In particular, mortality rates increased progressively with successive birth cohorts.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Keywords Age-period-cohort model · Prion · Temporal trend · Sporadic Creutzfeldt-Jakob disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Worldwide, the number of patients with sCJD appears to have progressively increased over time [13]. This increase can be partly explained by increasing life expectancy as well as by better case ascertainment due to improved diagnostic tests and awareness of the disease among clinicians. Indeed, a relationship between surveillance intensity and sCJD incidence has been shown [14]. It cannot be excluded, however, that an actual increase of sCJD cases has occurred, and this hypothesis can be examined using age-period-cohort (APC) models.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In this paper, we estimated mortality rates from sCJD in France over a 25-year period (1992–2016) based on data from the French national surveillance network.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/article/10.1007/s10654-023-01004-5" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/article/10.1007/s10654-023-01004-5</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: N/A</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgment: XXX</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 years ago;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2001 Singeltary on CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">February 14, 2001</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary, Sr</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author Affiliations</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FRIDAY, JANUARY 15, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">if not, why not?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD FOUNDATION Questionnaire</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">UK CJD Questionnaire</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">cjd questionnaire 1979</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">RE: re-Human Prion Diseases in the United States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> routine passive mortality CJD surveillance USA ?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> THIS has been proven not to be very useful in the U.K.;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">IDENTIFICATION OF CASES</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">full text;</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf</a></div></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD Questionnaire </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2007/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://cjdquestionnaire.blogspot.com/</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MONDAY, SEPTEMBER 11, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary sCJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">CANADA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Definite and probable CJD, 1998-2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">As of November 30, 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Year Sporadic Iatrogenic Genetic vCJD Total</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1998 22 1 1 0 24</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1999 27 2 3 0 32</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2000 32 0 3 0 35</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2001 27 0 3 0 30</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2002 30 0 5 1 36</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2003 27 1 1 0 29</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2004 42 0 4 0 44</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2005 42 0 2 0 44</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2006 39 0 5 0 44</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2007 35 0 4 0 39</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2008 48 0 1 0 49</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2009 48 0 5 0 53</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2010 35 0 3 0 38</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2011 46 0 4 1 51</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2012 62 0 1 0 63</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2013 50 0 1 0 51</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2014 51 0 5 0 56</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2015 44 0 8 0 52</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2016 57 1 6 0 64</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2017 82 0 5 0 87</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2018 75 1 5 0 81</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2019 76 0 3 0 79</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2020 65 0 4 0 69</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2021 60 0 3 0 63</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2022 88 0 2 0 90</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023 52 0 1 0 53</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Total 1263 6 86 2 1357</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cases with definite and probable diagnosis to date</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SNIP...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023, STILL NO MENTION OF VPSPr TSE Prion in Canada statistics...terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THIS problem must be addressed immediately imo.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">QUOTE;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://vpspr.blogspot.com/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</div><div style="outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;">WEDNESDAY, NOVEMBER 22, 2023 <br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Creutzfeldt-Jakob Disease in Mexico (1990-2023)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/11/creutzfeldt-jakob-disease-in-mexico.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/11/creutzfeldt-jakob-disease-in-mexico.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">CREUTZFELDT-JAKOB DISEASE IN THE UK (By Calendar Year)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">REFERRALS OF SUSPECT CJD DEATHS OF DEFINITE AND PROBABLE CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Year Referrals Year Sporadic1</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Iatrogenic Genetic2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">vCJD Total Deaths</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1990 [53]† 1990 28 5 0 - 33</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1991 75 1991 31 1 4 - 36</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1992 96 1992 45 2 6 - 53</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1993 79 1993 36 4 7 - 47</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1994 119 1994 53 1 9 - 63</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1995 87 1995 35 4 5 3 47</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1996 132 1996 40 4 6 10 60</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1997 163 1997 59 6 7 10 82</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1998 155 1998 64 3 5 18 90</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1999 170 1999 62 6 2 15 85</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2000 178 2000 48 1 3 28 80</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2001 179 2001 58 4 6 20 88</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2002 164 2002 73 0 5 17 95</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2003 163 2003 79 5 7 18 109</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2004 114 2004 50 2 6 9 67</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2005 124 2005 67 4 13 5 89</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2006 112 2006 68 1 9 5 83</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2007 119 2007 63 2 11 5 81</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2008 150 2008 84 5 6 2 97</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2009 153 2009 78 2 8 3 91</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2010 150 2010 85 3 6 3 97</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2011 158 2011 91 4 14 5 114</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2012 127 2012 92 5 12 0 109</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2013 152 2013 108 2 10 1 121</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2014 130 2014 100 3 13 0 116</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2015 140 2015 105 0 4 0 109</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2016 148 2016 118 1 6 1 126</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2017 159 2017 122 0 12 0 134</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2018 167 2018 137 2 12 0 151</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2019 147 2019 128 1 7 0 136</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2020 172 2020 135 1 8 0 144</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2021 179 2021 133 0 6 0 139</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2022 182 2022 150 0 8 0 158</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023 145 2023 113 1 3 0 117</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Total</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Referrals 4741 Total</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Deaths</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2738 85 246 178 3247</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">† Referral figure for 1990 is from 1 May onwards...*As at 4th December 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Summary of vCJD cases</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Deaths from definite vCJD (confirmed): 123</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Deaths from probable vCJD (without neuropathological confirmation): 55</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Number of deaths from definite or probable vCJD (as above): 178</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Number of definite/probable vCJD cases still alive: 0</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Total number of definite or probable vCJD (dead and alive): 178</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 There are, in addition, a total of 20 cases of vPSPr (death in 1997(1 case), 2004(1), 2006(1), 2008(3), 2010(1), 2012(4), 2013(1), 2016(3), 2017(1), 2018(1), 2019(1), 2020(2)) not included in the above figures.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2 includes all genetic prion disease, including GSS</div></div><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><a href="https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Case Report</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">An autopsy case of variably protease-sensitive prionopathy with Met/Met homogeneity at codon 129</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Akiko Uchino, Yuko Saito, Saori Oonuma, Shigeo Murayama, Saburo Yagishita, Tetsuyuki Kitamoto, Kazuko Hasegawa</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">First published: 30 May 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://doi.org/10.1111/neup.12911" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1111/neup.12911</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The typical clinical manifestations of sporadic Creutzfeldt-Jakob disease (sCJD) are rapid-progressive dementia and myoclonus. However, the diagnosis of atypical sCJD can be challenging due to its wide phenotypic variations. We report an autopsy case of variably protease-sensitive prionopathy (VPSPr) with Met/Met homogeneity at codon 129. An 81-year-old woman presented with memory loss without motor symptoms. Seventeen months after the onset, her spontaneous language production almost disappeared. Diffusion-weighted images (DWI) showed hyperintensity in the cerebral cortex while electroencephalogram (EEG) showed nonspecific change. 14-3-3 protein and real-time qualing-induced conversion (RT-QuIC) of cerebrospinal fluid were negative. She died at age 85, 3.5 years after the onset. Pathological investigation revealed spongiform change, severe neuronal loss, and gliosis in the cerebral cortex. Mild to moderate neuronal loss and gliosis were observed in the basal ganglia. PrP immunostaining revealed plaque-like, dotlike, and synaptic structures in the cerebral cortex and small plaque-like structures in the molecular layer of the cerebellum. Analysis of PRNP showed no pathogenic mutations, and Western blot examination revealed the lack of a diglycosylated band consistent with VPSPr. The present case, which is the first report on a VPSPr case in Japan, supports previously published evidence that VPSPr cases can present variable and nonspecific clinical presentations. Because a small number of VPSPr cases can show typical magnetic resonance imaging (MRI) change in sCJD. We should investigate the possibility of VPSPr in a differential diagnosis with atypical dementia that presented DWIs of high intensity in the cortex, even though 14-3-3 proteins and RT-QuIC are both negative. In addition, VPSPr cases can take a longer clinical course compared to that of sCJD, and long-term follow-up is important.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://onlinelibrary.wiley.com/doi/abs/10.1111/neup.12911?campaign=woletoc" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://onlinelibrary.wiley.com/doi/abs/10.1111/neup.12911?campaign=woletoc</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FRIDAY, OCTOBER 27, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion disease features in Japan based on the national surveillance from 1999 to 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/10/prion-disease-features-in-japan-based.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/10/prion-disease-features-in-japan-based.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FRIDAY, OCTOBER 27, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Geographic Diversity in the Incidence of Human Prion Diseases — China, 2006–2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/10/geographic-diversity-in-incidence-of.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/10/geographic-diversity-in-incidence-of.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saturday, February 2, 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CWD GSS TSE PRION SPINAL CORD, Confucius Ponders, What if?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">REVIEW</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none;">2023 TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE IN THE USA ZOONOSIS</div><div data-setdir="false" dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;">Detection of classical BSE prions in asymptomatic cows after inoculation with atypical/Nor98 scrapie</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Marina Betancor, Belén Marín, Alicia Otero, Carlos Hedman, Antonio Romero, Tomás Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan José Badiola, Olivier Andréoletti & Rosa Bolea * Veterinary Research volume 54, Article number: 89 (2023) Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The emergence of bovine spongiform encephalopathy (BSE) prions from atypical scrapie has been recently observed upon experimental transmission to rodent and swine models. This study aimed to assess whether the inoculation of atypical scrapie could induce BSE-like disease in cattle. Four calves were intracerebrally challenged with atypical scrapie. Animals were euthanized without clinical signs of prion disease and tested negative for PrPSc accumulation by immunohistochemistry and western blotting. However, an emergence of BSE-like prion seeding activity was detected during in vitro propagation of brain samples from the inoculated animals. These findings suggest that atypical scrapie may represent a potential source of BSE infection in cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Snip…</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Further in vivo experiments challenging different mouse lines have been started in order to confirm the infectivity of the PMCA products obtained in this study. However, in conclusion, our findings show that the propagation of atypical scrapie in cattle leads to the emergence of BSE-like seeding activity.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This is a concerning issue with far-reaching implications for public health and food safety. The possibility of interspecies transmission of prion diseases and the emergence of new prion strains highlight the critical need for continued surveillance and monitoring of these diseases in both animal and human populations. Early detection of prion diseases is crucial, and highly sensitive detection techniques such as PMCA can play an important role in this regard.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01225-2</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;">FRIDAY, MAY 19, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html</a><br style="outline: none;" /></div><div style="outline: none;"> </div></div><a href="https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">SATURDAY, MAY 20, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Docket No. APHIS–<span dir="ltr" style="outline: none;">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">see full submission;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div></div></div></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Experimental transmission of ovine atypical scrapie to cattle Experimental transmission of ovine atypical scrapie to cattle</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy McKenna, Lauren Read, Sara Canoyra, Alba Marín-Moreno & Juan María Torres </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Veterinary Research volume 54, Article number: 98 (2023) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Classical bovine spongiform encephalopathy (BSE) in cattle was caused by the recycling and feeding of meat and bone meal contaminated with a transmissible spongiform encephalopathy (TSE) agent but its origin remains unknown. This study aimed to determine whether atypical scrapie could cause disease in cattle and to compare it with other known TSEs in cattle. Two groups of calves (five and two) were intracerebrally inoculated with atypical scrapie brain homogenate from two sheep with atypical scrapie. Controls were five calves intracerebrally inoculated with saline solution and one non-inoculated animal. Cattle were clinically monitored until clinical end-stage or at least 96 months post-inoculation (mpi). After euthanasia, tissues were collected for TSE diagnosis and potential transgenic mouse bioassay. One animal was culled with BSE-like clinical signs at 48 mpi. The other cattle either developed intercurrent diseases leading to cull or remained clinical unremarkable at study endpoint, including control cattle. None of the animals tested positive for TSEs by Western immunoblot and immunohistochemistry. Bioassay of brain samples from the clinical suspect in Ov-Tg338 and Bov-Tg110 mice was also negative. By contrast, protein misfolding cyclic amplification detected prions in the examined brains from atypical scrapie-challenged cattle, which had a classical BSE-like phenotype. This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This is the first study in cattle inoculated with naturally occurring scrapie isolates that found the presence of prions resembling classical BSE in bovine brain although this was limited to detection by the ultrasensitive PMCA. The results from thermostability assay confirmed that the isolates were as thermoresistant as the BSE agent as proven in other studies [36, 48]. Previous PMCA studies with various British atypical scrapie isolates did not find any evidence of amplification [49, 50]. This may be explained by the use of ovine brain as substrate rather than brain from Bov-Tg110 mice, which may facilitate conversion to classical BSE prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Two hypotheses for prion strain propagation in cross-species transmission experiments have been proposed: conformational selection favours a particular strain conformation out of a mixture of conformations in a scrapie isolate whilst mutation results in the conformational shift of one conformation into another [51]. Following on from the study in mice [17], it has been subsequently suggested that classical BSE properties that arise in atypical scrapie isolates transmitted to cattle may be due to conformational mutation in a new host [52]. It does not confirm that the atypical scrapie agent is the origin of the classical BSE epidemic and further transmission studies would be required to see whether classical BSE can be generated.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Would PMCA applied to brains from cattle exposed to TSE agents other than classical BSE and atypical scrapie also produce a classical BSE-like molecular phenotype? The PMCA product obtained in the thermostability test using a thermosensitive classical scrapie control showed a profile unlike classical BSE. Atypical BSE has been linked to the origin of classical BSE because of its conversion into classical BSE following serial passages in wild-type mice (L-type BSE [11]) and bovine transgenic mice (H-type BSE [53]). Although we have not tested PMCA products of atypical BSE isolates as part of this study, there is no evidence that PMCA products from atypical BSE convert into classical BSE, at least for H-type BSE using bovine brain as substrate [54]. In fact, we were unable to propagate H-type BSE using the same methodology (S Canoyra, A Marín-Moreno, JM Torres, unpublished observation).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The study results support the decision to maintain the current ban on animal meal in feedstuffs for ruminants, particularly as atypical scrapie occurs world-wide, and eradication is unlikely for a sporadic disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In summary, experimental inoculation of cattle with the atypical scrapie agent may produce clinical disease indistinguishable from classical BSE, which cannot be diagnosed by conventional diagnostic tests, but prions can be amplified by ultrasensitive tests in both clinically affected and clinically unremarkable cattle, which reveal classical BSE-like characteristics. Further studies are required to assess whether a BSE-like disease can be confirmed by conventional tests, which may initially include a second passage in cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3" rel="nofollow" style="color: #338fe9; outline: none 0px;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3</a></div></div></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Presentation Type: Oral Presentation</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant Number: ALMA/APRI: 201400006, HC 414250</div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><div dir="ltr" style="outline: none;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">34 Scientific Commission/September 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">3. Atypical BSE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliations expand</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PMID: 21266763</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">see full text;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div></div></div><div dir="ltr" style="outline: none;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) <br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (<span dir="ltr" style="outline: none;">Tg338</span> and <span dir="ltr" style="outline: none;">Tg501</span>). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: No evidence of transmission was found on a first passage in <span dir="ltr" style="outline: none;">Tg338</span> nor <span dir="ltr" style="outline: none;">Tg501</span>ovinized mice, but on second passage, <span dir="ltr" style="outline: none;">4/10</span> <span dir="ltr" style="outline: none;">Tg338</span> mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and <span dir="ltr" style="outline: none;">1/12</span> <span dir="ltr" style="outline: none;">Tg501</span> mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed. </div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***thus questioning the origin of human sporadic cases. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">============== </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PRION 2015 CONFERENCE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"></div><div style="outline: none;">PRION <span dir="ltr" style="outline: none;">2016 TOKYO</span></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saturday, April 23, 2016</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SCRAPIE <span dir="ltr" style="outline: none;">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr" style="outline: none;">1933-6896</span> 1933-690X </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><span dir="ltr" style="outline: none;">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Tuesday, December 16, 2014</div><div style="outline: none;"> </div><div style="outline: none;">Evidence for zoonotic potential of ovine scrapie prions</div><div style="outline: none;"> </div><div style="outline: none;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014</div><div style="outline: none;"> </div><div style="outline: none;">Abstract</div><div style="outline: none;"> </div><div style="outline: none;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the humanprion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. ***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. ***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none;"> </div><div style="outline: none;">Subject terms: Biological sciences• Medical research At a glance</div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a></div><div style="outline: none;"> </div><div style="outline: none;">why do we not want to do TSE transmission studies on chimpanzees $</div><div style="outline: none;"> </div><div style="outline: none;">5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</div><div style="outline: none;"> </div><div style="outline: none;">snip...</div><div style="outline: none;"> </div><div style="outline: none;">R. BRADLEY</div><div style="outline: none;"> </div><div style="outline: none;"> <a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a></div><div style="outline: none;"> </div><div style="outline: none;">1: J Infect Dis 1980 Aug;142(2):205-8</div><div style="outline: none;"> </div><div style="outline: none;">Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.</div><div style="outline: none;"> </div><div style="outline: none;">Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.</div><div style="outline: none;"> </div><div style="outline: none;">Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.</div><div style="outline: none;"> </div><div style="outline: none;">snip...</div><div style="outline: none;"> </div><div style="outline: none;">The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.</div><div style="outline: none;"> </div><div style="outline: none;">PMID: 6997404</div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a></div><div style="outline: none;"> </div><div style="outline: none;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"</div><div style="outline: none;"> </div><div style="outline: none;">Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.</div><div style="outline: none;"> </div><div style="outline: none;">snip...</div><div style="outline: none;"> </div><div style="outline: none;">76/10.12/4.6</div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a></div><div style="outline: none;"> </div><div style="outline: none;">Nature. 1972 Mar 10;236(5341):73-4.</div><div style="outline: none;"> </div><div style="outline: none;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).</div><div style="outline: none;"> </div><div style="outline: none;">Gibbs CJ Jr, Gajdusek DC.</div><div style="outline: none;"> </div><div style="outline: none;">Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0</div><div style="outline: none;"> </div><div style="outline: none;">Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)</div><div style="outline: none;"> </div><div style="outline: none;">C. J. GIBBS jun. & D. C. GAJDUSEK</div><div style="outline: none;"> </div><div style="outline: none;">National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland</div><div style="outline: none;"> </div><div style="outline: none;">SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).</div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a></div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a></div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://scrapie-usa.blogspot.com/</a></div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://nor-98.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://nor-98.blogspot.com/</a></div><div style="outline: none;"> </div><div style="outline: none;">2001</div><div style="outline: none;"> </div><div style="outline: none;">Suspect symptoms</div><div style="outline: none;"> </div><div style="outline: none;">What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?</div><div style="outline: none;"> </div><div style="outline: none;">28 Mar 01</div><div style="outline: none;"> </div><div style="outline: none;">Like lambs to the slaughter</div><div style="outline: none;"> </div><div style="outline: none;">31 March 2001</div><div style="outline: none;"> </div><div style="outline: none;">by Debora MacKenzie Magazine issue 2284.</div><div style="outline: none;"> </div><div style="outline: none;">FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.</div><div style="outline: none;"> </div><div style="outline: none;">Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.</div><div style="outline: none;"> </div><div style="outline: none;">Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.</div><div style="outline: none;"> </div><div style="outline: none;">"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.</div><div style="outline: none;"> </div><div style="outline: none;">Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.</div><div style="outline: none;"> </div><div style="outline: none;">Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.</div><div style="outline: none;"> </div><div style="outline: none;">As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.</div><div style="outline: none;"> </div><div style="outline: none;">"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.</div><div style="outline: none;"> </div><div style="outline: none;">But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.</div><div style="outline: none;"> </div><div style="outline: none;">People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.</div><div style="outline: none;"> </div><div style="outline: none;">But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."</div><div style="outline: none;"> </div><div style="outline: none;">There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.</div><div style="outline: none;"> </div><div style="outline: none;">Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.</div><div style="outline: none;"> </div><div style="outline: none;"><a href="http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">REPORT OF THE ADVISORY COMMITTEE ON SCRAPIE 1976</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">IN CONFIDENCE</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">Such considerations suggest first that those responsible for work with scrapie should be selected with as much care as are astronauts. </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">THURSDAY, NOVEMBER 9, 2023 <br style="outline: none;" /></div></div></div></div></div></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">European Food Safety Authority (EFSA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">APPROVED: 25 October 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><div style="outline: none;"><div style="outline: none;">Monday, November 13, 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div style="outline: none;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none;">tg650</span> with fecal homogenates. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Published</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 August 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Accepted: 7 August 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><p class="ydp858d20ecyiv0662081683ydp7fad6b93yiv2780175211p1" style="font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp858d20ecyiv0662081683ydp7fad6b93yiv2780175211s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</span></p></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...see full text;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div></div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH and USDA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">end... <br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Fortuitous generation of a zoonotic cervid prion strain </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (<span dir="ltr" style="outline: none;">Tg12</span>), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the <span dir="ltr" style="outline: none;">Tg12</span> mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (<span dir="ltr" style="outline: none;">Tg12</span>) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the <span dir="ltr" style="outline: none;">Tg12</span> mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively</div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">"Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time."<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A probable diagnostic marker for CWD infection in humans </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xu Qi, Liuting Qing, Manuel Camacho, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Multiple in vitro CWD-seeded human PrP conversion experiments and some animal model studies indicate that the species barrier for CWD to human transmission can be overcome, but whether CWD prion can infect humans in real life remains controversial. The very limited understanding on the likely features of CWD infection in humans and the lack of a reliable diagnostic marker for identification of acquired human CWD cases contribute to this uncertainty. We aim to stablish such a reliable diagnostic marker for CWD infections in humans should they occur. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: A couple of PrPSc-positive spleens were identified from humanized transgenic mice inoculated with either CWD or sCJDMM1. Prions in these spleens were compared by bioassays in cervidized or humanized transgenic mice. A couple of PrPSc-positive spleens from UK sCJDMM1 patients were also examined similarly as controls with no exposure to CWD. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: We have detected two prion-positive spleens in humanized transgenic mice inoculated with some CWD isolates. Such experimentally generated splenic “humanized” CWD prions (termed eHuCWDsp) appear indistinguishable from prions in the brain of sCJDMM1 patients on Western blot. We compared eHuCWDsp with prions in the spleen from humanized mice infected with sCJDMM1 (termed sCJDMM1sp) by bioassays in cervidized or humanized transgenic mice. Significantly, we found that eHuCWDsp can efficiently infect not only the humanized mice but also cervidized transgenic mice, and cervidized mice infected by eHuCWDsp produced PrPSc and brain pathology that are practically identical to those of CWD-infected cervidized mice. In contrast, sCJDMM1sp, similar to prions from sCJDMM1 patient brains, is poorly transmissible in the cervidized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Our data demonstrate that high transmissibility with CWD features of splenic prions in cervidized transgenic mice is unique to acquired human CWD prions, and it may serve as a reliable marker to identify the first acquired human CWD cases. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively.</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====end </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /><div style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Prion 2023 Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author list: Sarah Zurbuchena,b , S. Jo Moorea,b , Jifeng Biana , Eric D. Cassmanna , and Justin J. Greenleea . a. Virus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, US b. Oak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, United States </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (<span dir="ltr" style="outline: none;">Tg12</span>; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection, analysis, decision to publish, or preparation of the manuscript. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We thank Ami Frank and Kevin Hassall for their technical contributions to this project.</div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====end </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">''Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">31 TAC §§65.82, 65.85, 65.88</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results show positive prion detection in all products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;"><div style="outline: none;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none;">tg650</span> with fecal homogenates. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> <a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">WEDNESDAY, NOVEMBER 01, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">TEXAS CHRONIC WASTING DISEASE RISES SUBSTANTIALLY TO 575 CONFIRMED CWD CASES TO DATE<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">FRIDAY, DECEMBER 08, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">TEXAS CWD TSE PRION DIRE CONSEQUENCES ARE HERE! </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/12/texas-cwd-tse-prion-dire-consequences.html</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">THURSDAY, DECEMBER 7, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic Wasting Disease CWD TSE Prion Cervid Update By State December 2023 </div><div style="outline: none;">(Long Version) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion.html</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(Short Version) <br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/12/chronic-wasting-disease-cwd-tse-prion_7.html</a></div></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;">NOW, THINK CONSUMPTION, EXPOSURE, FRIENDLY FIRE, PASS IT FORWARD, IATROGENIC CJD!</div><div data-setdir="false" dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Friday, October 20, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">An investigation has been opened into the death of a scientist who was studying a transmissible and deadly disease CJD in Spain DEGENERATIVE DISEASES</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">An investigation has been opened into the death of a scientist who was studying a transmissible and deadly disease in Spain </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Three institutions are trying to ascertain the origin of the infectious Creutzfeldt-Jakob disease samples discovered in the biochemist’s laboratory. The 45-year-old investigator died in 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The University of Barcelona’s School of Medicine, in L’Hospitalet de Llobregat, where laboratory 4141 is located.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MASSILIANO MINOCRI</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Manuel Ansede</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MANUEL ANSEDE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Madrid - OCT 19, 2023 - 16:15 EDT</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A prestigious Spanish researcher of Creutzfeldt-Jakob disease died last year after experiencing symptoms consistent with this deadly ailment, as EL PAÍS has learned from multiple sources at the three institutions involved. Three months ago, the University of Barcelona opened an internal investigation to ascertain the origin of thousands of unauthorized samples, some of them infectious, discovered in a freezer in its laboratory 4141, where the deceased biochemist worked. He was a member of the Bellvitge Biomedical Research Institute (IDIBELL) and the CIBER public consortium. These two institutions have joined the internal investigation, after noting concern among colleagues at the facility, who did not know the level of risk to which they were exposed without their knowledge. This neurodegenerative disease incubates silently for years, but when symptoms appear — rapid dementia and muscle stiffness — it is fatal. Life expectancy after diagnosis is barely six months. Its best-known animal equivalent is mad cow disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The biochemist joined the 4141 lab at the University of Barcelona in January 2018 as a principal investigator with a group of his own; his wife joined shortly after. Together, they identified characteristic substances in human cerebrospinal fluid, useful for the diagnosis of rapid dementia. In November 2020, the now deceased scientist began to feel unwell and asked to leave. After his colleagues found out that his symptoms were consistent with Creutzfeldt-Jakob disease, he demanded absolute privacy and decided to hide his diagnosis, according to the sources consulted for this article. He died at the age of 45.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">On December 18, 2020, the head of the 4141 laboratory, Isidre Ferrer, a professor of Pathology at the University of Barcelona and a member of IDIBELL, informed the directors of both institutions that suspicious samples of cerebrospinal fluid from people with Creutzfeldt-Jakob disease and other neurodegenerative types of dementia had been discovered by chance in a freezer at 80 degrees below zero, according to internal documentation to which EL PAÍS had access. The thousands of unauthorized samples from patients and animals were in a drawer reserved for the sick researcher’s group and lacked records indicating their presence. The University of Barcelona then ordered the immediate closure and decontamination of laboratory 4141, located in the School of Medicine at L’Hospitalet de Llobregat.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Doctor Gabriel Capellá, the director of IDIBELL, explains that they have identified “a maximum of eight people” who worked in the laboratory at that time, in addition to the deceased scientist and Isidre Ferrer. Some of these coworkers have required months of psychological care. The university’s safety office and IDIBELL’s prevention service determined that there was “an unacceptable risk,” although Capellá emphasizes that “there is no record of any occupational accident” in which a researcher could have been infected with contaminated material. Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies are caused by abnormal proteins called prions, which accumulate in the brain and cause a microscopic sponge-like appearance. There are only one or two cases per million inhabitants, the vast majority of which are of unknown cause, but cases of the disease have also been reported after contact with surgical instruments contaminated by these prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The three institutions involved took more than two years to send the suspect samples for analysis to a specialized center, the CIC bioGUNE, in Derio, Spain. A spokeswoman for the University of Barcelona says that they sent them in December 2022 and the three organizations received the results in March 2023. Four months later, in July, the legal departments at the three institutions finally informed the 4141 laboratory workers that the Creutzfeldt-Jakob disease samples were potentially infectious, as feared. “You can debate whether we have been quick [in our response] or not, but we have been transparent. We are [part of] three institutions that had to agree, and we have acted as guarantors,” says Capellá. A similar situation also occurred in France; following the death of a researcher from Creutzfeldt-Jakob in 2019 and the discovery of another suspected case, all public laboratories investigating prion diseases decided to temporarily close in July 2021 to review their protocols.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Laboratory 4141 was not equipped to handle high biohazard samples. It did not even have a biosafety hood. At the end of 2018, the CIBER public consortium signed an agreement so that the group could work with these dangerous samples at the high-security laboratory of the Animal Health Research Center (CReSA) in Bellaterra, Spain, near Barcelona. According to the sources we consulted, there was no reason to have the contaminated material in laboratory 4141, beyond saving time during experiments, since the CReSA bunker is 30 kilometers (about 19 miles) away and required waiting one’s turn to use. Isidre Ferrer, the head of the facility at the time, who has since retired, prefers not to comment on the case until the internal investigation is completed, but he emphasizes that he was unaware of the existence of these dangerous samples.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The IDIBELL director recalls that the deceased scientist was “a promising and brilliant researcher.” From 2013 to 2017, he worked at the University Medical Center of Göttingen (Germany) under neurologist Inga Zerr, a leading international expert in Creutzfeldt-Jakob disease. Physician Margarita Blázquez, who manages the CIBER public consortium, notes that the disease’s incubation period can last several years, so, if the deceased researcher really had it, he also could have become infected with it in Germany or at another of his previous laboratories. This newspaper has tried to contact the scientist’s widow via email but has not received a response. She asked to be discharged shortly after her husband did. The three institutions are now investigating whether the couple handled the dangerous samples without authorization in lab 4141. A third person affiliated with CIBER, a member of the now-deceased biochemist’s research group, worked with potentially infectious Creutzfeldt-Jakob samples without being informed that they were infectious.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The security office of the University of Barcelona believes that the samples would only have been a problem in the case of accidental inoculation or ingestion while handling them. But internal documents confirm the alarm the situation has caused on campus. “The laboratory technicians and investigators express their enormous concern about the fact that, so far, it has not been possible to determine the origin of the doctor’s illness. They are left to worry about whether they may suffer the same fate in a few years’ time as a result of uncontrolled contamination that may have been created in the laboratory,” according to the minutes of a December 22, 2020, meeting between workers and Carles Solsona, the director of the Department of Pathology at the University of Barcelona. “This fear causes them to suffer a state of permanent anguish, causing insomnia and irritability.”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The IDIBELL director sent a message to the center’s entire staff on the 11th, five days after EL PAÍS informed him that it was investigating the case. Gabriel Capellá then told his workers of “a very serious incident that became known on campus for the first time at the end of 2020.″ With “deep dismay,” Capellá announced the researcher’s death “due to a possible prion condition,” with “a possible iatrogenic [a disease acquired by contact with contaminated materials during a medical procedure].” The director also reported finding “potentially dangerous samples” in a freezer. “Our priority is to ensure that this situation is handled rigorously and transparently to limit the damage to the reputation of our institutions,” he said.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Do you have more information about this case or other similar ones? You can write to us at mansede@elpais.es.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sign up for our weekly newsletter to get more English-language news coverage from EL PAÍS USA Edition</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://english.elpais.com/science-tech/2023-10-20/an-investigation-has-been-opened-into-the-death-of-a-scientist-who-was-studying-a-transmissible-and-deadly-disease-in-spain.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://english.elpais.com/science-tech/2023-10-20/an-investigation-has-been-opened-into-the-death-of-a-scientist-who-was-studying-a-transmissible-and-deadly-disease-in-spain.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Direct neural transmission of vCJD/BSE in macaque after finger incision CORRESPONDENCE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Direct neural transmission of vCJD/BSE in macaque after finger incision</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Jacqueline Mikol1 · Jérôme Delmotte1 · Dolorès Jouy1 · Elodie Vaysset1 · Charmaine Bastian1 · Jean‑Philippe Deslys1 ·</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Emmanuel Comoy1 Received: 10 July 2020 / Revised: 8 September 2020 / Accepted: 25 September 2020 / Published online: 6 October 2020 © The Author(s) 2020</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Non-human primates appeared as the closest model to study human iatrogenic prion diseases [14]: we report here the consequences of variant Creutzfeldt–Jakob disease/bovine spongiform encephalopathy (vCJD/BSE) inoculation in a cynomolgus macaque finger, with the demonstration of an original mode of propagation and the practical risk for professional exposure.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The distal right middle finger handpad of a 4-year-old macaque was incised on both lateral sides to induce local inflammation, and then injected with the equivalent of 10 mg of a BSE, orally challenged macaque brain [18]. After an 18 months period of finger clumsiness, the clinical disease (behaviour abnormalities, fear, hyperesthesia, gait disturbances, shaking) began 7.5 years after inoculation and euthanasia took place 2 months later for welfare reasons. Motor conduction velocity of the right median nerve was reduced to one-third of the left counterpart and sensory potential was not detected.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Histological and biochemical studies were performed as previously described. All the elements of the triad were present [7–9]: spongiform change was moderate in neocortex, striatum, brain stem, mild in spinal cord but severe in thalamus and cerebellum; neuronal loss was globally moderate, but severe in cerebellum and sacral spinal cord (vacuolated neurons); gliosis was severe in thalamus, cerebellum and brain stem and moderate elsewhere (Supplementary Fig. 1). ELISA and western blot (WB) showed the expected accumulation of PrPres with BSE glycophoretic pattern at all levels of brain and spinal cord (Supplementary Fig. 2).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In the brain, PrPd deposits were laminar into the cortical deep layers, massive into thalamus, basal ganglia, cerebellum, and brain stem. In spinal cord, PrPd was symmetrically distributed, intense in the Substantia gelatinosa and nucleus dorsal of Clarke while decreased at sacral level. Deposits were diverse into the whole CNS: synaptic, perineuronal, reticular aggregates, mini-plaques, plaques, and incomplete florid plaques. The retinal plexiform layers were labelled (Supplementary Fig. 1i). There were no amyloid or tau deposits.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Unusual PrPd deposits were observed along dendrites, short and long axons, neuritic threads tracing fne networks of straight lines or like strings of pearls (Supplementary Fig. 3). They were present into deep neocortex, basal ganglia, and motoneurons. Such long processes are not frequent but have been reported in human [13] and experimental studies [10, 22]. PrPd deposits were also noted as very mild into striato-pallidal projections, both limbs of internal capsule and fornix (Supplementary Fig. 3). The presence of PrPd in white matter has been reported (Supplementary text 4).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Peripherally, the expected PrPd was undetectable in lymphoid organs, including spleen, through biochemical or immunohistochemical analyses, while prion replication was detected in the peripheral nervous system (PNS): PrPd staining was visualized in many dorsal root ganglia (DRG) but only in nerves innervating the forelimb site of injection (median and ulnar nerves). At the cellular level, PrPd was limited to ganglia and satellite cells in DRG and Schwann cells (Scs) all along nerves whereas axons were never labelled (Fig. 1). Previously, using postmortem immunohistochemical studies (listed in Supplementary text 5), PrPd has been shown in peripheral nervous system in all forms of human neuropathies, albeit more frequently in vCJD, mostly in posterior root nerve fbres at adaxonal location and/or in ganglion and satellite cells. The restricted amount of PrPd was repeatedly underlined but, recently, prion RTQuiC was positive in all nerves examined [2]. PrPd has also been described, frst in scrapie [17] then in BSE, as limited “adaxonal deposits” or/and Sc deposits, with or without DRG cell involvement (review in [4] and Supplementary text 6). Previous studies of the mode of propagation of PrPd have reported variable observations and analyses depending on strains, host species and genotype (Supplementary text 6); the authors discussed the role of the sensory route of trafficking of prions, the modifications of axonal transport, the centrifugal versus centripetal spread of PrPd .</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">After peripheral infection, accumulation of infectious agent is reputed to occur in lymphoid tissues before direct neuroinvasion [18, 19], even with very little apparent peripheral lymphoreticular deposition [6, 20]. Here, there is no apparent replication/amplification of vCJD/BSE agent in the lymphoid tissues of the exposed macaque. In this model, the neural contamination occurred directly in the highly innervated finger while neuroinvasion appears to occur in Scs along the median nerve to the DRG, with the appearance of the classical labelling of ganglion cells which indicates the onset of the first level of neuronal infection. This model provides direct evidence of the hypothesis of a sequential infection of Scs from the periphery to the CNS, followed by a secondary diffusion into the spinal cord, as already considered by our group [15] and others [1, 3, 11, 12, 21]. It is to note that studies based on intra-sciatic nerve injections in hamsters [16] and transgenic mice [12] had established a rate of transport of infectivity of, respectively, 0.5–2 mm and 0.7 mm per day. This key role of Scs could explain both the low speed of propagation and the discrepancy between the paucity of PrPd into the distal part of the sensory nerves followed by the positivity of DRG, satellite cells and proximal roots.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In conclusion, we have observed that the exposure of a primate to vCJD/BSE through a distal finger lesion induces, after more than 7.5 years of silent incubation, a massive deposit of PrPd , strictly restricted to the nervous system and the eye.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our data suggest a new type of pure unique peripheral nervous contamination in which the Scs would have a major role in the mode of centripetal progression of PrPd in the peripheral nervous system. Moreover, considering the fact that, recently, “a variant CJD diagnosed 7.5 years after occupational exposure” (cryomicrotomy) in a technician was observed [5], this experimental case report supports the risk linked to professional exposure and reinforces the necessity of adequate measures of prevention. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-020-02231-w.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Second death in France in a laboratory working on prions</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Creutzfeldt-Jakob disease has killed a person who handled this infectious agent at Inrae in Toulouse. After a first death in 2019, a moratorium on work on this pathogen has been extended.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">By Hervé Morin</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working with these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of mice infected with prions, in another unit of INRAE, in Jouy-en-Josas.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Computer representation of part of a prion protein on a light micrograph of pyramidal nerve cells (neurons, in black) in the cerebellum of the brain. ALFRED PASIEKA / SCIENCE PHOTO LIBRARY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.lemonde.fr/sciences/article/2021/11/30/second-deces-en-france-dans-un-laboratoire-travaillant-sur-les-prions_6104124_1650684.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Temporary suspension of work on prions in French public research laboratories</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PRESS RELEASE - The general directorates of ANSES, CEA, CNRS, INRAE and Inserm, have decided jointly and in agreement with the Ministry of Higher Education, Research and Innovation to suspend as a precaution all their research and experimentation work relating to prion diseases, for a period of three months.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This precautionary measure is motivated by the knowledge of a possible new case of a person suffering from Creutzfeldt-Jakob disease and who worked in a laboratory for research on prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Posted on July 27, 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The suspension period put in place as of this day will make it possible to study the possibility of a link between the observed case and the person's former professional activity and to adapt, if necessary, the preventive measures in force in the research laboratories. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The person with Creutzfeldt-Jakob disease (CJD)1, whose form is not yet known, is a retired INRAE agent. This could be the second case of infectious CJD affecting a scientist who worked on prions, after that of an assistant engineer who died of the disease in 2019, and who was injured in 2010 during of an experiment.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Following this death, a general inspection mission was launched in July 2019 by the ministries of research and agriculture with French laboratories handling prions. Submitted in October 2020, the report concluded on the regulatory compliance of the laboratories visited as well as the presence of a risk control culture within the research teams.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Research around prion proteins, with high public health issues, allows major advances in the understanding of the functioning of these infectious pathogens, and contributes to results that are transferable to other related degenerative diseases such as Alzheimer's and Alzheimer's diseases. Parkinson's.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">At the level of each establishment, regular and transparent information will be provided to all the working communities concerned by this measure.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 The disease Creutzfeldt-Jakob disease (CJD) is one of prion diseases - still called encephalopathies subacute spongiform transmitted(TSE) - of diseases rare, characterized by a degeneration rapid and fatal the system nervous central. They are caused by the accumulation in the brain of a normally expressed protein but poorly conformed - the prion protein - which leads to the formation of deleterious aggregates for neurons. For now , no treatment will allow to change the course of these diseases. It can be of origin sporadic , form the most frequent , original genetic or finally to form infectious following a contamination. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.inserm.fr/information-en-sante/dossiers-information/maladies-prions-maladie-creutzfeldt-jakob</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.inrae.fr/actualites/suspension-provisoire-travaux-prions-laboratoires-recherche-publics-francais</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">By Barbara CasassusJul. 28, 2021 , 4:35 AM</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PARIS—Five public research institutions in France have imposed a 3-month moratorium on the study of prions—a class of misfolding, infectious proteins that cause fatal brain diseases—after a retired lab worker who handled prions in the past was diagnosed with Creutzfeldt-Jakob disease (CJD), the most common prion disease in humans. An investigation is underway to find out whether the patient, who worked at a lab run by the National Research Institute for Agriculture, Food and Environment (INRAE), contracted the disease on the job.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">If so, it would be the second such case in France in the past few years. In June 2019, an INRAE lab worker named Émilie Jaumain died at age 33, 10 years after pricking her thumb during an experiment with prion-infected mice. Her family is now suing INRAE for manslaughter and endangering life; her illness had already led to tightened safety measures at French prion labs.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The aim of the moratorium, which affects nine labs, is to “study the possibility of a link with the [new patient’s] former professional activity and if necessary to adapt the preventative measures in force in research laboratories,” according to a joint press release issued by the five institutions yesterday.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">“This is the right way to go in the circumstances,” says Ronald Melki, a structural biologist at a prion lab jointly operated by the French national research agency CNRS and the French Alternative Energies and Atomic Energy Commission (CEA). “It is always wise to ask questions about the whole working process when something goes wrong.” "The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community, which is a small 'familial' community of less than 1000 people worldwide," Emmanuel Comoy, deputy director of CEA's Unit of Prion Disorders and Related Infectious Agents, writes in an email to Science. Although prion research already has strict safety protocols, "it necessarily reinforces the awareness of the risk linked to these infectious agents," he says.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In Jaumain’s case, there is little doubt she was infected on the job, according to a paper published in The New England Journal of Medicine (NEJM) in 2020. She had variant CJD (vCJD), a form typically caused by eating beef contaminated with bovine spongiform encephalopathy (BSE), or mad cow disease. But Europe’s BSE outbreak ended after 2000 and vCJD virtually disappeared; the chance that someone of Jaumain’s age in France would contract food-borne vCJD is “negligible or non-existent,” according to the paper.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A scientist with inside knowledge says the new patient, a woman who worked at INRAE’s Host-Pathogen Interactions and Immunity group in Toulouse, is still alive. French authorities were apparently alerted to her diagnosis late last week. The press release suggests it’s not yet clear whether the new case is vCJD or “classic” CJD, which is not known to be caused by prions from animals. Classic CJD strikes an estimated one person per million. Some 80% of cases are sporadic, meaning they have no known cause, but others are genetic or contracted from infected human tissues during transplantations. The two types of CJD can only be distinguished through a postmortem examination of brain tissue.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Lab infections are known to occur with many pathogens, but exposure to CJD-causing prions is unusually risky because there are no vaccines or treatments and the condition is universally fatal. And whereas most infections reveal themselves within days or weeks, CJD’s average incubation period is about 10 years.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">For Jaumain, who worked at INRAE’s Molecular Virology and Immunology Unit in Jouy-en-Josas, outside Paris, that long period of uncertainty began on 31 May 2010, when she stabbed her left thumb with a curved forceps while cleaning a cryostat—a machine that can cut tissues at very low temperatures—that she used to slice brain sections from transgenic mice infected with a sheep-adapted form of BSE. She pierced two layers of latex gloves and drew blood. “Émilie started worrying about the accident as soon as it had happened, and mentioned it to every doctor she saw,” says her widower, Armel Houel.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In November 2017, Jaumain developed a burning pain in her right shoulder and neck that worsened and spread to the right half of her body over the following 6 months, according to the NEJM paper. In January 2019, she became depressed and anxious, suffering memory impairment and hallucinations. “It was a descent into hell,” Houel says. She was diagnosed with “probable vCJD” in mid-March of that year and died 3 months later. A postmortem confirmed the diagnosis.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">“The occurrence of these harsh diseases in two of our scientific colleagues clearly affects the whole prion community.” Emmanuel Comoy, French Alternative Energies and Atomic Energy Commission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">INRAE only recently admitted the likely link between Jaumain’s illness and the accident. “We recognize, without ambiguity, the hypothesis of a correlation between Emilie Jaumain-Houel’s accident … and her infection with vCJD,” INRAE chair and CEO Philippe Mauguin wrote in a 24 June letter to an association created by friends and colleagues to publicize Jaumain’s case and lobby for improvements in lab safety. (Science has obtained a copy of the letter, which has not been made public.)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Jaumain’s family has filed both criminal charges and an administrative suit against INRAE, alleging a range of problems at Jaumain’s lab. She had not been trained in handling dangerous prions or responding to accidents and did not wear both metal mesh and surgical gloves, as she was supposed to, says Julien Bensimhon, the family’s lawyer. The thumb should have been soaked in a bleach solution immediately, which did not happen, Bensimhon adds.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Independent reports by a company specializing in occupational safety and by government inspectors have found no safety violations at the lab; one of them said there was a “strong culture” of risk management. (Bensimhon calls the reports “biased.”)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The government inspectors’ report concluded that Jaumain’s accident was not unique, however. There had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, five of whom stabbed or cut themselves with contaminated syringes or blades. Another technician at the same lab had a fingerprick accident with prions in 2005, but has not developed vCJD symptoms so far, Bensimhon says. “It is shocking that no precautionary measures were taken then to ensure such an accident never happened again,” he says.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In Italy, too, the last person to die of vCJD, in 2016, was a lab worker with exposure to prion-infected brain tissue, according to last year’s NEJM paper, although an investigation did not find evidence of a lab accident. That patient and the lab they worked at have not been identified.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">After Jaumain’s diagnosis, “We contacted all the research prion labs in France to suggest they check their safety procedures and remind staff about the importance of respecting them,” says Stéphane Haïk, a neuroscientist at the Paris Brain Institute at Pitié-Salpêtrière Hospital who helped diagnose Jaumain and is the corresponding author on the paper. Many labs tightened procedures, according to the government inspectors' report, for instance by introducing plastic scissors and scalpels, which are disposable and less sharp, and bite and cut-resistant gloves. A team of experts from the five research agencies is due to submit proposals for a guide to good practice in prion research to the French government at the end of this year.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The scientific community has long recognized that handling prions is dangerous and an occupational risk for neuropathologists, says neuropathologist Adriano Aguzzi of the University of Zurich. Aguzzi declined to comment on the French CJD cases, but told Science his lab never handles human or bovine prions for research purposes, only for diagnostics. “We conduct research only on mouse-adapted sheep prions, which have never been shown to be infectious to humans,” Aguzzi says. In a 2011 paper, his team reported that prions can spread through aerosols, at least in mice, which “may warrant re-thinking on prion biosafety guidelines in research and diagnostic laboratories,” they wrote. Aguzzi says he was “totally shocked” by the finding and introduced safety measures to prevent aerosol spread at his own lab, but the paper drew little attention elsewhere.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The moratorium will "obviously" cause delays in research, but given the very long incubation periods in prion diseases, the impact of a 3-month hiatus will be limited, Comoy says. His research team at CEA also works on other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, and will shift some of its efforts to those.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Although Jaumain’s diagnosis upset many in the field, it hasn't led to an exodus among researchers in France, Haïk says: “I know of only one person who resigned because they were so worried.”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">With reporting by Martin Enserink.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Posted in: EuropeHealthScientific Community</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">doi:10.1126/science.abl6587</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.sciencemag.org/news/2021/07/france-issues-moratorium-prion-research-after-fatal-brain-disease-strikes-two-lab</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">TO THE EDITOR:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We report a case of variant Creutzfeldt–Jakob disease (CJD) that was plausibly related to accidental occupational exposure in a technician who had handled murine samples contaminated with the agent that causes bovine spongiform encephalopathy (BSE) 7.5 years earlier.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In May 2010, when the patient was 24 years of age, she worked in a prion research laboratory, where she handled frozen sections of brain of transgenic mice that overexpressed the human prion protein with methionine at codon 129. The mice had been infected with a sheep-adapted form of BSE. During this process, she stabbed her thumb through a double pair of latex gloves with the sharp ends of a curved forceps used to handle the samples. Bleeding was noted at the puncture site.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In November 2017, she began having burning pain in the right shoulder and neck. The pain worsened and spread to the right half of her body during the following 6 months. In November 2018, an examination of a sample of cerebrospinal fluid (CSF) obtained from the patient was normal. Magnetic resonance imaging (MRI) of the brain showed a slight increase in the fluid-attenuated inversion recovery (FLAIR) signal in the caudates and thalami (Fig. S1A and S1B in the Supplementary Appendix, available with the full text of this letter at NEJM.org). In January 2019, she became depressed and anxious and had memory impairment and visual hallucinations. There was hypertonia on the right side of her body. At that time, an analysis of CSF for 14-3-3 protein was negative. In March 2019, MRI showed an increased FLAIR signal in pulvinar and dorsomedial nuclei of thalami (Fig. S1C through S1E).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Figure 1.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Detection of Abnormal Prion Protein in Biologic Fluid Samples and Postmortem Findings.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The patient was found to be homozygous for methionine at codon 129 of the prion protein gene without mutation. An analysis of a sample of CSF on real-time quaking-induced conversion analysis was negative for a diagnosis of sporadic CJD. However, an analysis of plasma and CSF by means of protein misfolding cyclic amplification was positive for the diagnosis of variant CJD (Figure 1A and 1B). The patient died 19 months after the onset of symptoms. Neuropathological examination confirmed the diagnosis of variant CJD (Figure 1C and 1D). Western blot analysis showed the presence of type 2B protease-resistant prion protein in all sampled brain areas. The clinical characteristics of the patient and the postmortem neuropathological features were similar to those observed in 27 patients with variant CJD who had previously been reported in France.1 (Additional details are provided in the Supplementary Appendix.)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">There are two potential explanations for this patient’s condition. Oral transmission from contaminated cattle products cannot be ruled out because the patient was born at the beginning of the French BSE outbreak in cattle. However, the last two patients who had confirmed variant CJD with methionine homozygosity at codon 129 in France and the United Kingdom died in 2014 and 2013, respectively, which makes oral transmission unlikely. In France, the risk of variant CJD in 2019 was negligible or nonexistent in the post-1969 birth cohort.2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Percutaneous exposure to prion-contaminated material is plausible in this patient, since the prion strain that she had handled was consistent with the development of variant CJD.3 The 7.5-year delay between the laboratory accident and her clinical symptoms is congruent with the incubation period in the transfusion-transmitted form of the disease. The ability of this strain to propagate through the peripheral route has been documented, and experimental studies with scrapie strains have shown that scarification and subcutaneous inoculation are effective routes.4,5 The last known Italian patient with variant CJD, who died in 2016, had had occupational contact with BSE-infected brain tissues, although subsequent investigation did not disclose a laboratory accident (Pocchiari M, Italian Registry of CJD: personal communication). Thus, the last two cases of variant CJD outside the United Kingdom have been associated with potential occupational exposure. Such cases highlight the need for improvements in the prevention of transmission of variant CJD and other prions that can affect humans in the laboratory and neurosurgery settings, as outlined in the Supplementary Appendix.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Jean-Philippe Brandel, M.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">M. Bustuchina Vlaicu, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Audrey Culeux, B.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Maxime Belondrade, M.Sc. Daisy Bougard, Ph.D. Etablissement Français du Sang, Montpellier, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Katarina Grznarova, Ph.D. Angeline Denouel, M.Sc. INSERM Unité 1127, Paris, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Isabelle Plu, M.D. Elodie Bouaziz-Amar, Pharm.D., Ph.D. Danielle Seilhean, M.D., Ph.D. Assistance Publique–Hôpitaux de Paris, Paris, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Michèle Levasseur, M.D. Groupe Hospitalier Nord-Essonne, Orsay, France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Stéphane Haïk, M.D., Ph.D. INSERM Unité 1127, Paris, France stephane.haik@upmc.fr</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Supported by a grant (ANR-10-IAIHU-06) from Programme d’Investissements d’Avenir and Santé Publique France.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Disclosure forms provided by the authors are available with the full text of this letter at NEJM.org.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">5 References</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">July 2, 2020</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">N Engl J Med 2020; 383:83-85</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DOI: 10.1056/NEJMc2000687</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Metrics</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">34 year old Doctor Orthopedic Surgeon dies from CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Dr. Adam Thomas Dialectos</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1987 - 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BORN</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">April 29, 1987</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DIED</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">June 21, 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FUNERAL HOME</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Bean Funeral Homes & Crematory Inc</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1605 Rockland St</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Reading, PA 19604</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">UPCOMING SERVICE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Visitation</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Jun, 24 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">9:00a.m. - 11:00a.m.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saints Constantine & Helen Greek Orthodox Church</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Send Flowers</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Share</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">On Monday June 21, 2021, Dr. Adam Thomas Dialectos, loving husband, father, son, brother, uncle, Nouno, friend at the age of 34. Adam was born on April 29, 1987 in Reading, PA to Athan and Gretchen Dialectos. Adam was a 2005 graduate of Governor Mifflin High School, before receiving his degree in Health Sciences from James Madison University in 2009. Adam attended Philadelphia College of Osteopathic Medicine for medical school and his subsequent residency in orthopedic surgery. Adam was completing his Spine Surgery Fellowship at New England Baptist Hospital in Boston, Massachusetts. On February 7, 2019 Adam married the love of his life and girlfriend of 12 years, Lindsey (Schuler) Dialectos. They brought a beautiful baby boy into this world on January 6, 2021, Athananosis Adam Dialectos. Adam’s passion in life was unceasingly seeking to help others, emphasized by his desire to be a surgeon— a decision he made in his early elementary years. Adam continued this love of medicine throughout his life, which led to his achieving of the Henrietta and Jack Avart Memorial Award in 2019, awarded to the Orthopedic surgery resident who exhibited unparalleled excellence in their field during the residency program. This passion to learn, teach and support was truly understood through the patients whose lives Adam touched. When it came to his patients and coworkers, there was never a job too small for Adam. Those who knew Adam saw his personality shine through in so many other aspects of his life. Adam loved traveling. Some of his most memorable trips were with his wife, and countless snowboard trips with his brother, family, and friends. Adam loved everyone he was around; he loved and was loved by so many. Adam was truly one in a million. Adam is survived by his loving wife, Lindsey, and their son, Athan Adam; His father and mother, Athan and Gretchen; His brother Jordan and sister-in-law Megan, and their daughter Livia, Adam’s Goddaughter. His sister, Rachel, and her significant other, Bo Wagner. Furthermore, Adam is survived by his Yiayia, Joanne Dialectos, wife of the late George Dialectos; his Pop Pop, Donald Harford, husband of the late Nancy Servent; his Aunt Angel and Uncle Scott Helm; his Aunt Kelly and Uncle Darrell Markley. Adam was preceded in death by his Aunt Maria and Uncle Bob Care. Funeral Service will be held at Saints Constantine & Helen Greek Orthodox Church, 1001 East Wyomissing Blvd. Reading on Thursday June 24th. Father Theodore Petrides and Father Thomas L. Pappalas will officiate. Interment will follow at Charles Evans Cemetery. The family will receive relatives and friends at Saints Constantine & Helen Greek Orthodox Church from 9:00am to 11:00am with services beginning at 11:00. In lieu of flowers, contributions may be made to the CJD Foundation at 3634 West Market Street Suite 110 Akron, Ohio 44333 or cjdfoundation.org in remembrance of Dr. Adam Dialectos. Donations may also be made to Saints Constantine & Helen Greek Orthodox Church. Bean Funeral Home, 1605 Rockland Street, Hampden Heights, is in charge of arrangements and online condolences may be made at www.beanfuneralhomes.com.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To plant trees in memory, please visit our Sympathy Store.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Published by Reading Eagle from Jun. 22 to Jun. 24, 2021.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.legacy.com/us/obituaries/readingeagle/name/adam-dialectos-obituary?pid=199144663" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.legacy.com/us/obituaries/readingeagle/name/adam-dialectos-obituary?pid=199144663</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our sincere condolences to the Family and Friends of Dr. Adam Thomas Dialectos. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I can't help but ponder, as a Orthopedic Surgeon, Spine Surgery Fellowship, and what the good Doctors work curtailed, i can't help but think this is a potential case of iatrogenic CJD. surgery on humans, i would imagine cadavers as well.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, provern, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd. ...terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">least we forget...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery *** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saturday, December 18, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Direct neural transmission of vCJD/BSE in macaque after finger incision </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://itseprion.blogspot.com/2021/12/direct-neural-transmission-of-vcjdbse.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://itseprion.blogspot.com/2021/12/direct-neural-transmission-of-vcjdbse.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Tuesday, November 30, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Second death in France in a laboratory working on prions</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://itseprion.blogspot.com/2021/11/second-death-in-france-in-laboratory.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Second lab worker with deadly prion disease prompts research pause in France</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A lab worker died of prion disease in 2019, nine years after a lab accident.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BETH MOLE - 7/29/2021, 5:16 PM</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://arstechnica.com/science/2021/07/second-lab-worker-with-deadly-prion-disease-prompts-research-pause-in-france/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://arstechnica.com/science/2021/07/second-lab-worker-with-deadly-prion-disease-prompts-research-pause-in-france/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A 2020 paper published in the New England Journal of Medicine left little doubt that Jaumain had been infected on the job. She had variant CJD, but since Europe’s ‘mad cow’ outbreak ended after 2000 and the disease virtually disappeared, the paper said it was virtually impossible for someone her age in France to contract food-borne vCJD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Science also said two independent reports – one by government inspectors – had found no safety violations at the lab where Jaumain worked. The press release also noted that the inspectors concluded there was “the presence of a risk control culture within the research teams”. The Jaumain family’s lawyer called the neutrality of the reports into question, however.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">At the same time, the government inspectors’ report also revealed that there had been at least 17 accidents among the 100 or so scientists and technicians in France working with prions in the previous decade, raising concerns about how effective this risk control culture is. Five of these occurred when workers “stabbed or cut themselves with contaminated syringes or blades”.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://science.thewire.in/the-sciences/second-case-of-fatal-disease-prompts-french-moratorium-on-prion-research/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://science.thewire.in/the-sciences/second-case-of-fatal-disease-prompts-french-moratorium-on-prion-research/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Wednesday, July 28, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Wednesday, July 28, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SATURDAY, AUGUST 01, 2020</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/08/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/08/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SUNDAY, JULY 19, 2020 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Joseph J. Zubak Orthopaedic surgeon passed away Monday, July 6, 2020, Creutzfeldt-Jakob Disease (CJD)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob disease was identified in a technician who had cut her thumb while handling brain sections of mice infected with adapted BSE 7.5 years earlier. The long incubation period was similar to that of the transfusion-transmitted form of the disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nejm.org/doi/full/10.1056/NEJMc2000687" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJMc2000687</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nejm.org/doi/suppl/10.1056/NEJMc2000687/suppl_file/nejmc2000687_appendix.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nejm.org/doi/suppl/10.1056/NEJMc2000687/suppl_file/nejmc2000687_appendix.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THURSDAY, JULY 02, 2020 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://vcjd.blogspot.com/2020/07/variant-creutzfeldtjakob-disease.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://vcjd.blogspot.com/2020/07/variant-creutzfeldtjakob-disease.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Thursday, July 29, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">TSE PRION OCCUPATIONAL EXPOSURE VIA ANIMAL OR HUMAN, iatrogenic transmission, nvCJD or sCJD, what if? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html</a></div></div></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;">Deep Throat to Singeltary BSE Mad Cow 2001 to 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I remember what “deep throat” told me about Scrapie back around 2001, I never forgot, and it seems it’s come to pass;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Confidential!!!!</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">---end personal email---end...tss </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(I never knew who this person was, but got me into the U.S. BSE Emergency 50 State conference call back 2001, and we corresponded for years about BSE TSE Prion, have not heard from in over a decade, but they were on the inside looking out. You can believe this or not, but this was real, i don’t make this stuff up…plus my endeavors to get those 1 million cattle tested for BSE failed. There was an ENHANCED BSE SURVEILLANCE put forth after 2003, we pushed for it, but it was abruptly shut down after the atypical BSE cases were popping up…a bit of history for anyone interested…terry)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at..... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!! And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!" </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">U.S. 50 State Emergency BSE Conference Call 2001</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary Comment Docket No: 2002N-0273 (formerly Docket No. 02N-0273)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MY comments/questions are as follows ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** Suppressed peer review of Harvard study October 31, 2002 *** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today ? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...see full text; </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20050308184249/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary Full Comments Submissions;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090419033608/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090424070455/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FSIS, HARVARD, REPLY TO SINGELTARY </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20091104042152/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><a href="https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.fsis.usda.gov/sites/default/files/media_file/2020-07/BSE_Risk_Assess_Response_Public_Comments.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">see full submission;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission Comment from Terry Singeltary Posted by the Animal and Plant Health Inspection Service on Jun 19, 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2018-0087-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2018-0087-0002</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Control of Chronic Wasting Disease OMB Control Number: 0579-0189APHIS-2021-0004 Singeltary Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2021-0004-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0004-0002</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/document/APHIS-2018-0011-0003" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/document/APHIS-2018-0011-0003</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Comment from Singeltary Sr., Terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PUBLIC SUBMISSION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Comment from Terry Singeltary Sr.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Posted by the Food and Drug Administration on May 17, 2016 Comment</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.regulations.gov/comment/FDA-2003-D-0432-0011" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/FDA-2003-D-0432-0011</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FRIDAY, APRIL 07, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MONDAY, APRIL 24, 2023 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html</a></div><div style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">THE PATHOLOGICAL PROTEIN</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">June 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BY Philip Yam</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CHAPTER 14 LAYING ODDS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.thepathologicalprotein.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.thepathologicalprotein.com/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate infor-mation showed that “we miss about 14 percent,” said CDC epidemiolo-gist Lawrence Schonberger. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/chapter/10.1007/0-387-21755-x_14" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/chapter/10.1007/0-387-21755-x_14</a></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary, retired (medically), CJD WATCH</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Submitted March 26, 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">August 10, 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Greetings,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">But, while sub-clinical, how many can one exposed human infect? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">please see history, and the ever evolving TSE science to date ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saturday, June 13, 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary 2000</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BMJ 2000; 320 doi: <a href="https://doi.org/10.1136/bmj.320.7226.8/b" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1136/bmj.320.7226.8/b</a> (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">02 January 2000 Terry S Singeltary retired</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rapid Response: </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Something else I find odd, page 16;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A few more factors to consider, page 15;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To be continued...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary Sr. Bacliff, Texas USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Competing interests: No competing interests</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Tracking spongiform encephalopathies in North America</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xavier Bosch</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Available online 29 July 2003. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Volume 3, Issue 8, August 2003, Page 463 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Volume 3, Number 8 01 August 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Newsdesk</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Tracking spongiform encephalopathies in North America</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xavier Bosch</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a></div></div></div></div><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Singeltary 2007</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">by Philip Yam </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Revisiting Sporadic CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow.org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">223</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on www.vegsource.com and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">224 CHAPTER 14</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Laying Odds 225</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">226 CHAPTER 14</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A Case for Undercounting</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Laying Odds 227</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a one in-a-million lottery, it’s more like one-in-2.5-million for African Americans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SNIP...SEE FULL TEXT;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary Submission SEAC 2007</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div></div></div><div style="outline: none;"><div style="outline: none !important;">Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 McCullough Bldg. Galveston, Texas 77555-0785</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FAX COVER SHEET</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DATE: 4-23-98</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TO: Mr. Terry Singeltary @ -------</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FROM: Gerald Campbell</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FAX: (409) 772-5315 PHONE: (409) 772-2881</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Number of Pages (including cover sheet):</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Message:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*CONFIDENTIALITY NOTICE*</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Snip…</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FINAL AUTOPSY DIAGNOSIS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***TYPE: Anatomic(A) or Clinical(C)Diagnosis. IMPORTANCE: 1-immediate cause of death (COD); 2.ureterlying COD;3-contributory COD: 4.concomitant, significant; 5-incidental ***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed Date; Time: 01/30/98 - 0832</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Page: 1 Continued .... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">--------------</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Pathology Report</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Autopsy NO,: AU-97-00435</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MICROSCOPIC DESCRIPTION: The spongiform change is evident in all areas of neocortex, varying from mild to moderate in severity with only very mild neuronal loss and gliosis. In the bilateral occipital lobes, there is severe loss cortical neurons and gliosis, with a corresponding pallor of the underlying white matter. There is only minimal, focal spongiform change in corpus striatum, lentiform nuclei, thalamus, hippocampus, brainstem and cerebellum. There is no significant loss of neurons from the lateral geniculate nucleus, and the optic chiasm and tracts are well-myelinated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SECTIONS TAKEN: N-l) Pituitary, N-2)Right frontal, N-3) Right inferior frontal, N-4) Right caudate putamen. N-5)Right lentiform nuclei, N-6) Right hippocampus, N-7) optic chiasm. N-8) Left inferior temporal lobe, N-9) Right inferior occipital, N-10} Cerabellum. N-l1)Midbrain, N-12) Pons, N-13) Medulla.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FINAL DIAGNOSES: BRAIN: 1. Clinical history of rapidly progressive dementia, clinically consistent with Creutzfeldt-Jakob Disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">a. spongiform encephalopathy, most Severe in occipital lobes, consistent with Heidenhain variant of Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">b. Ventriculer enlargement, moderate, consistent with atrophy. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1. Communicating spherical enlargement of occipital horn of left lateral ventricle (possible incidental congenital anomaly).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DURA; Left subdural hemorrhage, recent, minimal.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PITUITARY: Severe capillary congestion.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">COMMENTS; See also western blot report from Dr. Gambetti's lab Amyloid stains are not completed for this case as of this date. The results, which are not essential for the diagnosis, will be reported separately in an addendum.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(this was hand written notes) no amyloid evident in the special stains. no evidence of plaques. GAE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gerald A. Campbell, M.D., Pathologist Division of Neuropathology</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(Electronic Signature}. (Gross: 01/16/98Final: 02/08/98</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Snip…</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Page: 6 END OF REPORT </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">--------------</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The University of Texas Medical Branch at Galveston</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gerald A, Campbell, Ph.D., M.D, Associate Professor and Director Division of Neuropathology Department of Pathology</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 26, 1998</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Pierluigi Gambetti, M.D. Professor Institute of Pathology Case Western Reserve University 2085 Adelbert Road Cleveland Ohio 44106</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Dr, Gambetti:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Enclosed please find the microscopic slides and autopsy report from our patient, Barbara Poulter (Hosp.# 11851lQ,Autopsy # AU97-435). These slides are being sent for consultation at the request of Mr. Singletary, Ms. Poulter's son and next of kin. We will also send frozen tissue from the brain on dry ice next week, and someone will call you on the day the tissue is shipped. Please return the slides when you have finished with your examination. If you need any further information, please do not hesitate to call me. Thanks for your assistance with this case.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sincerely, Gerald A. Campbell</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">------------------ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CASE WESTERN RESERVE UNIVERSITY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 26, 1988</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gerald Campbell, M.D,, PhD. Division of Neuropathology, G85 University TX Medical Branch Galveston, TX 77555-0785</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Dr. Campbell,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">As per our telephone conversation concerning a recent case of CJD, I Will be willing to examine slides and the frozen tissue on western blotting, I will issue a report to you about our conclusions. Below is my address, Our Fed Ex number is XXXXXXXXXXXXXXX.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thank your for your assistance in this matter,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Best personal regards,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Pierluigi Gambetti, M.D.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PG:In</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Division of Neuropathology Pierluigi Gambetti, M.D. Director Institute Of Neuropathology 2085 Adelbert RoadCleveland, Ohio 44106</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Phone 216-368-0587 Fax 216-368-2546</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">------------------ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CASE WESTERN RESERVE UNIVERSITY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 27, 1998</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dr. Gerald A. Campbell The University of Texas Medical Branch at Galveston Division of Neuropathology, G85 Galveston. TX77555-0785</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Dr. Campbell,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We are in receipt of the slides you senton Mrs. Barbara Poulter (your #: AU97-435;our#098-28).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Best personal regards, Pierluigi Gambetti, M.D.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PG:sb</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Division of Neuropathology Pierluigi Gambetti, M.D., Director</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-----------------------------------</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CASE WESTERN RESERVE UNIVERSITY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">March 30, 1998</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dr. Gerald A, Campbell The University of Texas Medical Branch at Galveston Division of Neuropathology Department of Pathology Galveston, Texas</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Dr Campbell,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We performed Western immunoblot analysis on the frozen tissue from your case #AU97-435 (our #098-28). The Immunoblot reveals the presence of protease-resistant prion protein (PrPres) confirming the diagnosis of prion disease. The immunoblot pattern of PrPres is consistent with the diagnosis of Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thank you for referring to us this interesting case.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sincerely,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Piero Parchi, M.D.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Pierluigi Gambetti, M.D.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PP:sb</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Division of Neuropathology Pierluigi Gambetti, M.D., Director Case Western Reserve University</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This Autopsy report is for the use of anyone, who is trying to understand this hideous disease CJD. I hope it can be beneficial for some in researching human TSE. Please remember, this was my Mom, and to use this with great respect.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">thank you, kind regards,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-------------------------------</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BARBARA FREDERICK POULTER</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DIED 12-14-97</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">If I had one last thing I could tell you, it would be, I love you. I'm sorry for the stupid argument we had the last few months, BEFORE this hideous disease ROARED through your body. BUT, I PROMISE MOM, YOUR DEATH WILL NOT GO UNANSWERED!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">HEIDENHAN VARIANT CREUTZFELDT JAKOBDISEASE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We got a call from my Mother around the end of Oct. saying "the damn'est thing has happened, I can't see, and if I'm talking to you and I don't make sense, bare with me, I'll come back". It was a shock to all of us. It seems that a few days before, she was crossing the ferry and became frightened because she was having problems seeing. She explained it as looking down a tunnel or not being able to see from the sides, and seeing brown spots.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We had NOT been talking, over something, we had NO control of, for a few months. So I did not know she had been having these visual problems, until she was blind. These were her first symptoms. From that point on, I was with her most everyday. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I had to cross the Galveston/Bolivar ferry, and its about 30 minutes each way, so as the disease progressed, it gave me a great deal of time to think. When the visual problems started, it was about 2 weeks later, and she was blind. That led to coordination, and balance problems starting. But as this hideous disease progresses, it just GOES. You don't seem to catch up with it. It was like a fire in a hurricane. We would go out and get her things she needed one day, and the next day it would be obsolete, because the disease had gone to another stage. So you started over. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Her coordination and balancing led to being in a wheel-chair. She was starting to get these trembles. I also noticed how her hands and feet started to go inward. Her speech was nothing more than jerble at this time, and this was probably about the 6th week, (at this point we had to tie her to the wheel chair, to keep her from falling out). The trembles had turned into SEVERE JERKS, that at times would take 3 of us to hold her down. I will never forget that....About her 8th week she became comatose....She died around the 10th week. I had spent the night, she had problems through the night, so the nurse came. She checked her out and comforted us, (HOSPICE IS AWONDERFUL ORGANIZATION). The nurse said she seemed to be alright and that it would probably be alright to go home for a few hours. I was on the Ferry, going back to Galveston, when I got the call, she was gone. What can you do, Mom was gone, and I was stuck on the Damn Ferry, going the wrong direction.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">She knew what she had. I remember, before she had lost her speech completely. After a doctors conference, and CJD had come up. She heard us say CJD, and she screamed, SHE knew! At that point, I didn't know what was, much less, CREUTZFELDT JAKOB DISEASE.....I have learned a lot since. I have learned I truly miss my Mom and I am MAD as hell that she is gone!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry/MADSON!!!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SYMPTOMS:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">VISION - BLIND IN ABOUT 10 TO 14 DAYS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">COORDINATION AND MUSCLE CONTROL, SWALLOWING DIFFICULTY, CONFUSION AND DEMENTIA, SPEECH PROBLEMS, HALLUCINATIONS, TREMBLES TOO SEVERE JERKING, LOSS OF WEIGHT, HANDS AND FEET GREW INWARD, UPPERTRUNK STIFFNESS, SHOULDER, UPPER ARM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.fortunecity.com/healthclub/cpr/798/terry.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.fortunecity.com/healthclub/cpr/798/terry.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20021105163654/http://www.fortunecity.com/healthclub/cpr/798/terry.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20021105163654/http://www.fortunecity.com/healthclub/cpr/798/terry.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD WATCH</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20021212055813/http://www.fortunecity.com/healthclub/cpr/798/cjd.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20021212055813/http://www.fortunecity.com/healthclub/cpr/798/cjd.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD WATCH DATA BASE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20210609070241/http://community.fortunecity.ws/healthclub/cpr/349/index-2.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20210609070241/http://community.fortunecity.ws/healthclub/cpr/349/index-2.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD VOICE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20030204221724/http://www.cjdvoice.org/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20030204221724/http://www.cjdvoice.org/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20021003034835fw_/http://members.aol.com/larmstr853/cjdvoice/joindiscussions.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20021003034835fw_/http://members.aol.com/larmstr853/cjdvoice/joindiscussions.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD BLOOD/RECALL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20020220130646/http://members.aol.com/debbieoney/blood.htm" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20020220130646/http://members.aol.com/debbieoney/blood.htm</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20011122034307/http://disc.server.com/Indices/154185.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20011122034307/http://disc.server.com/Indices/154185.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD FOUNDATION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20010207193157/cjdfoundation.org/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20010207193157/cjdfoundation.org/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20050602083950/http://cjdfoundation.org/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20050602083950/http://cjdfoundation.org/</a></div></div><div style="outline: none;"><br /></div><div style="outline: none;">wasted days and wasted nights...Freddy Fender<br style="outline: none;" /></div></div></div></div></div></div></div></div></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net</div></div><br style="outline: none;" /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-86844877183107795182023-11-26T12:03:00.006-06:002023-11-26T12:03:49.974-06:00The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis<p><span style="background-color: white; font-family: arial; font-size: 16px;">Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 </span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023</div><div style="outline: none !important;"><br /></div><div style="outline: none !important;">The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Angéline Denouel1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Jean-Philippe Brandel1,2 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Danielle Seilhean1 · Jean-Louis Laplanche3,4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Alexis Elbaz5 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">· Stéphane Haik1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We included all cases aged 45–89 years old who died with a probable/definite sCJD diagnosis based on the French national surveillance network. We used age-period-cohort (APC) Poisson regression models to study variation in mortality rates by sex, age, period, and time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A total of 2475 sCJD cases aged 45–89 years were included. Mortality rates increased with age, reached a peak between 75 and 79 years, and decreased thereafter. Mortality rates were higher in women than men at younger ages and lower at older ages. The full APC model with a sex×age interaction provided the best fit to the data, thus in favour of sex, age, period, and cohort effects on mortality rates. In particular, mortality rates increased progressively with successive birth cohorts.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Based on 25 years of active surveillance in France, we show evidence for sex, age, period, and cohort effects on sCJD mortality. The identification of cohort effects suggests that environmental exposures may play a role in sCJD etiology.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Keywords Age-period-cohort model · Prion · Temporal trend · Sporadic Creutzfeldt-Jakob disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Worldwide, the number of patients with sCJD appears to have progressively increased over time [13]. This increase can be partly explained by increasing life expectancy as well as by better case ascertainment due to improved diagnostic tests and awareness of the disease among clinicians. Indeed, a relationship between surveillance intensity and sCJD incidence has been shown [14]. It cannot be excluded, however, that an actual increase of sCJD cases has occurred, and this hypothesis can be examined using age-period-cohort (APC) models.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In this paper, we estimated mortality rates from sCJD in France over a 25-year period (1992–2016) based on data from the French national surveillance network.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s10654-023-01004-5" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s10654-023-01004-5</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Experimental transmission of ovine atypical scrapie to cattle Experimental transmission of ovine atypical scrapie to cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy McKenna, Lauren Read, Sara Canoyra, Alba Marín-Moreno & Juan María Torres </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Veterinary Research volume 54, Article number: 98 (2023) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Classical bovine spongiform encephalopathy (BSE) in cattle was caused by the recycling and feeding of meat and bone meal contaminated with a transmissible spongiform encephalopathy (TSE) agent but its origin remains unknown. This study aimed to determine whether atypical scrapie could cause disease in cattle and to compare it with other known TSEs in cattle. Two groups of calves (five and two) were intracerebrally inoculated with atypical scrapie brain homogenate from two sheep with atypical scrapie. Controls were five calves intracerebrally inoculated with saline solution and one non-inoculated animal. Cattle were clinically monitored until clinical end-stage or at least 96 months post-inoculation (mpi). After euthanasia, tissues were collected for TSE diagnosis and potential transgenic mouse bioassay. One animal was culled with BSE-like clinical signs at 48 mpi. The other cattle either developed intercurrent diseases leading to cull or remained clinical unremarkable at study endpoint, including control cattle. None of the animals tested positive for TSEs by Western immunoblot and immunohistochemistry. Bioassay of brain samples from the clinical suspect in Ov-Tg338 and Bov-Tg110 mice was also negative. By contrast, protein misfolding cyclic amplification detected prions in the examined brains from atypical scrapie-challenged cattle, which had a classical BSE-like phenotype. This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This is the first study in cattle inoculated with naturally occurring scrapie isolates that found the presence of prions resembling classical BSE in bovine brain although this was limited to detection by the ultrasensitive PMCA. The results from thermostability assay confirmed that the isolates were as thermoresistant as the BSE agent as proven in other studies [36, 48]. Previous PMCA studies with various British atypical scrapie isolates did not find any evidence of amplification [49, 50]. This may be explained by the use of ovine brain as substrate rather than brain from Bov-Tg110 mice, which may facilitate conversion to classical BSE prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Two hypotheses for prion strain propagation in cross-species transmission experiments have been proposed: conformational selection favours a particular strain conformation out of a mixture of conformations in a scrapie isolate whilst mutation results in the conformational shift of one conformation into another [51]. Following on from the study in mice [17], it has been subsequently suggested that classical BSE properties that arise in atypical scrapie isolates transmitted to cattle may be due to conformational mutation in a new host [52]. It does not confirm that the atypical scrapie agent is the origin of the classical BSE epidemic and further transmission studies would be required to see whether classical BSE can be generated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Would PMCA applied to brains from cattle exposed to TSE agents other than classical BSE and atypical scrapie also produce a classical BSE-like molecular phenotype? The PMCA product obtained in the thermostability test using a thermosensitive classical scrapie control showed a profile unlike classical BSE. Atypical BSE has been linked to the origin of classical BSE because of its conversion into classical BSE following serial passages in wild-type mice (L-type BSE [11]) and bovine transgenic mice (H-type BSE [53]). Although we have not tested PMCA products of atypical BSE isolates as part of this study, there is no evidence that PMCA products from atypical BSE convert into classical BSE, at least for H-type BSE using bovine brain as substrate [54]. In fact, we were unable to propagate H-type BSE using the same methodology (S Canoyra, A Marín-Moreno, JM Torres, unpublished observation).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The study results support the decision to maintain the current ban on animal meal in feedstuffs for ruminants, particularly as atypical scrapie occurs world-wide, and eradication is unlikely for a sporadic disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In summary, experimental inoculation of cattle with the atypical scrapie agent may produce clinical disease indistinguishable from classical BSE, which cannot be diagnosed by conventional diagnostic tests, but prions can be amplified by ultrasensitive tests in both clinically affected and clinically unremarkable cattle, which reveal classical BSE-like characteristics. Further studies are required to assess whether a BSE-like disease can be confirmed by conventional tests, which may initially include a second passage in cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-023-01224-3</a></div></div></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Presentation Type: Oral Presentation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: ALMA/APRI: 201400006, HC 414250</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">34 Scientific Commission/September 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. Atypical BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations expand</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMID: 21266763</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full text;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) </span><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (<span dir="ltr" style="outline: none !important;">Tg338</span> and <span dir="ltr" style="outline: none !important;">Tg501</span>). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: No evidence of transmission was found on a first passage in <span dir="ltr" style="outline: none !important;">Tg338</span> nor <span dir="ltr" style="outline: none !important;">Tg501</span>ovinized mice, but on second passage, <span dir="ltr" style="outline: none !important;">4/10</span> <span dir="ltr" style="outline: none !important;">Tg338</span> mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and <span dir="ltr" style="outline: none !important;">1/12</span> <span dir="ltr" style="outline: none !important;">Tg501</span> mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed. </div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Transmission of scrapie prions to primate after an extended silent incubation period</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***thus questioning the origin of human sporadic cases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2015 CONFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"></div><div style="outline: none !important;">PRION <span dir="ltr" style="outline: none !important;">2016 TOKYO</span></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, April 23, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SCRAPIE <span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr" style="outline: none !important;">1933-6896</span> 1933-690X </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><span dir="ltr" style="outline: none !important;">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Tuesday, December 16, 2014</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Evidence for zoonotic potential of ovine scrapie prions</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the humanprion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. ***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. ***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Subject terms: Biological sciences• Medical research At a glance</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">why do we not want to do TSE transmission studies on chimpanzees $</div><div style="outline: none !important;"> </div><div style="outline: none !important;">5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">R. BRADLEY</div><div style="outline: none !important;"> </div><div style="outline: none !important;"> <a href="http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">1: J Infect Dis 1980 Aug;142(2):205-8</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">PMID: 6997404</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"> </div><div style="outline: none !important;">76/10.12/4.6</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">Nature. 1972 Mar 10;236(5341):73-4.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Gibbs CJ Jr, Gajdusek DC.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)</div><div style="outline: none !important;"> </div><div style="outline: none !important;">C. J. GIBBS jun. & D. C. GAJDUSEK</div><div style="outline: none !important;"> </div><div style="outline: none !important;">National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland</div><div style="outline: none !important;"> </div><div style="outline: none !important;">SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://scrapie-usa.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://scrapie-usa.blogspot.com/</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://nor-98.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://nor-98.blogspot.com/</a></div><div style="outline: none !important;"> </div><div style="outline: none !important;">2001</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Suspect symptoms</div><div style="outline: none !important;"> </div><div style="outline: none !important;">What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?</div><div style="outline: none !important;"> </div><div style="outline: none !important;">28 Mar 01</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Like lambs to the slaughter</div><div style="outline: none !important;"> </div><div style="outline: none !important;">31 March 2001</div><div style="outline: none !important;"> </div><div style="outline: none !important;">by Debora MacKenzie Magazine issue 2284.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."</div><div style="outline: none !important;"> </div><div style="outline: none !important;">There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.</div><div style="outline: none !important;"> </div><div style="outline: none !important;">Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.</div><div style="outline: none !important;"> </div><div style="outline: none !important;"><a href="http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">REPORT OF THE ADVISORY COMMITTEE ON SCRAPIE 1976</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">IN CONFIDENCE</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">Such considerations suggest first that those responsible for work with scrapie should be selected with as much care as are astronauts. </div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102161333mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190607mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12003001.pdf</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102190630mp_/http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">THURSDAY, NOVEMBER 9, 2023 <br style="outline: none !important;" /></div></div></div></div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annual Report of the Scientific Network on BSE-TSE 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">European Food Safety Authority (EFSA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">APPROVED: 25 October 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">LOT OF SPONTANEOUS ATYPICAL BSE GOING ON...terry</div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">WEDNESDAY, NOVEMBER 08, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Ireland Atypical BSE confirmed November 3 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-confirmed-november.html</a> </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;">FRIDAY, MAY 19, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bovineprp.blogspot.com/2023/05/usda-announces-atypical-l-type-bovine.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"> </div></div><a href="https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/122/announces-atypical-bovine-spongiform-encephalopa</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">SATURDAY, MAY 20, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">ABOUT 2+ WEEKS BEFORE THE DETECTION OF BSE IN THE USA IN 2023, I WROTE THIS;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Docket No. APHIS–<span dir="ltr" style="outline: none !important;">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full submission;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div></div></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">RECENT MAD COW CASES 2023</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">TUESDAY, NOVEMBER 14, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Ireland Atypical BSE case, 3 progeny of case cow to be culled </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/11/ireland-atypical-bse-case-3-progeny-of.html</a></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;">SUNDAY, JULY 16, 2023 </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;">Switzerland Atypical BSE detected in a cow in the canton of St. Gallen </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html" rel="nofollow" style="color: #196ad4; font-family: arial; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/07/switzerland-atypical-bse-detected-in.html</a><br style="outline: none !important;" /></div></div></div><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">WAHIS, WOAH, OIE, REPORT Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland Bovine Spongiform Encephalopathy Atypical L-Type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Switzerland - Bovine spongiform encephalopathy - Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></div></div></div></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;">Monday, March 20, 2023 <br style="outline: none !important;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;">WAHIS, WOAH, OIE, REPORT United Kingdom Bovine Spongiform Encephalopathy Atypical H-Type </div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4977</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gov.uk/government/news/single-case-of-atypical-bse-confirmed-on-a-farm-in-cornwall</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none !important;"><a href="https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/03/wahis-woah-oie-report-united-kingdom.html</a></div></div></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE START DATE 2023/01/18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE CONFIRMATION DATE 2023/02/22</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BRAZIL BSE END DATE 2023/03/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE START DATE 2023/01/21</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE CONFIRMATION DATE 2023/02/03</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SPAIN BSE END DATE 2023/02/06</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE START DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE CONFIRMATION DATE 2023/02/01</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NETHERLANDS BSE END DATE 2023/03/13</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div></div></div></div></div></div></div><div style="outline: none !important;"><div style="outline: none !important;">Monday, November 13, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Unforeseen decrease of full-length prion protein in macaques exposed to prion contaminated blood products</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel COMOY, Nina JAFFRE, Jérôme DELMOTTE, Jacqueline MIKOL, and Jean Philippe DESLYS Commissariat à l’Energie Atomique, DRF/IBFJ/SEPIA, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The presence of prion infectivity in blood from patients affected by variant of Creutzfeldt-Jakob disease (v-CJD) questions the risk of its inter-human transmission through transfusion. We have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD; however, after an exposure to low infectious doses, the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement which does not fulfill the classical diagnostic criteria of v-CJD, notably concerning the pathognomonic accumulation of abnormal prion protein. Here we aim to investigate the etiology and physiopathology of this original myelopathy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: CNS (brain and spinal cord) samples from myelopathic macaques were tested with different biochemical approaches in comparison to samples derived from either healthy animals or their counterparts exposed to different strains of prion diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Current conventional techniques failed to detect any accumulation of abnormal prion protein (PrPv-CJD) in the CNS of these myelopathic animals. Conversely, in their spinal cord we observed an alteration of their physiological cellular PrP pattern: PrP was not detectable under its full-length classical expression but mainly under its physiological terminal-truncated C1 fragment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: We here confirm the prion origin of this original syndrome, with a very specific biochemical signature linked to changes in PrP at the level of spinal cord lesions: contrary to what is classically described in prion diseases, host PrP is here altered in a form that is abnormally sensitive to degradation by cellular catabolism. This could provide the first experimental evidence of a link between loss of function of the cellular prion protein and the onset of disease. These observations open up new horizons in the field of prion diseases, which has hitherto been limited to pathologies associated with abnormal changes in cellular PrP towards highly structured conformations, with the possibility of unsuspected prion mechanisms/origins in certain neurodegenerative disorders.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Financial support for the study was provided by the French National Research Agency (ANR).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: ANR-10-BLAN-133001 and BIOTECS2010-BloodSecur</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We specially thank N. Lescoutra, A. Culeux, V. Durand, E. Correia, C. Durand and S. Jacquin for precious technical assistance</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none !important;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none !important;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none !important;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none !important;">tg650</span> with fecal homogenates. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Published</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Accepted: 7 August 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><p class="ydp7fad6b93yiv2780175211p1" style="font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="ydp7fad6b93yiv2780175211s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</span></p></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH and USDA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">end... <br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Fortuitous generation of a zoonotic cervid prion strain </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (<span dir="ltr" style="outline: none !important;">Tg12</span>), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the <span dir="ltr" style="outline: none !important;">Tg12</span> mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (<span dir="ltr" style="outline: none !important;">Tg12</span>) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the <span dir="ltr" style="outline: none !important;">Tg12</span> mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively</div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time."<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A probable diagnostic marker for CWD infection in humans </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xu Qi, Liuting Qing, Manuel Camacho, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Multiple in vitro CWD-seeded human PrP conversion experiments and some animal model studies indicate that the species barrier for CWD to human transmission can be overcome, but whether CWD prion can infect humans in real life remains controversial. The very limited understanding on the likely features of CWD infection in humans and the lack of a reliable diagnostic marker for identification of acquired human CWD cases contribute to this uncertainty. We aim to stablish such a reliable diagnostic marker for CWD infections in humans should they occur. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: A couple of PrPSc-positive spleens were identified from humanized transgenic mice inoculated with either CWD or sCJDMM1. Prions in these spleens were compared by bioassays in cervidized or humanized transgenic mice. A couple of PrPSc-positive spleens from UK sCJDMM1 patients were also examined similarly as controls with no exposure to CWD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: We have detected two prion-positive spleens in humanized transgenic mice inoculated with some CWD isolates. Such experimentally generated splenic “humanized” CWD prions (termed eHuCWDsp) appear indistinguishable from prions in the brain of sCJDMM1 patients on Western blot. We compared eHuCWDsp with prions in the spleen from humanized mice infected with sCJDMM1 (termed sCJDMM1sp) by bioassays in cervidized or humanized transgenic mice. Significantly, we found that eHuCWDsp can efficiently infect not only the humanized mice but also cervidized transgenic mice, and cervidized mice infected by eHuCWDsp produced PrPSc and brain pathology that are practically identical to those of CWD-infected cervidized mice. In contrast, sCJDMM1sp, similar to prions from sCJDMM1 patient brains, is poorly transmissible in the cervidized mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data demonstrate that high transmissibility with CWD features of splenic prions in cervidized transgenic mice is unique to acquired human CWD prions, and it may serve as a reliable marker to identify the first acquired human CWD cases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====end </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Prion 2023 Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Sarah Zurbuchena,b , S. Jo Moorea,b , Jifeng Biana , Eric D. Cassmanna , and Justin J. Greenleea . a. Virus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, US b. Oak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, United States </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (<span dir="ltr" style="outline: none !important;">Tg12</span>; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection, analysis, decision to publish, or preparation of the manuscript. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Ami Frank and Kevin Hassall for their technical contributions to this project.</div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">=====end </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">''Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">31 TAC §§65.82, 65.85, 65.88</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results show positive prion detection in all products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div style="outline: none !important;"><div style="outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none !important;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none !important;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none !important;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none !important;">tg650</span> with fecal homogenates. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> <a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">WEDNESDAY, NOVEMBER 01, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">TEXAS CHRONIC WASTING DISEASE RISES SUBSTANTIALLY TO 575 CONFIRMED CWD CASES TO DATE<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/11/texas-chronic-wasting-disease-rises.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Prion 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: N/A</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: XXX</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">22 years ago;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><div style="outline: none !important;">2001 Singeltary on CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 14, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, Sr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">FRIDAY, JANUARY 15, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">if not, why not?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD FOUNDATION Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">UK CJD Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a> </div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">cjd questionnaire 1979</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf</a></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">CJD Questionnaire </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div>NOW we know that CWD will transmit to cattle, pigs and sheep, by oral routes, seems some of the old documents pertaining to Pigs and BSE might come to light...terry</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #338fe9; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2007/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/</a> </div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">MONDAY, SEPTEMBER 11, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">Singeltary sCJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;">wasted days and wasted nights...Freddy Fender</div></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">terry</div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas, 77518, Galveston Bay, flounder9@verizon.net, </div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">on the bottom!</div><div><br /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-60206740426744138252023-11-22T15:55:00.001-06:002023-11-22T15:55:14.992-06:00Creutzfeldt-Jakob Disease in Mexico (1990-2023)<p><span style="background-color: white; font-family: arial; font-size: 16px;">Creutzfeldt-Jakob Disease in Mexico (1990-2023): Survival analysis, population characteristics and the first bioinformatics analysis database</span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Fabricio Cruz-Lópeza, b, c, Gustavo Reyes-Teránd, Petra Yescas-Gómezb, Sergio Iván Valdés-Ferrerc, e, f, Miguel Ángel Ramírez-Garcíab, Óscar Arias-Carrióng, Marie Catherine Boll-Woehrlenb, Carlos Alberto Gómez-Pérezb, Pablo Eduardo Irigoyen-Ruízh, i aFacultad de Medicina, Benemérita Universidad Autónoma de Puebla, H. Puebla de Zaragoza, Mexico; bGenetics Department, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Mexico City, Mexico; c Department of Neurology and Psychiatry, Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, Mexico City, Mexico; dCoordinating Commission of the National Institutes of Health and High Specialty Hospitals, Ministry of Health, Mexico City, Mexico; eInstitute of Bioelectronic Medicine, Feinstein Institutes for Medical Research, Manhasset, NY, USA; f Department of Infectious Diseases, Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, Mexico City, Mexico; gUnidad de Trastornos del Movimiento y Sueño, hHospital General Dr. Manuel Gea González, Mexico City, Mexico; i Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico City, Mexico</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Aims: Create the first database of Mexican patients with Creutzfeldt-Jakob Disease (CJD) from 1990 to 2023, to describe survival and presenting clinical features.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Materials and Methods: We performed a systematic scoping review from all published reports of Mexican patients with CJD following the PRISMA methodology. The quality was evaluated through the scale of The Joanna Briggs Institute. Likewise, we included new patients from INCMNSZ and INNN centers. Nonparametric estimation of Kaplan-Meier survival analysis was performed in R.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Results: Seventy-five Mexican patients with CJD were identified between 1990 and 2023; the median of age was 54 yo (ranges 20-90 yo). The main reported manifestations were: cerebellar or visual alterations (n=57), followed by myoclonus (n=43) and extrapyramidal alterations (n=41). The KaplanMeier curves show no differences in mean survival by gender (≈275 days); however, those patients reporting cerebellar and visual alterations (≈275 days) have longer survival compared to those who do not (≈125 days); Likewise, when grouping the Definite or Probable diagnosis (>400 days) in comparison to those with Possible diagnosis (200 days) of CJD; and patients with Familial etiology (≈325 days) versus patients with Sporadic etiology (≈275 days) we not found statistical differences.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Conclusions: The Creutzfeldt-Jakob Mexican cases show a longer survival than reported elsewhere; however, it could be due to classification bias (in the case of Possible diagnosis). Furthermore, the lack of application of diagnostic tools (such as 14-3-3, TAU, Biopsy, PRNP gene analysis, or RT-QUIC) does not allow the determination of Definitive or Probable diagnoses, which increases the uncertainty of misdiagnosed cases. </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">More information is available at: <a href="https://rpubs.com/MrKristarlx07/PrionMex_survival_analysis" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://rpubs.com/MrKristarlx07/PrionMex_survival_analysis</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Funded by: F.C.-L. has the support of the Consejo de Ciencia y Tecnología del Estado de Puebla, as well as the Vicerrectoría de Investigación y Estudios de Posgrado of the Benemérita Universidad Autónoma de Puebla (Autonomous University of Puebla).</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><span style="outline: none !important;">''The Creutzfeldt-Jakob Mexican cases show a longer survival than reported elsewhere; however, it could be due to classification bias (in the case of Possible diagnosis). Furthermore, the lack of application of diagnostic tools (such as 14-3-3, TAU, Biopsy, PRNP gene analysis, or RT-QUIC) does not allow the determination of Definitive or Probable diagnoses, which increases the uncertainty of misdiagnosed cases.''</span></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><span style="outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Prion 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: N/A</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: XXX</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">22 years ago;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><div style="outline: none !important;">2001 Singeltary on CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">February 14, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary, Sr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">MONDAY, SEPTEMBER 11, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Professor John Collinge on tackling prion diseases </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a> </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">FRIDAY, JANUARY 15, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">if not, why not?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CJD FOUNDATION Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">UK CJD Questionnaire</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a> </div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">cjd questionnaire 1979</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf</a></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">CJD Questionnaire </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div>NOW we know that CWD will transmit to cattle, pigs and sheep, by oral routes, seems some of the old documents pertaining to Pigs and BSE might come to light...terry</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #338fe9; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://cjdquestionnaire.blogspot.com/2007/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://cjdquestionnaire.blogspot.com/</a> </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">seems to be a colossal waste of time on my part, 23 years and nothing, were still talking about the same failures, imo...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">wasted days and wasted nights...Freddy Fender</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">SOME HISTORY ON MEXICO, BSE, SCRAPIE, CWD, CJD, TSE, PRION</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">MEXICO BSE GBR</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of MEXICO Question N° EFSA-Q-2003-083</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Adopted July 2004</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Summary</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in Mexico, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Mexico. This scientific report addresses the GBR of Mexico as assessed in 2004 based on data covering the period 1980-2003.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The BSE agent was probably imported into Mexico and could have reached domestic cattle. These cattle imported could have been rendered and therefore led to an internal challenge in the mid to late 1990’s. It is possible that imported meat and bone meal (MBM) into Mexico reached domestic cattle and leads to an internal challenge around 1993.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’ MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late 1990s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable. Thus it is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> EFSA concludes that the current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSEagent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20141222021040/http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20141222021040/http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Key words: BSE, geographical risk assessment, GBR, Mexico, third countries </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Annex to the EFSA Scientific Report (2004) 4, 1-13 on the Assessment of the Geographical BSE Risk of Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> - 7 -</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2.3 Overall assessment of the external challenge</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The level of the external challenge that has to be met by the BSE/cattle system is estimated according to the guidance given by the SSC in its final opinion on the GBR of July 2000 (as updated in January 2002).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Live cattle imports:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> According to the CD the country imported in total over the period 1980 to 2003, approximately 3.2 million live cattle from BSE - risk countries, of which conclusively none came from the UK. The numbers shown in table 1 are the raw import figures and are not reflecting the adjusted imports for the assessment of the external challenge. Broken down to 5 - years periods the resulting external challenge is as given in table 3. This assessment takes into account the evidence that certain imported cattle did not enter the domestic BSE/cattle system, i.e. were not rendered into feed. In the case of Mexico, it is assumed that “cattle still alive” (imports from Spain) did not enter the rendering system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> MBM imports:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> According to the CD the country imported in total over the period 1980 - 2003 approximately 826,000 tons MBM from BSE - risk countries (according to “other data”: ~ 919,000 tons), of which none came from the UK. The numbers shown in table 2 are the raw import figures and are not reflecting the adjusted imports for the assessment of the external challenge. Broken down to 5 - years periods the resulting external challenge is as given in table 3. This assessment takes into account the evidence that certain imported MBM did not enter the domestic BSE/cattle system or did not represent an external challenge for other reasons. However, in the case of Mexico, there was not sufficient evidence to remove any quantities of MBM from the external challenge.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Annex to the EFSA Scientific Report (2004) 4, 1-13 on the Assessment of the Geographical BSE Risk of Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> - 12 -</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> would harbour, while being pre - clinical, as much infectivity as a clinical BSE case. Hence cattle imports could have led to an internal challenge about 3 years after the import of breeding cattle (that are normally imported at 20 - 24 months of age) that could have been infected prior to import. In case of Mexico this implies that an internal challenge caused by live cattle imports (predominantly from USA or Canada) first occurred in the mid to late 1990’s and continued to the present.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">On the other hand imports of contaminated MBM would lead to an internal challenge in the year of import, if fed to cattle. The feeding system is of utmost importance in this context. If it could be excluded that imported, potentially contaminated feed stuffs reached cattle, such imports might not lead to an internal challenge at all. In case of Mexico this implies that an internal challenge caused by MBM imports (predominantly from USA or Canada) first occurred around 1993 and continued to the present.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In view of the above - described consideration the combination of the very / extremely high external challenges with a very unstable system makes the occurrence of an internal challenge likely in Mexico from approximately 1993 onwards.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 4.2 Risk that BSE infectivity entered processing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’ MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late 1990’s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable, leading to increased internal challenge.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 4.3 Risk that BSE infectivity was recycled and propagated It is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 5. CONCLUSION ON THE GEOGRAPHICAL BSE – RISK</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 5.1 The current GBR as function of the past stability and challenge The current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 5.2 The expected development of the GBR as a function of the past and present stability and challenge </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">• The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20150405091317/http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20150405091317/http://www.efsa.europa.eu/en/efsajournal/doc/4r.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2004.4r" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/j.efsa.2004.4r</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">http://www.oie.int/boutique/extrait/06heim937950.pdf </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20100415142358/http://www.oie.int/boutique/extrait/06heim937950.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20100415142358/http://www.oie.int/boutique/extrait/06heim937950.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: Mexico SAGARPA Assessment of BSE VS EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of Mexico </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: February 5, 2007 at 1:11 pm PST</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Empresa solicitante: SAGARPA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Tipo del análisis efectuado: Cuantitativo</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Temática: “Análisis de riesgo sobre la ocurrencia de la encefalopatía espongiforme bovina en México”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> INTRODUCTION:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The bovine spongiform encephalopathy (BSE), it is a neurological disease, invariably fatal and with long period of incubation, that affects cattle. Its etiologic agent is the prion. General consensus exists with respect to that the feeding of contaminated meat and bone flours, it is the most significant source in the dissemination and transmission of this etiologic agent. At this time there is no exist evidence that BSE is transmitted by means of embryos, their semen and in case of existing maternal transmission, if this could happened it would be in a so extremely low rate that it could not be considered like a trigger or leading factor of an epidemic. Controversy in respect to other probable ways of transmission remains. The BSE was diagnosed for the first time in 1986 in the United Kingdom. At this time it exists in 26 countries, including a Canada and the United States of North America (USA).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> This document summarizes the analyzed elements and the results of the study of the evaluation of the risk factors, of the epidemiology surveillance and related activities, as well as the quantitative estimation of the risk with respect to the probability of introduction of the disease to the Mexican herd.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> EVALUATED ELEMENTS:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Demography and characteristics of the Mexican cattle industry: Cattle is one of the main activities in the Mexican farming sector, due to its contribution in the supply of meat (beef) products, dairy, among others; as well as its participation in the international trade on cattle exports, mainly to the United States of North America. According to data of the 2001, cattle population is of 30.620.933 of heads, of which 28.480.803 are beef cattle and 2.140.130 dairy cattle. The main cattle production states are located in the center-north, where its operation is intensive and its feeding is based on grains; as well as in the coast of the Gulf of Mexico and the south-southeastern, with intensive programs and feeding is based mainly on the pasturing (grass). The national dairy herd, is calculated as specialized or technified that represent 17,44% of the herd, semi-specialized 14,90% of the herd, double-purpose herd (beef and dairy) 59,68% and the small family-run herd or the referred as “backyard” (traspatio) 7,98%.Previous numbers are to be considered as an estimation of the dairy livestock inventory by production units. Nevertheless, it is necessary to consider that all races of pure breed can be found in anyone of those groups.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Legal grounds: Mexico counts on a normative frame that covers (deals with) the relevant aspects of the Epidemiology Surveillance of the BSE, like the Federal Law of Animal Health, the Federal Law of Metrology and Regulation, the General Law of Health and several Mexican Official Norms (NOM-009-Z00-1994, Sanitary process of the meat, NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products destined for animal feed and NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding). Wider and extended covertures of these regulations were evaluated. Veterinary infrastructure: The veterinary services in the country are structural and normative organized by the Mexican State through the Secretariat of Agriculture, Livestock, Rural Affairs, Fishery and Alimentary (SAGARPA) Federally Empower, that is to say, that has the capacity and authority to negotiate an to come to agreement with the States Governments that integrate the Republic; to coordinate itself with the other Secretariats of State; to deal with organizations of the Private and Social sector as well as with the rest of the Civil Society as a whole.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The National Service of Health, Food Safety and Ag-alimentary Quality (SENASICA), it is an organism of this Secretariat, which has attributions in the matter of vegetable health, animal health and ag-alimentary safety and is conformed by the following main directorates: Sanidad Vegetal, Salud Animal, Inocuidad Agroalimentaria, Acuícola y Pesquera, Inspección Fitozoosanitaria, (equivalent to U.S. APHIS, FSIS and VS –Veterinary Services) Jurídica, Administración e Informática. In accordance with the assigned attributions, it corresponds to central offices the substantive part and the operative part, to the personnel assigned to the State Delegations of the SAGARPA and other instances of the SENASICA.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Consequently, the four main areas are assigned to the Main directorate of Salud Animal-Animal Health (DGSA) and to the Main directorate of Inspección Fotozoosanitaria-Plant Inspection (DGIF) and Veterinary Services (SV) in Mexico are in charge of: surveillance, epidemiology, animal movement, zoo-sanitary campaigns and emergencies.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Imports This is perhaps one of the medullar points, in the sense that it represents the information of the imports made during the risk periods and therefore, it provides the fundamental information for the risk assessment. In 1991, Mexico implemented measures to avoid the appearance of BSE, as the disease had become a serious worldwide problem, reason why, live bovines imports were prohibited, beef, beef products and by-products and in 1994 flour of meat and bone from countries affected by this disease was also prohibited and in the 2000 MBM feeding ban was imposed. In order to mitigate the risk of transmission of the EEB, a revision of the established requirements for import for ruminants’ products began.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Cattle imports and its products and by-products, as well as specific risk materials played a very important role in this study, where considerable amounts of cattle imports from countries now affected by BSE were identified, countries that at the time of the import they remained clean and therefore just some preventive risk measures were in place.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Slaughter, Cattle disposition and Offal.- Different cattle slaughter schemes were analyzed as well as the processes in use, finding some significant differences among them, being the most important the sanitary jurisdiction of the organizations that regulate us.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In Mexico, the slaughter is divided in three different systems, Federal Inspection Type Plants (TIF), which has been increased in the past years; in 1992 they participated with the 13,5%, in 1997 with the 19,40% and in 2002 with the 26,60% of the national total. In the case of the municipal slaughterhouses from 1992 to 1997, their slaughtered animals corresponded to the 49,5% and for 2002 it was increased to 73,4%, whereas the slaughter in private plants decreased of 37,10% in 1992 to 31,10%, in 1997 and from 1998 to date, we have no information.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The procedures to be followed by the establishments in the animal slaughter and those that industrialize, process, packing, chilled/froze beef products or by-products for human consumption, in order to obtain products of optimal hygienic quality, are written in the NOM-009-ZOO-1994 “sanitary Process of Beef”.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The direct consumption of beef can be stratified in three great destinies, differentiated by the market that are destined to, the rural one, the one of small centers of population, (and) the one of the big cities, characterized each one of them by its consumption and the partial or integral industrialization by direct consumer and by means of commercialization or points of sale, as well as for the origin of the own supplier.(?) Rendering of Cattle Products.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The processes applied by the rendering plants for obtaining the protein from inedible offal, were evaluated. Food elaboration and its use for animal feeding.- This analysis was focused in the processes of food elaboration for animal consumption.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In Mexico, the control in the production of food from animal origin, as much as the elaboration of the meat flour as that of the balanced food manufacture it is regulated by the Mexican Official Norm NOM-061-ZOO-1999, “Zoo-sanitary Specifications of nutritional products for animal consumption”, which bans the use of MBM flours of ruminant origin or any mixture that contains it for the elaboration of balanced meals for ruminants, and the Mexican Official Norm NOM-060-ZOO-1999, “Zoo-sanitary Specifications for the transformation of animal offal and its use in the animal feeding”.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In accordance with the Section of Manufacturers of Balanced Food for Animals of the National Camera of the Industry of Transformation (CANACINTRA), there are 396 balanced food plants registered, same that have the capacity to produce more than 20 million tons a year, according to the numbers registered during 1999-2002. 63% of such plants are integrated and produce 64% of the animal feed produced nationwide, the rest corresponds to commercial plants.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The animal feed produced by the integrated plants, that is the most significant part, during the 2002 it produced the 58,7% of the products destined for raising of poultry, the 16,5% for swine, the 14,3% for dairy and 9,2% for feedlots (cattle) and 1,3% for other species.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> As far as the composition of the main raw materials to produce balanced foods, these mainly correspond in 45% to domestic sorghum and 55% sorghum concentrated; 16% to domestic yellow maize and 84% imported; 91% domestic protein pastes and 9% imported; 80% of other domestic forage grains (broken maize, wheat, barley, oats, etc.) and 20% imported and other ingredients (wheat by-products, maize, vitamins, minerals, oils, etc.).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> PARAGRAPH – BLOCKED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PARAGRAPH – BLOCKED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> PARAGRAPH – BLOCKED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> PARAGRAPH – BLOCKED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> PARAGRAPH – BLOCKED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Neuropathies in Mexico, Epidemiology Surveillance Program.- For this analysis, the legal elements related to the notification of the BSE were taken into account, in Mexico, as well as the activities made by the Commission Mexico - United States for the Prevention of the Aftosa Fever and Other Exotic Diseases of Animals (CPA), official entity in charge of carrying out this activity and other connected activities as training, taking of samples and the diagnosis of laboratory.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE Diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Veterinary Services diagnoses capacity was evaluated as well as its adherence to the international standards, according to what is indicated by the International Organization of Animal Health (OIE), as well as the processes of taking and shipping of samples.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> For the diagnosis of the BSE, the OIE recommends five laboratory tests: Histopathology (HP), Immunohistochemistry (IHQ), Western blot (immunotransferency), ELISA (enzimoinmunoassay) and Bio-assay in mouse. At this time Mexico counts with two laboratories of diagnosis for this disease: the National Center of Services of Diagnosis in Animal Health (CENASA) and the Laboratory of high security of the CPA. The CENASA performs the histopathology test and at the CPA the Immunohistochemistry test is carried out.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The reception of samples at CPA, it depends to a great extent on the economic resources which are accounted for this activity, expense that is approximately of $400,00 ($ 36.50 USD.) per sample received (includes the material for conservation, packing and shipping), reason for what, have to wait for the collection of several to be sent at the same time and in order to reduce costs, but delaying the result. As the CPA does not have a certified pathologist to carry-out the HP test technique, these samples are sent to the CENASA for their diagnosis; this implies that such samples are stored by approximately one week, since it doesn’t have the human resources for its transfer.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The main problem at CENASA, for the right operation of the diagnosis of the BSE, it is the lack of coordination on shipping and receiving of samples, which is not done accordingly to the calendar of the laboratory and the operative area, because in a short period of time the expected/projected number of samples is exceeded, resulting in delays in accomplishment of the tests and the disposition in excess of material and human resources.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> At this moment, the techniques are being standardized, Immunohistochemistry at the CENASA and the western blot (immunmotransferency) at the CPA, which will allow us to have more tools for the diagnose in Mexico; in addition, the WB allow us to count on another technique of the higher sensitivity and specificity, that guarantees optimal result in less time (approximately 8 hours).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> It must be mentioned that, we have had contemplated the formation of a network of laboratories of diagnosis of TSE´s to specialized on the HP technique, where we will have 6 regional laboratories and 4 universities involved, this will in the future allow the processing and diagnosis of the sample from its place of origin and only its confirmation by other techniques at central level. For this, we already count with the procedure for the authorization and verification of a laboratory of histopathology for the diagnosis of the BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Monetary Compensation to cattle dealers: Because the BSE is considered as an exotic disease, a contingency fund that could be put to work in case the disease appears, does not exist at this time.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the case of the contingency funds, the national campaign for diseases relies on a section on this subject. Nevertheless, for the exotic diseases official norms do not exist and article 36 of the Federal Law of Animal Health only establishes that will be due to create, but it does not explain the mechanism to be considered for its creation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The pre-established form to compensate the possible producers that are themselves affected by the presence of BSE in their cattle, it will be from the federal budget that is agreed upon the program Alliance for the Country for the corresponding fiscal year.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In this sense, it is necessary to pinpoint that the minimum amount to consider for this budget will be a 4% of the total assigned to the Fito-zoo-sanitary Contingencies Plan on behalf of the Federal Government.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> This will have a distribution by federal entity, which a specific amount will be able to be assigned to joint, if necessary, to the DINESA against the BSE. Also, the State Governments will proportionally contribute an equal amount to the federal to be incorporated to the compensation funds of the Device of Emergency. As for the cattlemen, they will have to come-up with resources equivalent to the third part of the total amount assigned by the Federal and State governments.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Animal identification and traceability of cattle products.- Different elements were considered with which Mexico counts on to carry out the traceability of animals and its products upon a sanitary problem, including the animal identification and the organizations related to this activity.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Actually, the identification system of the cattle in Mexico is organized in two forms, one State-ID with aims of demonstration of property and control of cattle rustling and another Federal-ID, with aims of identification for the development of the zoo-sanitary campaigns against the bovine tuberculosis and brucellosis, first it is based on the registry and recognition of the Hot-Brands of each producer, and the second in addition to the previous system, one is based on a metallic earring of blue color with a number of identification, which is described in the NOM-031-ZOO-1995, Campaña Nacional Contra la Tuberculosis Bovina (Mycobacterium bovis), National Campaign Against Bovine Tuberculosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This procedure assigns a number to an animal, which is used during zoo-sanitary surveillance campaigns, these activities are registered along with an identification number, in a document called test-opinion, in which it is written down, in addition to the test results applied to the animals, the identification and data of the cattle herd and ranch of origin of the animal for its later traceability. This test-opinion is along with the certificate of “Herd free of bovine tuberculosis” as described in the same Mexican Official Norm.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">According to the procedure previously described, in Mexico, there were around 3.291 registered herds with 282.932 heads of bovines identified in 2003, that represents 0,94% of the total population in this country. Nevertheless, in the same NOM, it’s expressed in point 11 referring to mobilization that the animals coming from disease-free herds, they will be able to be mobilized to any destination within the national territory with no need to be tested for tuberculosis before its mobilization, if the following requirements are met: obtain a zoo-sanitary certificate, and for the zoo-sanitary certificate to be issued, certification that they come from a disease-free herd and that the animals must have a disease-free herd identification.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Considering that in order to mobilize the animals it is necessary to have a valid disease-free zoo-sanitary certificate, we can estimate that there are more than 3,431,022 identified animals, according to the information obtained from the Statistical Report of the Cattle Mobilization of FY2000, with information captured up to the 24 of August of 2001 by the National Organism of Herd Certification, A.C., that represents the 11,4% of the bovine total population on which we can observe that more than 50% of these mobilizations are directed to slaughterhouses, 17% to feedlots, 15% for export and 11% for pasturing.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Based on the above, experience of a suitable animal traceability is shown specially in the case of the animals destined to be exported, where the USDA when finding a positive animal reactor to the tests of tuberculosis in the United States, it has been possible to trace it back to its the original herd; on the other hand, the identification system used on dairy cattle, which counts on a homogenous system of identification for production and genetic improvement control, nevertheless, this mechanism although is available for the federal government, it would make use of, only in the presence of a serious epidemiology event.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Educational Programs, Awareness and Training.- The CPA, one of its activities, is to maintain a permanent program of training courses on exotic diseases of the animals, on a national context. In 1994, BSE awareness programs were incorporated , with the diffusion of information, talks and courses on the following areas: disease history, economic consequences, etiology, transmission mechanisms, clinical signage, histopathology injuries, differential diagnosis, measures of prevention and activities of epidemiologist surveillance, supported by audio-visual means, these programs are taken to a diverse audience, including the students of the last semesters of Veterinary Medicine, to the personnel that conforms the Quarantine National System, as well as Veterinary Doctors, government, private and to other specialists.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> ESTIMATION OF RISK (Risk Assessment)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> According to the qualitative estimation in this assessment, it was determined that the risk of occurrence of the disease in the bovine population, is low.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The quantitative estimation index was located at 5.268908E 08 of the risk of disease exposure of the national herd, number that represents numerically like a low probability of occurrence of the problem in Mexico.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> CONCLUSIONS AND RECOMMENDATIONS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The BSE is a disease that was described for the first time in 1986, nevertheless, today, epidemiologists have many unanswered questions on how is transmitted.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The introduction of the BSE in Mexico would cause a serious socioeconomic impact, commercial, political and probably of public health concern, because the presence of the disease would restrict sanitarily and commercially, disrupting the actual distribution of meat products at national level and to other countries, independently of the impact in the consumption of the inhabitants with respect to the beef consumption and products of bovine origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Considering the way of transmission, in case of a breakout, the native animals that are at greater risk of being infected in Mexico, those are the dairy cattle in specialized systems and the bovines at feedlots in the arid and tropical regions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In Mexico, we got Laws, Mexican Official Norms and Agreements, that cover relevant aspects of the epidemiology surveillance of the BSE, same that must be fortified in its operative phase, mainly in its application and enforcement.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The Mexican Official Norm NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, prohibits the import of cattle products, however, fresh beef has been imported, chilled, frozen and beef preparations, as long as it comes from animals smaller of thirty months of age, which diminishes the risk but does not exclude it.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The evaluation showed that the four great areas of concern are assigned to the Main Directorate of Animal Health (DGSA) and to the Main Directorate of Fito-zoo-sanitary Inspection (DGIF); responsibility of the Veterinary Services in Mexico, in relation to the BSE are: epidemiology surveillance, animal movement control, zoo-sanitary campaigns and emergencies; functionality and capability of communicating among them was evaluated as we as the capacity of response before a sanitary emergency caused by the BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> It is necessary to increase and to better coordination of the surveillance activities, particularity between the areas of diagnoses and operational, for the correct execution of the surveillance activities in the field. The imported bovines (1996-2003) have been slaughtered and those destined to improve genetics, once they conclude their productive life and are discarded, will also be slaughtered.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The actions of detection of downer-cows need to be reinforced for its processing at TIF plants, till now a deficient activity, where the majority of the animals with such clinical characteristics, regularly are not taken this plants but rather are slaughtered at same ranch/location and consumed regionally or they are taken to slaughterhouses without supervision and sanitary inspection.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Ante mortem inspections need to be reinforced at Federal Inspection Type Plants, municipal and private slaughterhouses, mainly in these two last ones, with the purpose of detecting bovines clinically affected by BSE. There is commercial interest to incorporate flours of meat and bone of ruminants in the rations destined to the feeding of the bovines, like an alternative source of protein matter, reason why official mechanisms must be reinforced in preventing this type of illegal practices.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> One of the tools in preventing the BSE is to avoid the exposure of the native bovines to the consumption of presumably contaminated feed with the pathological agent or unless is processed by means of a thermal process that guarantees its inactivation. However, the heat treatment that the flours of meat and bone are put under in </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">XXXXXX XXXXX XXXXX XXXXXX XXXXXX XXXXXX XXXXXX XXXXX XXXXX XXXX XXXXX XXXXXX XXXXX XXXXX XXXXXX XXXXX XXXXX XXXX </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">during 20 minutes), even though this one, it does not guarantee the destruction of the prion either, but it reduces its infectivity significantly.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The Country counts on regulations, in respect to the transformation of offal (NOM-061-ZOO-1999), there still are deficiencies as to the number and qualification of the personnel responsible in supervising their fulfillment through inspection and verification.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Deficiencies in the availability of technical information at official and private levels were detected, crucial information necessary for the elaboration of the present assessment, such as the case of the information on product imports and on rendering plants, were not available for the study.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Blood was not considered as a potential source of transmission of the BSE, by-product in form of flour (dry blood), that is also produced by the rendering plants and is used in animals feeds.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The BSE epidemiology surveillance program in Mexico must be reinforced by focusing on a target animal study (bovine suspected of BSE and with suggestive clinical signs of the disease). On the other hand, as a result from this study, we found that a percentage of the obtained samples for BSE testing have been inadequately collected and among other causes were: absence of cerebral stem, incomplete cerebral stem, over-manipulated samples, advanced changes (decompose) postmortem, not enough tissue to work on, low concentration of conservative (solution) or samples taken from inadequate age of animal (too young); showing all of these, a necessity to review these procedures.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> It was also detected the fact that, as a routine practice the samples sent for the diagnosis of bovine rabies, whenever they come out positive to this disease, they are no longer processed for the BSE testing, discarding with this, the possibility of finding both diseases in a same animal, rabies virus and the BSE prion. It is also concluded that with the loss of diagnosis material, it prevented us from obtaining valuable epidemiology information useful in restructuring our surveillance program.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The identification of the cattle, as well as the traceability of its products and by-products, presents serious deficiencies at national level, which is important in case the BSE is detected in the Country, given its importance like a primordial component to trace, to prevent and to eradicate this and other animal diseases, turning out to be an additional vital tool to determine the dissemination degree in case of break-out in the country, that would immediately allow us to be able to establish its origin (native or imported) and to take the appropriate counter-epidemic measures.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> From 1994, the Commission Mexico - United States for the Prevention of Foot-and-Mouth Disease (FMD) and other Exotic Diseases (CPA), it has carried out activities of awareness and training on BSE, however, this has been centered to certain zones of the country, leaving some other zones, particularly the rural zones without cover, same that can provide with valuable epidemiology information and some cases for diagnosis of neuropathy in ruminants.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> According to the analysis made on the risk assessment in its qualitative modality, it is considered like low-risk, the risk of introduction of the BSE to the national herd, whereas the quantitative study locates it in values of 5.268908E-08.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Recommendations:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> We ought:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> to reinforce the inspection and supervision activities by the sanitary authority of the SAGARPA over all of those involved in the cattle production chain, in respect to the fulfillment and application of the established technical regulations expressed on the official norms on the monitoring of BSE, specially the NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding and the NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products for animal consumption; to increase the number of inspectors (Vet Doctors) as much as governmental as private, with a vision of having a better supervision of the rendering plants and feed factories. It is recommendable that such inspectors have a veterinary doctor’s degree. to reinforce the active epidemiology surveillance subsystem, having special attention to aim at target animals and the size of the statistical test, as well as its stratification at national level; to review, to update and to homologate the criteria and definitions of the Mexican official norms related to the monitoring of the BSE and the requirements of import, according to norms NOM-008, NOM-030 and NOM-060; to provide technical and legal elements in the official norms, that may allow to optimize the use of financial and human resources (federal, state and private), with the purpose of that the material and human infrastructure, the installed diagnoses and the potential, can be used with greater efficiency, in the prevention activities, diagnosis and surveillance of the BSE in Mexico; to homologate the mechanism of training in the obtaining of the samples for the BSE, using the technique of the teaspoon, by means of a national program; to have a certified pathologist for the high security laboratory of the CPA, because this situation of not having one, causes the delay in the processing of samples, as well as the loss of economic resources by requiring the support of the CENASA; to plan the taking of samples at a national level and to coordinate its shipment to the CPA for its processing in the laboratory of high security or its re-expedition to the CENASA, with the purpose of optimizing the diagnosis; to obtain funds and allocate them at each state, in order to compensate cattle dealers affected by the animal culling at risk by BSE, in case of BSE showing up in Mexico, the same or similar mechanism are to established for the handling of monetary compensation, like the one used on the Alliance for the Country or to extend the already existing state government faculties, by means of an exclusive and specific account for the implementation of BSE comp payment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> to implement a national animal identification and traceability system, its products and by-products, that it may allow us to apply prevention measures and control of diseases, as it would be the case of the BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 6, paragraph 1): Gallinaza and pollinaza- feather meal (hay bed or substrate on which birds grow up, constituted by rice husk, straw or another type of hay, agriculturist, that at the end of the raising cycle of young hens or chicken, contains the feces of the animals that were bred on it, as well as rest of non-consumed food by the birds), it has been considered in multiple occasions, like an element of potential risk in the transmission of the bovine spongiform encephalopathy (BSE), when it is used to feed ruminants. The risk is generated, as it is common, the bird feed, contains flours of meat and bone of ruminant like source of protein. In this way, in theory, if some of the bovines with which the meat and bone flour was prepared as bird feed were infected with the BSE prion and given the high resistance of the agent (prion) to high temperatures, in the industrial process as the making of the flour, like the making of the nutritional concentrated feed for birds, and even the passage by digestive-tract of the bird, it would not guarantee the destruction of the BSE prion, reason why the possibility would exist, when gallinaza or pollinaza is used in the feeding of ruminants, this could infect susceptible ruminants.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 6, paragraph 2): As much gallinaza as pollinaza, they can contain up to a 3% of wasted food, independently of bird feces that could also contain the prion, all implying that the flours of meat and bone of bovine origin, can be consumed by other bovines and by doing this, constituting a possible situation of BSE risk.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Norma NOM-060-ZOO-1999 Zoo-sanitary specifications for the transformation of animal’s offal and its use in animal feeding and the NOM-061-ZOO-1999 Zoo-sanitary Specifications of nutritional products for animal consumption, they clearly indicate the prohibition to feed ruminants with flours of meat and bone of ruminant origin, however, the prohibition to feed ruminants with gallinaza or pollinaza, is not contemplated in these norms. With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 6, paragraph 3): Other elements to consider are the production cycles of the farms of birds in Mexico, a common practice is that when a cycle is reached (ended) “all inside, all outside”, and the pollinaza and gallinaza are destined to feed the cattle. Depending on the type and the characteristics of the bed, it is possible to calculate an approximated weight of 13,9 kg. by square meter of bird farm surface.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 6, paragraph 4): In the Mexican market, two types of products are accepted: pollinaza and gallinaza, which has been consolidated as a production system, considering that near 90% of the feces are used as ruminant’s feed, with prices reaching near those of cereal grains, the rest is used in agriculture.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 6, paragraph 5): The use of the animal feces like source of high nutrients supply, it obeys mainly to its high content of mineral matter and non-protein nitrogen. In general, nitrogen is concentrated in greater amount in bird feces. What is doubtless, it is that the feces are raw material available all the year long for animal feeding, especially bovines. The FAO (1980) made a description of the physical composition of pollinaza as it is detailed next: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Feces 62%</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Bed 31%</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Wasted Feed 3%</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Feathers 2%</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Unknown ingredients related to fresh matter 2%</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Source: The FAO. Feed from Wastes Animal: State of knowledge, Production animal and Health, to paper 18. Rome, Italy 1980.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusion:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Making public the information that has been eliminated of the report, it would open the door for those in the grain business to use it for their benefit and by pressing the government/the authority to establish a NOM banning such products as ruminant feed. This would bring/cause an important alteration in the commercialization of these products nationwide, which in turn would remarkably increase the production costs of the cattle in feed lots. Today, we foresee escalating grains prices at medium term, originated by its use in the ethanol production; this would aggravate the situation and force a NOM as described before, which in addition, if our sanitary status with respect to the BSE is considered low, it would be obviously excessive cost and highly harmful for the producer of birds and cattle. It is why, that it was decided to block the reference information.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked: Justification of the blockade (p. 12, paragraph 3): During the period between 1996 to 2003 years in which, considering the long period of incubation of the BSE, the disease was already present in the United States of America and Canada, Mexico as usual, imported considerable amounts of calves destined for dairy production. In the same term “bullfight” bulls from Spain were imported once Europe reached a free status from FMD, same that allowed the import of some cattle for reproduction from other European countries, with exception of the United Kingdom and Ireland, countries in which BSE already existed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> In all the cases these imports were immediately stopped even before the confirmation of BSE in those countries, nevertheless, as already indicated, the ample period of incubation of the disease, those imports are looked as of certain risk, even though in that moment they were not.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The nonexistence of a national animal identification and traceability system at that time made it impossible to establish the destiny of most of those animals and to even know if they have been eliminated at the end of its productive life. It is possible to indicate that the recent imports of heifers coming from the United States of America and in the near future from Canada, new requirements and actions that guarantee their traceability and other measures to mitigate the BSE risk, are in place.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Even though during the administration of the Lic. Vicente Fox, the SAGARPA made a concerted effort to establish the National System of Individual Cattle Identification (SINIIGA), the magnitude, cost and coverage of the project, its conclusion in the short and medium term are way far distant, what implies that it will be long time before Mexico can count on a suitable (working) national system of identification and traceability of animals and products of origin animal.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The blockade of the above paragraph obeys to the convenience of not exposing to the Federal Government to unnecessarily critics that even though funded, it would not contribute to the solution of a problem that, although is of urgent attention, by its magnitude and cost, it exceeds in much, the present capacities as much of the Government, like of the National Cattlemen Sector. The critic would sustain in that what it is said in such paragraph is purely speculative, without possibility of corroborating it documentarily.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> END...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Hola Amigo Terry,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Finally, here is a translation - if you can call it that - i'm not happy with it but guess that some paragraphs are very literally translated (poorest job i have ever done translating a document), please read it and if something is not clear enough or not right just let me know it and i'll correct it...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> If you don't find anything of importance; if it is to vague and shows that they have done nothing about it; if somehow it gives you the impresion that they don't know a thing and are trying to cover their butts in a very stupid way;...yea! you got the right impression!!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> All they are saying it's a "mea culpa" and we ought to do this and that; we don't know how they came in or where they are; we are looking into it; we screwed up all the BSE testing and we don't know how to do it right; it is too costly and we don't have the money; we didn't do it, past administration did it; we are trying to fix it; etc., All of the above and more, but we are following OIE rules, we have NO BSE anywhere and risk is extremely low or null, but CATTLEMEN WIL BE COMPENSATED!!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Conclusion- they are a bunch of murderers and me a national security threat for having them to admit it!! .....Oh my Lord!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> snip...end (tss)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Have a wonderful weekend and our best regards,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> xxxxxxxxxxxxxxxxxxxxxxx</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> ====================</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">EEB BSE MAD COW IN MEXICO ???</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.gob.mx/cms/uploads/attachment/file/870613/e-scad-report-feb-2023-2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gob.mx/cms/uploads/attachment/file/870613/e-scad-report-feb-2023-2.pdf</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://bse-atypical.blogspot.com/2011/03/mad-cow-disease-bse-prion-mexico.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://bse-atypical.blogspot.com/2011/03/mad-cow-disease-bse-prion-mexico.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.gob.mx/senasica/documentos/encefalopatia-espongiforme-bovina?state=published" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gob.mx/senasica/documentos/encefalopatia-espongiforme-bovina?state=published</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.gob.mx/busqueda#gsc.tab=0&gsc.sort=&gsc.q=Encefalopat%C3%ADa%20espongiforme" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.gob.mx/busqueda#gsc.tab=0&gsc.sort=&gsc.q=Encefalopat%C3%ADa%20espongiforme</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">CHRONIC WASTING DISEASE CWD TSE PRION IN MEXICO</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Note: Effective September 2023 Mexico has placed a ban on all shipments of cervids from the entire United States due to Chronic Wasting Disease (CWD).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Captive Wild Ruminants - Health Certificate — Available Upon Request Only Note: Includes all camelids, excludes bovines and wild cervids</div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/export/international-standard-setting-activities-oie/sa_by_country/sa_m/ct_animal_mexico" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/export/international-standard-setting-activities-oie/sa_by_country/sa_m/ct_animal_mexico</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">CERVID CWD SHIPPED TO MEXICO FROM USA???</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><span style="outline: none !important;">''TPWD records indicate that within the last five years, the seven CWD-positive facilities transferred a total of 2,530 deer to 270 locations in 102 counties and eight locations in Mexico (the destinations included 139 deer breeding facilities, 118 release sites, five Deer Management Permit sites, and three nursing facilities).''</span><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Special Work Session Chronic Wasting Disease -Disease Detection and Response Request Permission to Publish Proposed Rules in the Texas Register October 5, 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I. Executive Summary: Staff seek authorization to publish proposed amendments to the comprehensive Chronic Wasting Disease (CWD) rules in the Texas Register for public comment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">II. Discussion: CWD, a fatal neurodegenerative disorder that affects cervid species such as white-tailed deer, mule deer, elk, red deer, sika, and others (which will be referred to as susceptible species), has been detected in seven permitted deer breeding facilities since March 23, 2021. Texas Parks and Wildlife Department (TPWD), along with Texas Animal Health Commission (TAHC), has been engaged in an ongoing battle with CWD in Texas since 2002. Regarding the current situation involving CWD in permitted deer breeding facilities, TPWD records indicate that within the last five years, the seven CWD-positive facilities transferred a total of 2,530 deer to 270 locations in 102 counties and eight locations in Mexico (the destinations included 139 deer breeding facilities, 118 release sites, five Deer Management Permit sites, and three nursing facilities). Emergency rules were filed on June 22, 2021, intended to address significant concerns for CWD being transferred from deer breeding facilities where the disease may exist undetected, but those rules expire after 180 days, and amendments to the comprehensive rules are necessary to mitigate risks not currently addressed in the current rules.</div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://tpwd.texas.gov/business/feedback/meetings/2022/0915/agenda/item.phtml?item=7" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/business/feedback/meetings/2022/0915/agenda/item.phtml?item=7</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Texas CWD Surveillance Positives</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Counties where CWD Exposed Deer were Released</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Number of CWD Exposed Deer Released by County</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic Wasting Disease CWD Captive Herds updated April 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic Wasting Disease CWD Captive Herds updated April 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic Wasting Disease CWD TSE PrP in Texas</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://storymaps.arcgis.com/stories/b93f528938ac48e9b56dcc79953cbec0" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://storymaps.arcgis.com/stories/b93f528938ac48e9b56dcc79953cbec0</a></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Características clínicas de la enfermedad de Creutzfledt-Jakob en México: un análisis retrospectivo</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">José A. Choreño-Parra1 2 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Francisco J. Pacheco-Sánchez1 3 4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alberto I. Rodríguez-Nava1 3 4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Gabriela García-Quintero1 3 4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Patricia E. Rodríguez-Muñoz1 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Parménides Guadarrama-Ortiz1 * </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Department of Neurosurgery, Centro Especializado en Neurocirugía y Neurociencias México (CENNM). Mexico City, Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2Escuela Nacional de Ciencias Biológicas, Instituto Politécnico Nacional. Mexico City, Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3Internado Médico de Pregrado, CENNM. Mexico City, Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4Escuela Nacional de Medicina y Homeopatía, Instituto Politécnico Nacional. Mexico City, Mexico</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ABSTRACT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Little is known about the clinical characteristics and significance of Creutzfeldt-Jakob disease (CJD) in Mexico.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Objective:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This study aimed to conduct a retrospective revision and analysis of the clinical cases of Mexican patients with CJD available in the literature.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We systematically searched electronic databases for studies in English and Spanish conducted in Mexico over the period of 1990-2020 that involved Mexican patients with any of the clinical forms of CJD. Clinical variables were extracted from the selected studies that met eligibility criteria. Descriptive statistics were used to characterize the study population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A total of seven studies were included in the analysis. From these, 29 cases were revised, and their clinical characteristics analyzed. The median age at the time of diagnosis was 54 years (range 23-75 years). CJD was more frequent among females than male patients (male:female ratio 1:1.41). Most patients resided in Mexico City and the State of Mexico, and 93% attended public hospitals. The most frequent form of CJD was sporadic, with only two probable cases of familiar disease. The most common clinical symptoms observed in order or frequency were rapidly progressive dementia (68.9%), cerebellar signs (51.7%), neuropsychiatric symptoms (51.7%), akinetic mutism (51.7%), myoclonus (44,8%), extrapyramidal signs (44.8%), visual disturbances (41.3%), pyramidal signs (31%), and sleep disorders (17.2%). Only 20% of the cases were confirmed by histopathological analysis of brain biopsy or autopsy specimens.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study provides an overview of the main clinical characteristics of CJD in Mexican patients.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Key words Creutzfeldt-Jakob disease; Prion; Cognitive decline; Rapidly progressive dementia; Spongiform encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DISCUSSION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The knowledge about the incidence, clinical characteristics, and epidemiological significance of CJD in Mexico is limited. Our study aimed to retrospectively revise and analyze available reports about the main manifestations of CJD in Mexican patients. A striking finding of our analysis was the low amount of cases from Mexico formally described in the literature. Indeed, only 29 patients with CJD have been reported over the past three decades in our country, which is <1 case/year. This finding undoubtedly reflects a high grade of underreporting and sub-diagnosis of CJD cases in Mexicans. For instance, if we took the global incidence of sCJD as a reference (1 case per million people per year)3, then the expected number of CJD cases occurring in Mexico would be much higher.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These data are in part due to the rarity and unspecific manifestations of CJD. In this sense, it is well recognized that rapidly progressive dementia is not a unique characteristic of this disease. In fact, many other neurological disorders can be confused with CJD, including some variants of Alzheimer disease (AD), dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), viral, bacterial, parasitic, or autoimmune meningoencephalitis (e.g., Hashimotos encephalitis, and limbic encephalitis), corticobasal degeneration, progressive supranuclear palsy, paraneoplastic encephalomyelitis, and even vascular dementia18,19. Furthermore, a wide range of other clinical manifestations, such as pyramidal/extrapyramidal dysfunction, ataxia, cerebellar signs, psychiatric symptoms, visual disturbances, sleep disorders, akinetic mutism, and persistent painful sensory symptoms, may be present among patients with CJD1, adding complexity to the clinical spectrum of the disease. This fact led physicians to subclassify various forms of CJD according to the mean symptoms, including the classic (dyskinetic), Heidenhain (visual), myoclonic, cerebellar (ataxic), thalamic, amyotrophic, and panencephalopathic forms4,7.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Furthermore, it is, currently, well known that, in the specific case of sCJD, the clinical heterogeneity is also associated with some genetic and molecular features of the pathogenic PrPSc. Specifically, methionine (M) or valine (V) polymorphism at codon 129 of the PRNP gene20, as well as the type of electrophoretic mobility pattern of PrPSc after protease digestion (type 1 and 2), are used to classify patients into several phenotypes separated into three categories: sCJD cognitive subtypes (MM1, MV1, MM2, and VV1), ataxic subtypes (VV2 and MV2), and other non-sCJD subtypes (types 3 and 4 electrophoretic mobility for sporadic fatal insomnia (sFI) and variably protease-sensitive prionopathy (VPSPr), respectively)4,7. Each category has unique characteristics differing from the others in age at onset, duration of the disease, dominant neurological findings, and among others. However, the age at onset of all CJD cases ranges from 50 to 70 years with no predilection for any gender.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The polymorphisms in the PRNP gene that determine the susceptibility for the development of sCJD are differentially distributed among various populations around the world21-24. However, the frequency of M129V genotypes and alleles in the Mexican population has not been addressed. Our results confirm that sCJD is the most frequent variant of this disease in Mexicans. Furthermore, there are only two cases of probable fCJD not confirmed by the demonstration of an inherited mutation in the PRNP gene. There is no registry of any case of acquired CJD through iatrogenic exposure or contagion, but there are cases in very young patients, and the wide range of interval between disease onset to hospital admission opens the possibility of the occurrence of vCJD/iCJD among Mexican individuals. We also observed that rapidly progressive dementia, akinetic mutism, and myoclonus were among the most common symptoms observed in Mexican CJD patients. Furthermore, neuropsychiatric symptoms were frequently reported, although only one case was formally categorized as a Heidenhain variant of CJD15. These data indicate that the sCJD cognitive subtypes are common in Mexicans with this disease. Thus, we can predict that the underlying genetic and molecular traits of these phenotypes (MM1, MV1, MM2, and VV1) would be frequent in our population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Interestingly, we found a high number of patients with CJD that presented cerebellar symptoms (51.72%), including ataxia. The frequency of cerebellar affection in CJD patients from other regions has not been comprehensively estimated. In a study conducted in the United Kingdom, cerebellar ataxia occurred as the only clinical manifestation in 5% of patients with CJD25. In contrast, in a study of Chinese patients with CJD, up to 51.9% of affected individuals presented cerebellar ataxia26, which coincides with our findings. Thus, based on our results, we also predict that the genetic and molecular characteristics of the PrPSc underlying ataxic subtypes of sCJD (VV2 and MV2) may have a high incidence in Mexico.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The variable clinical characteristics of CJD complicate the diagnostic approach and opportune detection of positive cases. Furthermore, the low level of clinical suspicion among physicians and the absence of a formal surveillance strategy may further contribute to the underestimation of the burden of CJD in the Mexican population. To improve the diagnosis and global surveillance of CJD, the CDCs have established several diagnostic criteria that classify each case according to the likelihood of the disease based on clinical features and the results of different laboratory and imaging tests9. Possible and probable categories are based on clinical symptoms and positive results in EEG, MRI, and CSF 14-3-3. Most such studies have high specificity but low sensitivity, and it is important to mention that their diagnostic reliability varies according to the CJD form and even to the sCJD subtype27.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EEG recording in patients with sCJD typically shows pseudo-periodic sharp-wave complexes (PSWC) with diffuse slowly background activity at the middle and late stages of the disease. The diagnostic value of EEG is due to its 64% sensitivity and 91% specificity28. On the other hand, MRI has shown to have better diagnostic performance due to improvements in the DWI sequence29. MRI does not allow to distinguish between clinical forms of CJD, but in cases of vCJD, a posterior thalamus involvement (pulvinar sign) supports the diagnosis30. CSF levels of neuron-specific enolase (NSE) and T-tau protein have also been proposed as biomarkers of CJD. From these, T-tau protein has the highest sensitivity and specificity with a cutoff of 1150pg/mL31. However, CSF levels of T-tau protein have not been integrated into the 2018 CDCs diagnostic criteria for CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Notably, in the last decade, RT-QuIC has emerged as a novel alternative for premortem diagnosis of CJD with better performance compared with other CSF tests. This assay relies on the in vitro template conversion of recombinant PrPC into PrPSc, evidenced through a fluorescent indicator, allowing to detect minute amounts of PrPSc in biological samples with high sensitivity and specificity17. Indeed, we recently reported the first case of a Mexican patient with sCJD confirmed by a complete battery of diagnostic tests, including EEG, MRI-DWI, CSF levels of T-tau, and 14-3-3 protein, as well as CSF RT-QuIC, which allowed us to detect this case premortem (16). In such a report, we demonstrated an excellent correlation between the results of the RT-QuIC test and other clinical and laboratory parameters. Despite this, RT-QuIC is not considered as a diagnostic tool that can classify a patient as a definitive case of CJD by the CDCs. Thus, the definitive diagnosis of CJD is still based on the histopathological analysis of brain biopsy and autopsy specimens.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study reveals concerning data about the diagnostic approach to CJD in our country. First, we observed that only 20% of cases were confirmed by histopathology. The definitive confirmation of CJD in Mexicans was exclusively performed in public health-care institutions. More specific and sophisticated imaging and CSF tests were rarely performed in public hospital care, and they were carried out in laboratories outside Mexico, even in cases diagnosed at private hospitals. These facts must claim the attention of national health-care authorities to make the efforts needed to create laboratories with the technical capacity to perform special CSF studies and to introduce new tests that allow confirming CJD in Mexico, such as the RT-QuIC test.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONCLUSIONS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study provides an overview of the main clinical characteristics of CJD in Mexican patients. Also, our study reveals that the incidence of CJD in our country could be higher than supposed. Finally, we remarked several deficiencies in the diagnostic approach to this neurological disorder in our country that needs the attention of the Mexican authorities of health. Our findings should motivate Mexican physicians and researchers to get involved in the surveillance and improvement of diagnosis of the disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1665-50442020000600228" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S1665-50442020000600228</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;">MEXICO CREUTZFELDT JAKOB DISEASE CJD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://creutzfeldt-jakob-disease.blogspot.com/2012/06/mexico-is-under-or-mis-diagnosing.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://creutzfeldt-jakob-disease.blogspot.com/2012/06/mexico-is-under-or-mis-diagnosing.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">THURSDAY, NOVEMBER 9, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div></div><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2023.EN-8386</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Professor John Collinge on tackling prion diseases, sCJD accounts for around 1 in 5000 deaths worldwide</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, SEPTEMBER 11, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Professor John Collinge on tackling prion diseases “The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.” </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is accumulating evidence also for iatrogenic AD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="https://prpsc.proboards.com/attachment/download/5" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/attachment/download/5</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">Terry S. Singeltary Sr.</div><br style="outline: none !important;" /></div><br style="outline: none !important;" /></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-8845262050241121312023-11-22T11:53:00.002-06:002023-11-22T13:31:14.215-06:00Creutzfeldt-Jakob Disease ‘CJD Awareness Day’ November 12, 2023<p><span style="background-color: white; font-family: arial; font-size: 16px;">Creutzfeldt-Jakob Disease ‘CJD Awareness Day’ </span><span style="font-family: arial; font-size: 16px; outline: none;">November 12, 2023</span></p><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">My name is Terry S. Singeltary Sr., I lost my Mom to the Heidenhain Variant Creutzfeldt Jakob disease hvCJD, confirmed, way back on December 14, 1997. </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">I just want to thank you all, for helping to bring the National CJD Awareness Day official. MANY THANKS!</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">I have wasted 25 years, every day, trying to bring awareness to this damn disease, but it seems i have failed. </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">i would like to bring to your attention, the dire situation that we find ourselves in with the human and animal TSE Prion disease, and just how much it has spread, and the science showing that sporadic CJDs, may not be spontaneous at all. i hope you find this information helpful...</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">with kindest regards, terry</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">Terry S. Singeltary Sr., flounder9@verizon.net</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;">Chronic Wasting Disease CWD TSE Prion Environmental and Zoonosis Risk Factors</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Price of TSE Prion Poker goes up substantially, all you cattle ranchers and such, better pay close attention here...terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Strain characterization of chronic wasting disease in bovine-PrP transgenic mice </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Nuria Jerez-Garrido1, Sara Canoyra1, Natalia Fernández-Borges1, Alba Marín Moreno1, Sylvie L. Benestad2, Olivier Andreoletti3, Gordon Mitchell4, Aru Balachandran4, Juan María Torres1 and Juan Carlos Espinosa1. 1 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Madrid, Spain. 2 Norwegian Veterinary Institute, Ås, Norway. 3 UMR Institut National de la Recherche Agronomique (INRA)/École Nationale Vétérinaire de Toulouse (ENVT), Interactions Hôtes Agents Pathogènes, Toulouse, France. 4 Canadian Food Inspection Agency, Ottawa, Canada. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Chronic wasting disease (CWD) is an infectious prion disease that affects cervids. Various CWD prion strains have been identified in different cervid species from North America and Europe. The properties of the infectious prion strains are influenced by amino acid changes and polymorphisms in the PrP sequences of different cervid species. This study, aimed to assess the ability of a panel of CWD prion isolates from diverse cervid species from North America and Europe to infect bovine species, as well as to investigate the properties of the prion strains following the adaptation to the bovine-PrP context. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: BoPrP-Tg110 mice overexpressing the bovine-PrP sequence were inoculated by intracranial route with a panel of CWD prion isolates from both North America (two white-tailed deer and two elk) and Europe (one reindeer, one moose and one red deer). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Our results show distinct behaviours in the transmission of the CWD isolates to the BoPrP-Tg110 mouse model. Some of these isolates did not transmit even after the second passage. Those able to transmit displayed differences in terms of attack rate, survival times, biochemical properties of brain PrPres, and histopathology. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: MCIN/AEI /10.13039/501100011033 and by European Union NextGeneration EU/PRTR </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: PCI2020-120680-2 ICRAD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Interpretive Summary:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">cwd scrapie pigs oral routes </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CONFIDENTIAL</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">LINE TO TAKE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Monday, November 13, 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) Singeltary Another Request for Update 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://fdabse589.blogspot.com/2023/11/food-and-drug-administrations-bse-feed.html</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none; text-align: justify;">CWD TSE PRION CERVID ENVIRONMENTAL RISK FACTORS 2023</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div></div><div style="outline: none;">"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."</div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Detection of prions in soils contaminated by multiple routes</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Stuart Siegfried Lichtenberg1,2 , Heather Inzalaco3 , Sam Thomas4 , Dan Storm5 , Dan Walsh6</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1Department of Veterinary and Biomedical Sciences, University of Minnesota, St. Paul, Minnesota, U.S.A. 2Minnesota Center for Prion Research and Outreach, University of Minnesota, St. Paul, Minnesota, U.S.A. 3 Wisconsin Cooperative Wildlife Research Unit, Department of Forest and Wildlife Ecology, University of Wisconsin-Madison, Madison, Wisconsin, U.S.A 4Department of Soil Science, University of Wisconsin-Madison, Madison, Wisconsin, U.S.A. 5Wisconsin Department of Natural Resources, Eau Claire, Wisconsin, U.S.A. 6U.S. Geological Survey, Montana Cooperative Wildlife Research Unit, University of Montana, Missoula, Montana, U.S.A.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Free-ranging animals afflicted with transmissible spongiform encephalopathies frequently shed infectious prions into the broader environment. The quintessential example is chronic wasting disease, the TSE of cervids. Over the course of the disease, an infected animal will shed infectious prions in blood, urine, saliva, and feces. Upon death, the total prion load interred in the animal’s tissues will be deposited wherever the animal falls. This contamination creates substantial risk to naïve animals, and likely contributes to disease spread. Identification and quantification of prions at contamination hotspots is essential for any attempt at mitigation of environmental transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: Surfactant extraction of soils followed by precipitation yields a sample that is amenable to analysis by real-time quaking induced conversion. However, differences in extraction yield are apparent depending on the properties of the matrix from which the prions are being extracted, principally soil clay content.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: We are able to detect prion seeding activity at multiple types of environmental hotspots, including carcass sites, contaminated captive facilities, and scrapes (i.e. urine and saliva). Differences in relative prion concentration vary depending on the nature and source of the contamination.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Wisconsin Department of Natural Resources</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">=====end</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">The detection and decontamination of chronic wasting disease prions during venison processing</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Marissa S. Milstein1,2, Marc D. Schwabenlander1,2, Sarah C. Gresch1,2, Manci Li1,2, Stuart Lichtenberg1,2, Rachel Shoemaker1,2, Gage R. Rowden1,2, Jason C. Bartz2,3 , Tiffany M. Wolf2,4, Peter A. Larsen1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Presenting author: Tiffany M. Wolf 1 Department of Veterinary and Biomedical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 2 Minnesota Center for Prion Research and Outreach, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA 3 Department of Medical Microbiology and Immunology, School of Medicine, Creighton University, Omaha, Nebraska, USA 4 Department of Veterinary Population Medicine, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota, USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: There is a growing concern that chronic wasting disease (CWD) prions in venison pose a risk to human health. CWD prions accumulate in infected deer tissues that commonly enter the human food chain through meat processing and consumption. The United States (US) Food and Drug Administration and US Department of Agriculture now formally consider CWD-positive venison unfit for human and animal consumption. Yet, the degree to which prion contamination occurs during routine venison processing is unknown. Here, we use environmental surface swab methods to: a) experimentally test meat processing equipment (i.e., stainless steel knives and polyethylene cutting boards) before and after processing CWD-positive venison and b) test the efficacy of five different disinfectant types (i.e., Dawn dish soap, Virkon-S, Briotech, 10% bleach, and 40% bleach) to determine prion decontamination efficacy.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: We used a real-time quaking-induced conversion (RT-QuIC) assay to determine CWD infection status of venison and to detect CWD prions in the swabs. We collected three swabs per surface and ran eight technical replicates on RT-QuIC.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: CWD prions were detected on all cutting boards (n= 3; replicates= 8/8, 8/8, 8/8 and knives (n= 3; replicates= 8/8, 8/8, 8/8) used in processing CWD-positive venison, but not on those used for CWD-negative venison. After processing CWD-positive venison, allowing the surfaces to dry, and washing the cutting board with Dawn dish soap, we detected CWD prions on the cutting board surface (n= 3; replicates= 8/8, 8/8, 8/8) but not on the knife (n= 3, replicates = 0/8, 0/8, 0/8). Similar patterns were observed with Briotech (cutting board: n= 3; replicates= 7/8, 1/8, 0/8; knife: n= 3; replicates = 0/8, 0/8, 0/8). We did not detect CWD prions on the knives or cutting boards after disinfecting with Virkon-S, 10% bleach, and 40% bleach.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: These preliminary results suggest that Dawn dish soap and Briotech do not reliably decontaminate CWD prions from these surfaces. Our data suggest that Virkon-S and various bleach concentrations are more effective in reducing prion contamination of meat processing surfaces; however, surface type may also influence the ability of prions to adsorb to surfaces, preventing complete decontamination. Our results will directly inform best practices to prevent the introduction of CWD prions into the human food chain during venison processing.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: Funding was provided by the Minnesota Environment and Natural Resources Trust Fund as recommended by the Legislative-Citizen Commission on Minnesota Resources (LCCMR), the Rapid Agriculture Response Fund (#95385/RR257), and the Michigan Department of Natural Resources.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Theme: Animal prion diseases</div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====end</div></div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><div style="outline: none;">Prion 2023 Abstracts</div><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div></div></div><div style="outline: none;"><div style="outline: none; text-align: justify;">***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="outline: none;"><div style="outline: none; text-align: justify;">Rapid recontamination of a farm building occurs after attempted prion removal</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">First published: 19 January 2019 https://doi.org/10.1136/vr.105054</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">snip...</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***>This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://pubmed.ncbi.nlm.nih.gov/30602491/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/30602491/</a> </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Front. Vet. Sci., 14 September 2015 | <a href="https://doi.org/10.3389/fvets.2015.00032" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.3389/fvets.2015.00032</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">172. Establishment of PrPCWD extraction and detection methods in the farm soil</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Conclusions: Our studies showed that PrPCWD persist in 0.001% CWD contaminated soil for at least 4 year and natural CWD-affected farm soil. When cervid reintroduced into CWD outbreak farm, the strict decontamination procedures of the infectious agent should be performed in the environment of CWD-affected cervid habitat.</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none; text-align: justify;"><div style="outline: none;"><div style="outline: none;">SUBJECT MATTER: Chronic Wasting Disease Carcass Disposal Dumpster Management and Biosecurity</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BACKGROUND INFORMATION:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">State and tribal wildlife agencies may identify collection points (dumpsters) within an identified chronic wasting disease (CWD) management zone for the disposal of hunter-harvested cervid carcasses to remove potentially infected carcasses off the landscape for disposal by an approved method (Gillin & Mawdsley, 2018, chap.14). However, depending on their placement and maintenance these dumpsters could potentially increase the risk of CWD transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In several different states, photographic evidence has shown dumpsters in state identified CWD management zones overflowing with deer carcasses and limbs scattered on the land nearby. This could provide an opportunity for scavengers to potentially move infected carcass material to non-infected zones or increase contamination of the ground material around the dumpster’s location.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Federal guidance does not explicitly address uniform standards for collection locations for carcasses of free-ranging cervids; however, the United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services Program Standards on CWD outlines procedures for carcass disposal, equipment sanitation, and decontamination of premises for captive cervid facilities.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">RESOLUTION:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The United States Animal Health Association urges the Association of Fish and Wildlife Agencies (AFWA), Wildlife Health Committee to further refine the AFWA Technical Report on Best Management Practices for Prevention, Surveillance, and Management of Chronic Wasting Disease; Chapter 14, Carcass Disposal to address the placement and management of chronic wasting disease carcass disposal dumpsters or other carcass collection containers.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Reference:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1. Gillin, Colin M., and Mawdsley, Jonathan R. (eds.). 2018. AFWA Technical Report on Best Management Practices for Surveillance, Management and Control of Chronic Wasting Disease. Association of Fish and Wildlife Agencies, Washington, D. C. 111 pp. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.usaha.org/upload/Resolution/2022/2022_Resolution_30_CWD_Dumpste.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.usaha.org/upload/Resolution/2022/2022_Resolution_30_CWD_Dumpste.pdf</a></div></div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none; text-align: justify;">THE tse prion aka mad cow type disease is not your normal pathogen. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">you cannot cook the TSE prion disease out of meat. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">the TSE prion agent also survives Simulated Wastewater Treatment Processes. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">you can bury it and it will not go away. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">it’s not your ordinary pathogen you can just cook it out and be done with. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent. I’m thinking tools used to dress a deer, knives with wooden handles, carcass disposal, burial only 3ft, scavengers, exposure of Cwd to soil and surrounding area, plants intake, …I could go on…Terry</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8 </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Laboratory of Central Nervous System Studies, National Institute of </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Neurological Disorders and Stroke, National Institutes of Health, </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Bethesda, MD 20892. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">PMID: <span dir="ltr" style="outline: none;">8006664</span> [PubMed - indexed for MEDLINE] </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="http://www.pnas.org/content/97/7/3418.full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.pnas.org/content/97/7/3418.full</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"></div><div style="outline: none; text-align: justify;">Detection of protease-resistant cervid prion protein in water from a CWD-endemic area </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">THURSDAY, FEBRUARY 28, 2019 </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">BSE infectivity survives burial for five years with only limited spread</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">5 or 6 years quarantine is NOT LONG ENOUGH FOR CWD TSE PRION !!!</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">QUARANTINE NEEDS TO BE 21 YEARS FOR CWD TSE PRION !</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">You can take this communication from my old files with how ever many grains of salt you wish…Terry</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">FRIDAY, APRIL 30, 2021 </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Should Property Evaluations Contain Scrapie, CWD, TSE PRION Environmental Contamination of the land?</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> Confidential!!!!</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">---end personal email early BSE days---end...tss</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">and so it seems...</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Scrapie Agent (Strain 263K) Can Transmit Disease via the Oral Route after Persistence in Soil over Years</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Published: May 9, 2007</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">snip...</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Our results showed that 263K scrapie agent can persist in soil at least over 29 months. Strikingly, not only the contaminated soil itself retained high levels of infectivity, as evidenced by oral administration to Syrian hamsters, but also feeding of aqueous soil extracts was able to induce disease in the reporter animals. We could also demonstrate that PrPSc in soil, extracted after 21 months, provides a catalytically active seed in the protein misfolding cyclic amplification (PMCA) reaction. PMCA opens therefore a perspective for considerably improving the detectability of prions in soil samples from the field.</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">snip...</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0000435" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0000435</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Dr. Paul Brown Scrapie Soil Test BSE Inquiry Document</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://web.archive.org/web/20090505211734/http://www.bseinquiry.gov.uk/files/sc/Seac07/tab03.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090505211734/http://www.bseinquiry.gov.uk/files/sc/Seac07/tab03.pdf</a></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none; text-align: justify;">Trucking CWD TSE PrP</div><div dir="ltr" style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none; text-align: justify;">Friday, December 14, 2012 <div style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><span dir="ltr" style="outline: none;">http://webarchive.nationa</span>... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20170404125557/http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20170404125557/http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none; text-align: justify;">Published: 06 September 2021<br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">***> Chronic wasting disease: a cervid prion infection looming to spillover</div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Alicia Otero, Camilo Duque Velásquez, Judd Aiken & Debbie McKenzie </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;">Veterinary Research volume 52, Article number: 115 (2021) </div><div style="outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="outline: none; text-align: justify;"><a href="https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-021-00986-y" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://veterinaryresearch.biomedcentral.com/articles/10.1186/s13567-021-00986-y</a></div><div style="font-family: Helvetica, Arial, sans-serif; outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Detection of chronic wasting disease prions in processed meats</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rebeca Benavente1 , Francisca Bravo1,2, J. Hunter Reed3 , Mitch Lockwood3 , Glenn Telling4 , Rodrigo Morales1,2 1 Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; 2 Universidad Bernardo O’Higgins. Santiago, Chile; 3 Texas Parks and Wildlife Department, Texas, USA. 4 Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: identify the presence of CWD prions in processed meats derived from elk. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: In this study, we analyzed different processed meats derived from a CWD-positive (pre-clinical) free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, seasoned chili meats, and spiced meats. The presence of CWD-prions in these samples were assessed by PMCA using deer and elk substrates. The same analyses were performed in grilled and boiled meats to evaluate the resistance of the infectious agent to these procedures. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities. This data suggests that CWD-prions are available to people even after meats are processed and cooked. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: These results suggest CWD prions are accessible to humans through meats, even after processing and cooking. Considering the fact that these samples were collected from already processed specimens, the availability of CWD prions to humans is probably underestimated. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH and USDA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: 1R01AI132695 and APP-20115 to RM </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We would like to thank TPWD personnel for providing us with valuable samples</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">"Our results show positive prion detection in all the samples analyzed using deer and elk substrates. Surprisingly, cooked meats displayed increased seeding activities."</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">end... <br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Fortuitous generation of a zoonotic cervid prion strain </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in many states or provinces of USA and Canada. Multiple in vitro conversion experiments and in vivo animal studies indicate that the CWD-to-human transmission barrier is not unbreakable. A major long-term public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: We inoculated several sCJD brain samples into cervidized transgenic mice (<span dir="ltr" style="outline: none;">Tg12</span>), which were intended as negative controls for bioassays of brain tissues from sCJD cases who had potentially been exposed to CWD. Some of the <span dir="ltr" style="outline: none;">Tg12</span> mice became infected and their brain tissues were further examined by Western blot as well as serial passages in humanized or cervidized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (<span dir="ltr" style="outline: none;">Tg12</span>) resulted in a “cervidized” CJD strain that we termed CJDElkPrP. We observed 100% transmission of the original CJDElkPrP in transgenic mice expressing human PrP. We passaged CJDElkPrP two more times in the <span dir="ltr" style="outline: none;">Tg12</span> mice. We found that such second and third passage CJDElkPrP prions retained 100% transmission rate in the humanized mice, despite that the natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively</div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">"Our data indicate that highly zoonotic cervid prion strains are not only possible but also can retain zoonotic potential after serial passages in cervids, suggesting a very significant and serious long-term risk of CWD zoonosis given that the broad and continuing spread of CWD prions will provide fertile grounds for the emergence of zoonotic CWD strains over time."<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A probable diagnostic marker for CWD infection in humans </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xu Qi, Liuting Qing, Manuel Camacho, Ignazio Cali, Qingzhong Kong. Department of Pathology, Case Western Reserve University, Cleveland, USA </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Multiple in vitro CWD-seeded human PrP conversion experiments and some animal model studies indicate that the species barrier for CWD to human transmission can be overcome, but whether CWD prion can infect humans in real life remains controversial. The very limited understanding on the likely features of CWD infection in humans and the lack of a reliable diagnostic marker for identification of acquired human CWD cases contribute to this uncertainty. We aim to stablish such a reliable diagnostic marker for CWD infections in humans should they occur. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: A couple of PrPSc-positive spleens were identified from humanized transgenic mice inoculated with either CWD or sCJDMM1. Prions in these spleens were compared by bioassays in cervidized or humanized transgenic mice. A couple of PrPSc-positive spleens from UK sCJDMM1 patients were also examined similarly as controls with no exposure to CWD. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: We have detected two prion-positive spleens in humanized transgenic mice inoculated with some CWD isolates. Such experimentally generated splenic “humanized” CWD prions (termed eHuCWDsp) appear indistinguishable from prions in the brain of sCJDMM1 patients on Western blot. We compared eHuCWDsp with prions in the spleen from humanized mice infected with sCJDMM1 (termed sCJDMM1sp) by bioassays in cervidized or humanized transgenic mice. Significantly, we found that eHuCWDsp can efficiently infect not only the humanized mice but also cervidized transgenic mice, and cervidized mice infected by eHuCWDsp produced PrPSc and brain pathology that are practically identical to those of CWD-infected cervidized mice. In contrast, sCJDMM1sp, similar to prions from sCJDMM1 patient brains, is poorly transmissible in the cervidized mice. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Our data demonstrate that high transmissibility with CWD features of splenic prions in cervidized transgenic mice is unique to acquired human CWD prions, and it may serve as a reliable marker to identify the first acquired human CWD cases. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: NIH Grant number: R01NS052319, R01NS088604, R01NS109532 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O'Rourke for providing the sCJD samples and the CWD samples used in this study, respectively.</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====end </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /><div style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Prion 2023 Experimental Oronasal Inoculation of the Chronic Wasting Disease Agent into White Tailed Deer </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author list: Sarah Zurbuchena,b , S. Jo Moorea,b , Jifeng Biana , Eric D. Cassmanna , and Justin J. Greenleea . a. Virus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, US b. Oak Ridge Institute for Science and Education (ORISE), U.S. Department of Energy, Oak Ridge, TN, United States </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: The purpose of this experiment was to determine whether white-tailed deer (WTD) are susceptible to inoculation of chronic wasting disease (CWD) via oronasal exposure. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and methods: Six male, neutered WTD were oronasally inoculated with brainstem material (10% w/v) from a CWD-positive wild-type WTD. The genotypes of five inoculated deer were Q95/G96 (wild-type). One inoculated deer was homozygous S at codon 96 (96SS). Cervidized (<span dir="ltr" style="outline: none;">Tg12</span>; M132 elk PrP) mice were inoculated with 1% w/v brainstem homogenate from either a 96GG WTD (n=10) or the 96SS WTD (n=10). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: All deer developed characteristic clinical signs of CWD including weight loss, regurgitation, and ataxia. The 96SS individual had a prolonged disease course and incubation period compared to the other deer. Western blots of the brainstem on all deer yielded similar molecular profiles. All deer had widespread lymphoid distribution of PrPCWD and neuropathologic lesions associated with transmissible spongiform encephalopathies. Both groups of mice had a 100% attack rate and developed clinical signs, including loss of body condition, ataxia, and loss of righting reflex. Mice inoculated with material from the 96SS deer had a significantly shorter incubation period than mice inoculated with material from 96GG deer (Welch two sample T-test, P<0.05). Serial dilutions of each inocula suggests that differences in incubation period were not due to a greater concentration of PrPCWD in the 96SS inoculum. Molecular profiles from western blot of brain homogenates from mice appeared similar regardless of inoculum and appear similar to those of deer used for inoculum. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: This study characterizes the lesions and clinical course of CWD in WTD inoculated in a similar manner to natural conditions. It supports previous findings that 96SS deer have a prolonged disease course. Further, it describes a first pass of inoculum from a 96SS deer in cervidized mice which shortened the incubation period. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection, analysis, decision to publish, or preparation of the manuscript. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgement: We thank Ami Frank and Kevin Hassall for their technical contributions to this project.</div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">=====end </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">PRION 2023 CONTINUED; </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a></div></div></div></div></div></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;">''Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">31 TAC §§65.82, 65.85, 65.88</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. Our results show positive prion detection in all products. To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results show positive prion detection in all products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">9 Carrot plants as potential vectors for CWD transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Paulina Soto1,2, Francisca Bravo-Risi1,2, Claudio Soto1, Rodrigo Morales1,2</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> We show that edible plant components can absorb prions from CWD-contaminated soils and transport them to their aerial parts.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our results indicate that edible plants could participate as vectors of CWD transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to <span dir="ltr" style="outline: none;">tg650</span> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of <span dir="ltr" style="outline: none;">tg650</span> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Unprecedented in human prion disease, feces of CWD-inoculated <span dir="ltr" style="outline: none;">tg650</span> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and <span dir="ltr" style="outline: none;">tg650</span> with fecal homogenates. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286 </div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the disease within humans might become endemic.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acta Neuropathol 144, 767–784 (2022). <a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Published</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 August 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifn1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">© The Author(s) 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Keywords Chronic wasting disease · CWD · Zoonotic potential · Prion strains · Zoonotic prions</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">HIGHLIGHTS OF THIS STUDY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">================================</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In this study, we evaluated the zoonotic potential of CWD using a transgenic mouse model overexpressing human M129-PrPC (<span dir="ltr" style="outline: none;">tg650</span> [12]). We inoculated <span dir="ltr" style="outline: none;">tg650</span> mice intracerebrally with two deer CWD isolates, Wisc-1 and 116AG [22, 23, 27, 29]. We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions. Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage. Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for infection. We demonstrated in a human transgenic mouse model that the species barrier for transmission of CWD to humans is not absolute. The fact that its signature was not typical raises the questions whether CWD would manifest in humans as a subclinical infection, whether it would arise through direct or indirect transmission including an intermediate host, or a silent to uncovered human-to-human transmission, and whether current detection techniques will be suffcient to unveil its presence.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of <span dir="ltr" style="outline: none;">tg650</span> mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=================================</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Supplementary Information The online version contains supplementary material available at </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...see full text;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/article/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/article/10.1007/s00401-022-02482-9</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/content/pdf/10.1007/s00401-022-02482-9.pdf</a></div><div style="outline: none;"> </div></div><div dir="ltr" style="outline: none;"><div style="outline: none;">EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ... See all authors </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">First published: 17 January 2018 https://doi.org/10.2903/j.efsa.2018.5132</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">also, see; </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers.. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Research Paper</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cellular prion protein distribution in the vomeronasal organ, parotid, and scent glands of white-tailed deer and mule deer</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Anthony Ness, Aradhana Jacob, Kelsey Saboraki, Alicia Otero, Danielle Gushue, Diana Martinez Moreno, Melanie de Peña, Xinli Tang, Judd Aiken, Susan Lingle & Debbie McKenzieORCID Icon show less</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Pages 40-57 | Received 03 Feb 2022, Accepted 13 May 2022, Published online: 29 May 2022</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Download citation</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://doi.org/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1080/19336896.2022.2079888</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ABSTRACT</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic wasting disease (CWD) is a contagious and fatal transmissible spongiform encephalopathy affecting species of the cervidae family. CWD has an expanding geographic range and complex, poorly understood transmission mechanics. CWD is disproportionately prevalent in wild male mule deer and male white-tailed deer. Sex and species influences on CWD prevalence have been hypothesized to be related to animal behaviours that involve deer facial and body exocrine glands. Understanding CWD transmission potential requires a foundational knowledge of the cellular prion protein (PrPC) in glands associated with cervid behaviours. In this study, we characterized the presence and distribution of PrPC in six integumentary and two non-integumentary tissues of hunter-harvested mule deer (Odocoileus hemionus) and white-tailed deer (O. virginianus). We report that white-tailed deer expressed significantly more PrPC than their mule deer in the parotid, metatarsal, and interdigital glands. Females expressed more PrPC than males in the forehead and preorbital glands. The distribution of PrPC within the integumentary exocrine glands of the face and legs were localized to glandular cells, hair follicles, epidermis, and immune cell infiltrates. All tissues examined expressed sufficient quantities of PrPC to serve as possible sites of prion initial infection, propagation, and shedding.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ARS RESEARCH Generation of human chronic wasting disease in transgenic mice </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Publication Acceptance Date: 9/8/2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Generation of human chronic wasting disease in transgenic mice</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author item WANG, ZERUI - Case Western Reserve University (CWRU) item QIN, KEFENG - University Of Chicago item CAMACHO, MANUEL - Case Western Reserve University (CWRU) item SHEN, PINGPING - Case Western Reserve University (CWRU) item YUAN, JUE - Case Western Reserve University (CWRU) item Greenlee, Justin item CUI, LI - Jilin University item KONG, QINGZHONG - Case Western Reserve University (CWRU) item MASTRIANNI, JAMES - University Of Chicago item ZOU, WEN-QUAN - Case Western Reserve University (CWRU)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Submitted to: Acta Neuropathologica Publication Type: Peer Reviewed Journal Publication Acceptance Date: 9/8/2021 Publication Date: N/A Citation: N/A</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Interpretive Summary: Prion diseases are invariably fatal neurologic diseases for which there is no known prevention or cure. Chronic wasting disease (CWD) is the prion disease of deer and elk and is present in farmed and free ranging herds throughout North America. To date there is no clear evidence that the CWD agent could be transmitted to humans. This manuscript describes the use of an in vitro technique, cell-free serial protein misfolding cyclic amplification (sPMCA), to generate a CWD prion that is infectious to transgenic mice expressing the human prion protein. This study provides the first evidence that CWD prions may be able to cause misfolding in the human prion protein. This information will impact medical experts and those involved in making policy for farmed cervids and wildlife.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Technical Abstract: Chronic wasting disease (CWD) is a cervid spongiform encephalopathy or prion disease caused by the infectious prion or PrPSc, a misfolded conformer of cellular prion protein (PrPC). It has rapidly spread in North America and also has been found in Asia and Europe. In contrast to the zoonotic mad cow disease that is the first animal prion disease found transmissible to humans, the transmissibility of CWD to humans remains uncertain although most previous studies have suggested that humans may not be susceptible to CWD. Here we report the generation of an infectious human PrPSc by seeding CWD PrPSc in normal human brain PrPC through the in vitro cell-free serial protein misfolding cyclic amplification (sPMCA). Western blotting confirms that the sPMCA-induced proteinase K-resistant PrPSc is a human form, evidenced by a PrP-specific antibody that recognizes human but not cervid PrP. Remarkably, two lines of humanized transgenic (Tg) mice expressing human PrP-129Val/Val (VV) or -129Met/Met (MM) polymorphism develop prion disease at 233 ± 6 (mean ± SE) days post-inoculation (dpi) and 552 ± 27 dpi, respectively, upon intracerebral inoculation with the sPMCA-generated PrPSc. The brain of diseased Tg mice reveals the electrophoretic profile of PrPSc similar to sporadic Creutzfeldt-Jakob disease (sCJD) MM1 or VV2 subtype but different neuropathological patterns. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We believe that our study provides the first evidence that CWD PrPSc is able to convert human PrPC into PrPSc in vitro and the CWD-derived human PrPSc mimics atypical sCJD subtypes in humanized Tg mice.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=382551" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=382551</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''The brain of diseased Tg mice reveals the electrophoretic profile of PrPSc similar to sporadic Creutzfeldt-Jakob disease (sCJD) MM1 or VV2 subtype but different neuropathological patterns.'' </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''We believe that our study provides the first evidence that CWD PrPSc is able to convert human PrPC into PrPSc in vitro and the CWD-derived human PrPSc mimics atypical sCJD subtypes in humanized Tg mice.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Published: 26 September 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Generation of human chronic wasting disease in transgenic mice</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Zerui Wang, Kefeng Qin, Manuel V. Camacho, Ignazio Cali, Jue Yuan, Pingping Shen, Justin Greenlee, Qingzhong Kong, James A. Mastrianni & Wen-Quan Zou</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acta Neuropathologica Communications volume 9, Article number: 158 (2021)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic wasting disease (CWD) is a cervid prion disease caused by the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC). It has been spreading rapidly in North America and also found in Asia and Europe. Although bovine spongiform encephalopathy (i.e. mad cow disease) is the only animal prion disease known to be zoonotic, the transmissibility of CWD to humans remains uncertain. Here we report the generation of the first CWD-derived infectious human PrPSc by elk CWD PrPSc-seeded conversion of PrPC in normal human brain homogenates using in vitro protein misfolding cyclic amplification (PMCA). Western blotting with human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPSc was derived from the human PrPC substrate. Two lines of humanized transgenic mice expressing human PrP with either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPSc patterns and neuropathological changes in the brain. Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSc can cross the species barrier to convert human PrPC into infectious PrPSc that can produce bona fide prion disease when inoculated into humanized transgenic mice.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">It is worth noting that the annual number of sporadic CJD (sCJD) cases in the USA has increased, with the total number of suspected and confirmed sCJD cases rising from 284 in 2003 to 511 in 2017 (<span dir="ltr" style="outline: none;">https://www.cdc.gov/prions/cjd/occurrence-transmission.html</span>). The greatly enhanced CJD surveillance and an aging population in the USA certainly contributed to the observed increase in annual sCJD case numbers in recent years, but the possibility cannot be excluded that some of the increased sCJD prevalence is linked to CWD exposure.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In the present study, using serial protein misfolding cyclic amplification (sPMCA) assay we generate PrPSc by seeding CWD prions in normal human brain homogenates. Importantly, we reveal that two lines of humanized Tg mice expressing human PrP-129VV and 129MM develop prion diseases upon intracerebral inoculation of the abnormal PrP generated by sPMCA. We believe that our study provides the first opportunity to dissect the clinical, pathological and biochemical features of the CWD-derived human prion disease in two lines of humanized Tg mice expressing two major human PrP genotypes, respectively.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://actaneurocomms.biomedcentral.com/articles/10.1186/s40478-021-01262-y" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://actaneurocomms.biomedcentral.com/articles/10.1186/s40478-021-01262-y</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">i thought i might share some news about cwd zoonosis that i got, that i cannot share or post to the public yet, i promised for various reasons, one that it will cause a shit storm for sure, but it was something i really already knew from previous studies, but, i was told that ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">==================</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''As you can imagine, 2 and 5 (especially 5) may raise alarms. The evidence we have for 4 are not as strong or tight as I would like to have. At this point, please do not post any of the points publicly yet, but you can refer to points 1-3 in private discussions and all 5 points when discussing with relevant public officials to highlight the long-term risks of CWD zoonosis.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">====================</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">so, i figure your as about as official as it gets, and i think this science is extremely important for you to know and to converse about with your officials. it's about to burn a whole in my pocket. this is about as close as it will ever get for cwd zoonosis to be proven in my time, this and what Canada Czub et al found with the Macaques, plus an old study from cjd surveillance unit back that showed cjd and a 9% increase in risk from folks that eat venison, i will post all this below for your files Sir. i remember back in the BSE nvCJD days, from when the first BSE case in bovine was confirmed around 1984 maybe 83, i forget the good vets named that screwed it up first, Carol something, but from 83ish to 95 96 when nvCJD was linked to humans from BSE in cattle, so that took 10 to 15 years. hell, at that rate, especially with Texas and cwd zoonsis, hell, i'll be dead before it's official, if ever, so here ya go Sir. there was a grant study on cwd zoonosis that had been going on for some time, i followed it over the years, then the grant date for said study had expired, so, i thought i would write the good Professor about said study i.e. Professor Kong, CWRU et al. i will post the grant study abstract first, and then after that, what reply i got back, about said study that i was told not to post/publish...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CWD ZOONOSIS GRANT FIRST;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><span style="background-color: whitesmoke; color: #333333; outline: none; text-align: justify;"><br style="outline: none;" /></span></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Cervid to human prion transmission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Kong, Qingzhong </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Case Western Reserve University, <span dir="ltr" style="outline: none;">Cleveland, OH, United States</span></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Abstract Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that: (1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; (2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence; (3) Reliable essays can be established to detect CWD infection in humans; and (4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of humanized Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental human CWD samples will also be generated for Aim 3. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental human CWD samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> Funding Agency Agency National Institute of Health (NIH) Institute National Institute of Neurological Disorders and Stroke (NINDS) Type Research Project (R01) Project # 1R01NS088604-01A1 Application # <span dir="ltr" style="outline: none;">9037884</span> Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) Program Officer Wong, May Project Start 2015-09-30 Project End 2019-07-31 Budget Start 2015-09-30 Budget End 2016-07-31 Support Year 1 Fiscal Year 2015 Total Cost $337,507 Indirect Cost $118,756</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://grantome.com/grant/NIH/R01-NS088604-01A1#panel-abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://grantome.com/grant/NIH/R01-NS088604-01A1#panel-abstract</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Professor Kongs reply to me just this month about above grant study that has NOT been published in peer reveiw yet...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=================================</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Here is a brief summary of our findings:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...can't post, made a promise...tss</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">On Sat, Apr 3, 2021 at 12:19 PM Terry Singeltary <<span dir="ltr" style="outline: none;">flounder9@verizon.net</span>> wrote:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">end...tss</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">==============</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CWD ZOONOSIS THE FULL MONTY TO DATE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">International Conference on Emerging Diseases, Outbreaks & Case Studies & 16th Annual Meeting on Influenza March 28-29, 2018 | Orlando, USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Qingzhong Kong</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Case Western Reserve University School of Medicine, USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Zoonotic potential of chronic wasting disease prions from cervids</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic wasting disease (CWD) is the prion disease in cervids (mule deer, white-tailed deer, American elk, moose, and reindeer). It has become an epidemic in North America, and it has been detected in the Europe (Norway) since 2016. The widespread CWD and popular hunting and consumption of cervid meat and other products raise serious public health concerns, but questions remain on human susceptibility to CWD prions, especially on the potential difference in zoonotic potential among the various CWD prion strains. We have been working to address this critical question for well over a decade. We used CWD samples from various cervid species to inoculate transgenic mice expressing human or elk prion protein (PrP). We found infectious prions in the spleen or brain in a small fraction of CWD-inoculated transgenic mice expressing human PrP, indicating that humans are not completely resistant to CWD prions; this finding has significant ramifications on the public health impact of CWD prions. The influence of cervid PrP polymorphisms, the prion strain dependence of CWD-to-human transmission barrier, and the characterization of experimental human CWD prions will be discussed.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Speaker Biography Qingzhong Kong has completed his PhD from the University of Massachusetts at Amherst and Post-doctoral studies at Yale University. He is currently an Associate Professor of Pathology, Neurology and Regenerative Medicine. He has published over 50 original research papers in reputable journals (including Science Translational Medicine, JCI, PNAS and Cell Reports) and has been serving as an Editorial Board Member on seven scientific journals. He has multiple research interests, including public health risks of animal prions (CWD of cervids and atypical BSE of cattle), animal modeling of human prion diseases, mechanisms of prion replication and pathogenesis, etiology of sporadic Creutzfeldt-Jacob disease (CJD) in humans, normal cellular PrP in the biology and pathology of multiple brain and peripheral diseases, proteins responsible for the α-cleavage of cellular PrP, as well as gene therapy and DNA vaccination.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><span dir="ltr" style="outline: none;">qxk2@case.edu</span> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.alliedacademies.org/conference-abstracts-files/zoonotic-potential-of-chronic-wasting-disease-prions-from.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.alliedacademies.org/conference-abstracts-files/zoonotic-potential-of-chronic-wasting-disease-prions-from.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://prionconference.blogspot.com/2018/02/prion-round-table-conference-2018-may.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prionconference.blogspot.com/2018/02/prion-round-table-conference-2018-may.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://prionconference.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://prionconference.blogspot.com/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SUNDAY, JULY 25, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">North American and Norwegian Chronic Wasting Disease prions exhibit different potential for interspecies transmission and zoonotic risk </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''Our data suggest that reindeer and red deer from Norway could be the most transmissible CWD prions to other mammals, whereas North American CWD prions were more prone to generate human prions in vitro.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2021/07/north-american-and-norwegian-chronic.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2021/07/north-american-and-norwegian-chronic.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MONDAY, JULY 19, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> U Calgary researchers at work on a vaccine against a fatal infectious disease affecting deer and potentially people</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2021/07/u-calgary-researchers-at-work-on.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2021/07/u-calgary-researchers-at-work-on.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion Conference 2018 Abstracts</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BSE aka MAD COW DISEASE, was first discovered in 1984, and it took until 1995 to finally admit that BSE was causing nvCJD, the rest there is history, but that science is still evolving i.e. science now shows that indeed atypical L-type BSE, atypical Nor-98 Scrapie, and typical Scrapie are all zoonosis, zoonotic for humans, there from. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">HOW long are we going to wait for Chronic Wasting Disease, CWD TSE Prion of Cervid, and zoonosis, zoonotic tranmission to humans there from?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Studies have shown since 1994 that humans are susceptible to CWD TSE Prion, so, what's the hold up with making CWD a zoonotic zoonosis disease, the iatrogenic transmissions there from is not waiting for someone to make a decision.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion Conference 2018 Abstracts</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Background</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Methods</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = <span dir="ltr" style="outline: none;">1.01 - 1.23</span>), as did low endemic states (RR: 1.15, 95% CI = <span dir="ltr" style="outline: none;">1.04 - 1.27</span>). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = <span dir="ltr" style="outline: none;">1.10 – 2.24</span>) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = <span dir="ltr" style="outline: none;">1.02 - 1.26</span>) and low endemic states (RR: 1.16, 95% CI = <span dir="ltr" style="outline: none;">1.04 - 1.29</span>). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = <span dir="ltr" style="outline: none;">1.10 - 1.48</span>), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P172 Peripheral Neuropathy in Patients with Prion Disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Wang H(1), Cohen M(1), Appleby BS(1,2)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Background</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Methods</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P180 Clinico-pathological analysis of human prion diseases in a brain bank series</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Background and objective:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Methods:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Discussion:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Methods:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions:</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. <span dir="ltr" style="outline: none;">After 5-7</span> years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">See also poster P103</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WA16 Monitoring Potential CWD Transmission to Humans</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Belay ED</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">=====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Source Prion Conference 2018 Abstracts</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://prionconference.blogspot.com/2018/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://prionconference.blogspot.com/2018/</a> </div></div><div style="outline: none;"><span style="background-color: whitesmoke; color: #333333; outline: none; text-align: justify;"><br style="outline: none;" /></span></div><div style="outline: none;"><span style="background-color: whitesmoke; color: #333333; outline: none; text-align: justify;">Volume 24, Number <span dir="ltr" style="outline: none;">8—August</span> 2018 </span><br style="outline: none;" /></div></div><div style="outline: none;"><div style="font-size: 30.2px; font-stretch: normal; line-height: normal; margin: 0px 0px 3px; outline: none;"><span style="background-color: whitesmoke; color: #333333; font-size: 16px; outline: none; text-align: justify;">Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions</span></div></div></div><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="font-size: 13.3333px; outline: none; text-align: justify;"><div style="font-size: 10pt; outline: none;"><div dir="ltr" style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px; outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none;"><div dir="ltr" style="color: #333333; font-family: sans-serif; margin-bottom: 1em; margin-top: 1em; outline: none;"><div style="outline: none;">Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion</a></div></div><div style="color: #333333; font-family: sans-serif; margin-bottom: 1em; margin-top: 1em; outline: none;"><span style="color: #222222; outline: none;">Prion 2017 Conference Abstracts</span></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: none;"><div style="font-family: arial; font-size: 13.3333px; outline: none;"><div style="font-size: 10pt; outline: none;"><div style="font-family: arial, helvetica; font-size: 12px; margin-bottom: 24px; outline: none;"><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 </span></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen </span><br clear="none" style="outline: none;" /></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">This is a progress report of a project which started in 2009. </span></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. </span><br clear="none" style="outline: none;" /></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. </span><br clear="none" style="outline: none;" /></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range <span dir="ltr" style="outline: none;">from 6.4 to 7.10</span> years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. </span><br clear="none" style="outline: none;" /></div><div style="margin-bottom: 24px; outline: none;"><span style="font-size: 16px; outline: none;">PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE</span></div><div dir="ltr" style="margin-bottom: 24px; outline: none;"><div dir="ltr" style="outline: none;"><span style="font-size: 16px; outline: none;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available. </span></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div></div><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">SATURDAY, FEBRUARY 23, 2019 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">TUESDAY, NOVEMBER 04, 2014 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011 Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. " </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Transmission Studies Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip.... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://jvi.asm.org/content/83/18/9608.full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://jvi.asm.org/content/83/18/9608.full</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://science.sciencemag.org/content/311/5764/1117..long" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://science.sciencemag.org/content/311/5764/1117..long</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans” </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ??? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Date: September 30, 2002 at 7:06 am PST </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From: "Belay, Ermias" </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Ermias Belay, M.D. Centers for Disease Control and Prevention </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-----Original Message----- From: </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Thursday, April 03, 2008 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... *** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***, </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... full text ; </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way; </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">sporadic = 54,983 hits </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">spontaneous = 325,650 hits </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">> However, to date, no CWD infections have been reported in people. key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ *** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From: Steve Dealler </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To: BSE-L@ References: </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Dear Terry,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Steve Dealler </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">====</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Table 9 presents the results of an analysis of these data.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...see full report ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> <a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BSE Inquiry Steve Dealler</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Management In Confidence</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BSE: Private Submission of Bovine Brain Dealler</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...see full text;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MONDAY, FEBRUARY 25, 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Prion 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: N/A</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgment: XXX</div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><span style="outline: none;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."</span><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><div style="outline: none;">2001 Singeltary on CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">February 14, 2001</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary, Sr</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author Affiliations</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div></div></div></div></div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">RE: re-Human Prion Diseases in the United States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT I would kindly like to add to my initial concerns, something I brought up years ago, and I believe that still hold true today, more so even than when I first stated these concerns in 2003 ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">routine passive mortality CJD surveillance USA ?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THIS has been proven not to be very useful in the U.K.;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys by 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20040521215716/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">IDENTIFICATION OF CASES</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">full text;</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;">THE PATHOLOGICAL PROTEIN</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">June 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BY Philip Yam</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CHAPTER 14 LAYING ODDS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.thepathologicalprotein.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.thepathologicalprotein.com/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate infor-mation showed that “we miss about 14 percent,” said CDC epidemiolo-gist Lawrence Schonberger. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://link.springer.com/chapter/10.1007/0-387-21755-x_14" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/chapter/10.1007/0-387-21755-x_14</a></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary, retired (medically), CJD WATCH</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Submitted March 26, 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">August 10, 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Greetings,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">But, while sub-clinical, how many can one exposed human infect? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">please see history, and the ever evolving TSE science to date ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Saturday, June 13, 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary 2000</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BMJ 2000; 320 doi: <a href="https://doi.org/10.1136/bmj.320.7226.8/b" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1136/bmj.320.7226.8/b</a> (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">02 January 2000 Terry S Singeltary retired</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Rapid Response: </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Something else I find odd, page 16;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A few more factors to consider, page 15;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To be continued...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary Sr. Bacliff, Texas USA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Competing interests: No competing interests</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Tracking spongiform encephalopathies in North America</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xavier Bosch</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Available online 29 July 2003. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Volume 3, Issue 8, August 2003, Page 463 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Volume 3, Number 8 01 August 2003</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Newsdesk</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Tracking spongiform encephalopathies in North America</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Xavier Bosch</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a></div><div style="outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="outline: none;"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary 2007</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">by Philip Yam </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Revisiting Sporadic CJD</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">www.madcow.org</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">223</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">www.vegsource.com</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">224 CHAPTER 14</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Laying Odds 225</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">226 CHAPTER 14</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">A Case for Undercounting</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Laying Odds 227</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a onein-a-million lottery, it’s more like one-in-2.5-million for AfricanAmericans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">SNIP...SEE FULL TEXT;</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary Submission SEAC 2007</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a></span></p><div style="outline: none;"><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><br style="outline: none;" /></div></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">2023</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">United States of America - Bovine spongiform encephalopathy - Immediate notification<br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">GENERAL INFORMATION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">COUNTRY/TERRITORY OR ZONE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">COUNTRY/TERRITORY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ANIMAL TYPE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">TERRESTRIAL</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DISEASE CATEGORY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Listed disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EVENT ID 5067</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DISEASE Bovine spongiform encephalopathy</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CAUSAL AGENT Bovine spongiform encephalopathy prion, atypical strain, L-type</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">START DATE 2023/05/15</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">REASON FOR NOTIFICATION Recurrence of an eradicated disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DATE OF LAST OCCURRENCE 2018/08/28</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CONFIRMATION DATE 2023/05/18</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EVENT STATUS On-going</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">END DATE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EPIDEMIOLOGY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SOURCE OF EVENT OR ORIGIN OF INFECTION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Spontaneous mutation</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Unknown or inconclusive</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EPIDEMIOLOGICAL COMMENTS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">As part of the United States’ targeted surveillance program for bovine spongiform encephalopathy (BSE), a case of atypical BSE was identified in a nine year old beef type cow. This atypical BSE case was classified as L-type. In over 25 years of surveillance, the six native cases detected in the United States have all been atypical cases. The identified animal did not enter any food supply channels and at no time presented a risk to human health. Specified risk material removal and ruminant-to-ruminant feed bans continue to be effectively applied.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">QUANTITATIVE DATA SUMMARY</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MEASURING UNIT</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Animal</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Species Susceptible Cases Deaths Killed and Disposed of Slaughtered/ Killed for commercial use Vaccinated</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cattle (DOMESTIC)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">NEW-1-1--TOTAL-1-1--</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DIAGNOSTIC DETAILS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CLINICAL SIGNS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">YES</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">METHOD OF DIAGNOSTIC</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Diagnostic test</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Test name Laboratory Species sampled Number of outbreaks sampled First result date Latest result date Result</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Immunohistochemistry (IHC) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 2023/05/22 2023/05/22 Positive</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Antigen capture enzyme-linked immunosorbent assay (AC-ELISA) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023/05/18 2023/05/18 Positive</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Antigen detection Western blot (Ag Western blot) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 2023/05/18 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2023/05/18 Positive</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CONTROL MEASURES AT EVENT LEVEL</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CONTROL MEASURES AT EVENT LEVEL</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DOMESTIC ANIMALS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WILD ANIMALS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Official disposal of carcasses, by-products and waste</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Applied</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Screening</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Applied</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Traceability</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Applied</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">NEW OUTBREAKS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">OB_118941 - TENNESSEE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">OUTBREAK REFERENCE -</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">START DATE 2023/05/15</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">END DATE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DETAILED CHARACTERISATION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">FIRST ADMINISTRATIVE DIVISION Tennessee</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SECOND ADMINISTRATIVE DIVISION Davidson</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THIRD ADMINISTRATIVE DIVISION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">EPIDEMIOLOGICAL UNIT Farm</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">LOCATION Tennessee</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Latitude, Longitude 36.165 , -86.784</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(Approximate location) OUTBREAKS IN CLUSTER</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">MEASURING UNIT Animal</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">AFFECTED POPULATION DESCRIPTION</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Nine year old beef type cow. Note: Tracing efforts to date place this animal’s origin in the State of Tennessee. Location coordinates are approximate to the Tennessee State Capitol.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Species Susceptible Cases Deaths Killed and Disposed of Slaughtered/Killed for commercial use Vaccinated</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cattle (DOMESTIC)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">NEW-1-1--TOTAL-1-1--</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">METHOD OF DIAGNOSTIC</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Diagnostic test</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CONTROL MEASURES DIFFERENT FROM EVENT LEVEL MEASURES NOT IMPLEMENTED</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ADDITIONAL MEASURES</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">-</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/5067</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">snip...see full text and more here;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;">TWO WEEKS BEFORE, I WROTE AND SUBMITTED THIS TO USDA ET AL;</div><div data-setdir="false" dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><span style="outline: none;">''ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year.'' </span><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;">MAY 2, 2023</div><div data-setdir="false" dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Docket No. APHIS–2023–0027 Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Greetings again APHIS et al,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I would kindly like to again, post my concern or urgency, on why said information is so critical, and why the 3 year extension is so critical, especially today, with the recent mad cow cases in the UK, Switzerland, Brazil, Spain, and The Netherlands all atypical BSE cases, and the fact the OIE is so concerned with the recent science about atypical L-type BSE and atypical H-type BSE, both of which can transmit orally, (see OIE BSE atypical in my reference materials), new outbreak of a new Prion disease in a new livestock species, i.e. the camel. The fact Chronic Wasted Disease CWD TSE Prion of Cervid, is spreading across the USA, with no stopping it in the near future, now with 10 different strains, a spillover into cattle or sheep would be devastating, and the ramifications of human zoonosis there from, has great concern throughout the scientific community. The fact that the USA BSE feed ban was and is a joke today (see why, with the fact that CWD positive deer could enter the food/feed chain for other ruminants and what the DEFRA says), how the BSE surveillance and testing has failed us so terribly bad to date, by testing only 25k bovines a year for BSE, you will not find BSE until it's too late, again. THIS is all why INFORMATION COLLECTION is so vital for BSE and all human and animal Transmissible Spongiform Encephalopathy TSE Prion disease.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''The purpose of this notice is to solicit comments from the public (as well as affected agencies) concerning our information collection. These comments will help us:''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(1) Evaluate whether the collection of information is necessary for the proper performance of the functions of the Agency, including whether the information will have practical utility;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(2) Evaluate the accuracy of our estimate of the burden of the collection of information, including the validity of the methodology and assumptions used;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(3) Enhance the quality, utility, and clarity of the information to be collected; and</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(4) Minimize the burden of the collection of information on those who are to respond, through use, as appropriate, of automated, electronic, mechanical, and other collection technologies; ...end</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The question should be, what will the burden be, if WE DON'T COLLECT SAID INFORMATIONS ON BSE, and we find ourselves again facing a BSE epidemic?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I want to bring your attention too, and emphasize;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">(3) Enhance the quality, utility, and clarity of the information to be collected; and...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I remember that infamous TEXAS MAD COW that instead of a 48 turnaround at Weybridge, said suspect positive, was declared NEGATIVE, until an Act of Congress and the Honorable Phyllis Fong overrode Texas negative test, sent that BSE sample to Weybridge, and 6 MONTHS LATER ON A 48 HOUR TURNAROUND (BSE REDBOOKS), that BSE sample was CONFIRMED POSITIVE (see history in my references).</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Let's not kid ourselves, the BSE ENHANCED BSE SURVEILLANE efforts way back was a total failure, that's why it was shut down, too many atypical BSE cases were showing up.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THE world was set back to square one with the BSE Minimal Risk Regions, from the BSE GBRs.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">WE must enhance our BSE Surveillance and BSE Testing, and the FDA PART 589 TSE PRION FEED BAN must be revised to include Cervid by-products and SRM, and it should be made MANDATORY, AND THIS SHOULD BE WELL DOCUMENTED with information collection.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary References</div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">see full submission;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Atypical BSE detected St. Gallen Bern, 13.07.2023 case 2 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.blv.admin.ch/blv/de/home/dokumentation/nsb-news-list.msg-id-96688.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.blv.admin.ch/blv/de/home/dokumentation/nsb-news-list.msg-id-96688.html</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">WAHIS, WOAH, OIE, REPORT Switzerland BSE 2023/03/08 case 1 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Wednesday, May 24, 2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">WAHIS, WOAH, OIE, USA BSE</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/5067</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Monday, March 20, 2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">WAHIS, WOAH, OIE, REPORT UK BSE</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">BRAZIL BSE START DATE 2023/01/18</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">SPAIN BSE START DATE 2023/01/21</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">NETHERLANDS BSE START DATE 2023/02/01</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></span></p><div style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;">'Spontaneous mutation'<br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">***> PLEASE NOTE!</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">34 Scientific Commission/September 2019</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">3. Atypical BSE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliations expand</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">PMID: 21266763</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">see full text;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: <a href="https://doi.org/10.3389/fvets.2019.00430" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.3389/fvets.2019.00430</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1985</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle... </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf</a></div></div></div><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Friday, May 19, 2023 | 04:12pm NASHVILLE — The Tennessee State Veterinarian is confirming a case of atypical bovine spongiform encephalopathy (BSE) in a cow with ties to Tennessee.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The cow appeared unwell after arriving at a packing company in South Carolina. In alignment with the United States Department of Agriculture’s BSE surveillance program, the animal was isolated and euthanized. It did not enter the food supply. Preliminary investigation has determined the cow originated in southeast Tennessee.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">“We are working closely with our federal partners and animal health officials in South Carolina for this response,” State Veterinarian Dr. Samantha Beaty said. “That includes determining prior owners and locations where the affected cow lived in Tennessee and tracing siblings and offspring for testing.”</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BSE is a chronic degenerative disease affecting the central nervous system of cattle. It is caused by an abnormal prion protein. The atypical form occurs spontaneously at very low levels in all cattle populations, particularly in older animals. Atypical BSE poses no known risk to human health. It is different from the classical form of BSE, which has not been detected in the U.S. since 2003.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">BSE is not contagious and therefore is not spread through contact between cattle or with other species. There is no treatment for or vaccine to prevent BSE. The U.S. has a strong surveillance program in place for early detection and to prevent suspect cattle from entering the food supply chain.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Cattle owners are always advised to monitor their herds for health. Cattle affected by BSE may display changes in temperament, abnormal posture, poor coordination, decreased milk production, or loss of condition without noticeable loss of appetite. Owners should report any herd health concerns to their veterinarian or to the State Veterinarian’s office at <span dir="ltr" style="outline: none;">615-837-5120</span>.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The Tennessee Department of Agriculture Animal Health Division is responsible for promoting animal health in Tennessee. The State Veterinarian’s office seeks to prevent the spread of disease through import and movement requirements, livestock traceability, disaster mitigation, and the services of the C.E. Kord Animal Health Diagnostic Laboratory. The division collaborates with other health-related stakeholders, academic institutions, and extension services to support One Health, an initiative to improve health for people and animals.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html</a> </div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><div dir="ltr" style="outline: none;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div></div></div><div style="outline: none;">The U.S. Department of Agriculture (USDA) is announcing an atypical case of Bovine Spongiform Encephalopathy (BSE), a neurologic disease of cattle, in an approximately five-year-old or older beef cow at a slaughter plant in South Carolina. This animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the United States. Given the United States’ negligible risk status for BSE, we do not expect any trade impacts as a result of this finding. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">USDA Animal and Plant Health Inspection Service’s (APHIS) National Veterinary Services Laboratories (NVSL) confirmed that this cow was positive for atypical L-type BSE. The animal was tested as part of APHIS’s routine surveillance of cattle that are deemed unsuitable for slaughter. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> This is the nation’s 7th detection of BSE. Of the six previous U.S. cases, the first, in 2003, was a case of classical BSE in a cow imported from Canada; the rest have been atypical (H- or L-type) BSE.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">The World Organization for Animal Health (WOAH) recognizes the United States as negligible risk for BSE. As noted in the WOAH guidelines for determining this status, atypical BSE cases do not impact official BSE risk status recognition as this form of the disease is believed to occur spontaneously in all cattle populations at a very low rate. Therefore, this finding of an atypical case will not change the negligible risk status of the United States, and should not lead to any trade issues. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"> The United States has a longstanding system of interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials - or the parts of an animal that would contain BSE should an animal have the disease - from all animals presented for slaughter. The second safeguard is a strong feed ban that protects cattle from the disease. Another important component of our system - which led to this detection - is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">More information about this disease is available in the BSE factsheet.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"># </div></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Docket No. APHIS–<span dir="ltr" style="outline: none;">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="outline: none;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">see full submission;</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div></div></div></div><br style="outline: none;" /></div><div style="outline: none;"><div dir="ltr" style="outline: none;"><span face="Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif" style="color: #141414; outline: none;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</span><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><span face="Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif" style="color: #141414; outline: none;"><br style="outline: none;" /></span></div><div dir="ltr" style="outline: none;"><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a><span face="Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif" style="color: #141414; outline: none;"><br style="outline: none;" /></span></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a></div></div><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div dir="ltr" style="outline: none;">BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?<br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">1st a bit of history </div></div></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p4" style="font-size: 30.2px; font-stretch: normal; line-height: normal; margin: 0px 0px 3px; outline: none;"><span style="font-family: UICTFontTextStyleBody; font-size: 30.24px; font-weight: bold; outline: none;">Chronic Wasting Disease in Texas</span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none;">A Real Disease with Proven Impacts</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Produced by a coalition of concerned hunters, landowners, & conservationists (last update 08/2023)</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Since 2012, CWD has been detected in wild deer in just 7 counties in Texas and is only established in the western panhandle and far west Texas.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">In that same period of time, captive deer breeders have exposed almost half of Texas counties to CWD. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://bit.ly/3xL16Gm" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bit.ly/3xL16Gm</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">As of August 2023, 116 Texas counties have received possibly infected breeder deer that cannot be located, putting more than 140,000 landowners at risk of the disease. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://bit.ly/3xL16Gm" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bit.ly/3xL16Gm</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">“It is interesting to note that, in 2001, the State of Texas shifted its deer management strategies toward the same leanings that Kroll has suggested for Wisconsin. In Texas, the change was brought about via heavy lobbying from the high-fence deer ranching industry. This pressure helped convince the Texas Parks and Wildlife to change their regulations and allow private landowners to select the own deer biologists.”</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://www.texasmonthly.com/story/which-side-fence-are-you" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.texasmonthly.com/story/which-side-fence-are-you</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">2012 “For 10 years, Texas has had an aggressive Chronic Wasting Disease prevention and monitoring program. Wildlife agency regulations prohibit importing deer into the state, and the agency has tested more than 26,000 hunter-taken deer and 7,400 animals from the captive-deer industry. None of those deer tested positive.”</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://www.chron.com/news/houston-texas/article/Brain-eating-disease-found-in-Texas-deer-3697731.php" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.chron.com/news/houston-texas/article/Brain-eating-disease-found-in-Texas-deer-3697731.php</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Texas CWD Surveillance Positives</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Counties where CWD Exposed Deer were Released</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Number of CWD Exposed Deer Released by County</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Chronic Wasting Disease CWD Captive Herds updated April 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"><a href="https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Chronic Wasting Disease CWD Captive Herds updated April 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"><a href="https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">CWD poses a significant threat to the future of hunting in Texas. Deer population declines of 45 and 50 percent have been documented in Colorado and Wyoming. A broad infection of Texas deer populations resulting in similar population impacts would inflict severe economic damage to rural communities and could negatively impact land markets. Specifically, those landowners seeking to establish a thriving herd of deer could avoid buying in areas with confirmed CWD infections. As they do with anthrax-susceptible properties, land brokers may find it advisable to inquire about the status of CWD infections on properties that they present for sale. Prospective buyers should also investigate the status of the wildlife on prospective properties. In addition, existing landowners should monitor developments as TPWD crafts management strategies to identify and contain this deadly disease. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Dr. Gilliland (c-gilliland@tamu.edu) is a research economist with the Texas Real Estate Research Center at Texas A&M University.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://www.recenter.tamu.edu/articles/tierra-grande/oh-deer-2314" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.recenter.tamu.edu/articles/tierra-grande/oh-deer-2314</a></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none; text-decoration-line: underline;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">TPWD Executive Order No. 23-003 CWD Emergency Rules Adopted for Movement of Breeder Deer </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Executive Orders</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Executive Order No. 23-003</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Date: July 24, 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The Executive Director finds that additional discoveries of CWD in free-ranging white-tailed deer within deer breeding facilities regulated under Parks and Wildlife Code, Chapter 43, Subchapter L and regulations adopted pursuant to that subchapter (31 TAC Chapter 65, Subchapters B and T) constitute an immediate danger to the white-tailed deer and mule deer resources of Texas and that the adoption of rules on an emergency basis with fewer than 30 days’ notice is necessary to address an immediate danger.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://tpwd.texas.gov/publications/executive_orders/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tpwd.texas.gov/publications/executive_orders/</a></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">15 minute mark video shows sick deer with cwd, and this deer DIED FROM CWD, IT'S DOCUMENTED, commentator says ''so if anyone every tells you, that a deer has never died from CWD, think of this picture, because the Wisconsin Veterinary Lab told us, what when they looked at her sample under a microscope, she was the hottest animal they had ever seen, and that's in terms of the fluorescents that comes off the slide when the look at it, so, a lot of Prion in her system.''</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">see much more about 2 hours long...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://www.youtube.com/watch?v=O3CAI-EwlgM" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.youtube.com/watch?v=O3CAI-EwlgM</a></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">TEXAS BREEDER DEER ESCAPEE WITH CWD IN THE WILD, or so the genetics would show?</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">OH NO, please tell me i heard this wrong, a potential Texas captive escapee with cwd in the wild, in an area with positive captive cwd herd?</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">apparently, no ID though. tell me it ain't so please...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">23:00 minute mark</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">''Free Ranging Deer, Dr. Deyoung looked at Genetics of this free ranging deer and what he found was, that the genetics on this deer were more similar to captive deer, than the free ranging population, but he did not see a significant connection to any one captive facility that he analyzed, so we believe, Ahhhhhh, this animal had some captive ahhh, whatnot.''</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://youtu.be/aoPDeGL6mpQ?t=1384" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://youtu.be/aoPDeGL6mpQ?t=1384</a></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Commission Agenda Item No. 5 Exhibit B</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">DISEASE DETECTION AND RESPONSE RULES</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">PROPOSAL PREAMBLE</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">1. Introduction. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> A third issue is the accuracy of mortality reporting. Department records indicate that for each of the last five years an average of 26 deer breeders have reported a shared total of 159 escapes. Department records for the same time period indicate an average of 31 breeding facilities reported a shared total of 825 missing deer (deer that department records indicate should be present in the facility, but cannot be located or verified). </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://tpwd.texas.gov/business/feedback/meetings/2022/1104/agenda/item.phtml?item=5" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tpwd.texas.gov/business/feedback/meetings/2022/1104/agenda/item.phtml?item=5</a></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">On January 21, 2017 a tornado took down thousands of feet of fence for a 420-acre illegal deer enclosure in Lamar County that had been subject to federal and state investigation for illegally importing white-tailed deer into Mississippi from Texas (a CWD positive state). Native deer were free to move on and off the property before all of the deer were able to be tested for CWD. Testing will be made available for a period of three years for CWD on the property and will be available for deer killed within a 5-mile radius of the property on a voluntary basis. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://www.mdwfp.com/media/254796/2016-17-deer-report.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.mdwfp.com/media/254796/2016-17-deer-report.pdf</a><br style="outline: none;" /></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="outline: none;"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Deep Throat to Singeltary BSE Mad Cow 2001 to 2023</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I remember what “deep throat” told me about Scrapie back around 2001, I never forgot, and it seems it’s come to pass;</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Confidential!!!!</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">---end personal email---end...tss </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(I never knew who this person was, but got me into the U.S. BSE Emergency 50 State conference call back 2001, and we corresponded for years about BSE TSE Prion, have not heard from in over a decade, but they were on the inside looking out. You can believe this or not, but this was real, i don’t make this stuff up…plus my endeavors to get those 1 million cattle tested for BSE failed. There was an ENHANCED BSE SURVEILLANCE put forth after 2003, we pushed for it, but it was abruptly shut down after the atypical BSE cases were popping up…a bit of history for anyone interested…terry)</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss) </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at..... </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!! And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall... </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!" </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.) </span></p><div style="outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div></div>END...TSS</div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;">and so it was...</div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Rapid recontamination of a farm building occurs after attempted prion removal</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">First published: 19 January 2019 <a href="https://doi.org/10.1136/vr.105054" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1136/vr.105054</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">snip...</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">***>This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://pubmed.ncbi.nlm.nih.gov/30602491/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/30602491/</a> </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Front. Vet. Sci., 14 September 2015 | <a href="https://doi.org/10.3389/fvets.2015.00032" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.3389/fvets.2015.00032</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">172. Establishment of PrPCWD extraction and detection methods in the farm soil</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Conclusions: Our studies showed that PrPCWD persist in 0.001% CWD contaminated soil for at least 4 year and natural CWD-affected farm soil. When cervid reintroduced into CWD outbreak farm, the strict decontamination procedures of the infectious agent should be performed in the environment of CWD-affected cervid habitat.</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">THE tse prion aka mad cow type disease is not your normal pathogen. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">you cannot cook the TSE prion disease out of meat. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">the TSE prion agent also survives Simulated Wastewater Treatment Processes. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">you can bury it and it will not go away. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">it’s not your ordinary pathogen you can just cook it out and be done with. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">***> that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8 </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">***> Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Laboratory of Central Nervous System Studies, National Institute of </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Neurological Disorders and Stroke, National Institutes of Health, </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Bethesda, MD 20892. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">PMID: <span dir="ltr" style="outline: none;">8006664</span> [PubMed - indexed for MEDLINE] </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="http://www.pnas.org/content/97/7/3418.full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.pnas.org/content/97/7/3418.full</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Detection of protease-resistant cervid prion protein in water from a CWD-endemic area </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">THURSDAY, FEBRUARY 28, 2019 </div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;">BSE infectivity survives burial for five years with only limited spread</div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Volume 15, Number </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">5—May</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> 2009</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s7" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic; outline: none;">Research</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s7" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic; outline: none;"><br style="outline: none;" /></span></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none;">Chronic Wasting Disease Prions in Elk Antler Velvet</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Rachel C. Angers<a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article#fn1" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">1</a>, Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O’Rourke, Aru Balachandran, and Glenn C. Telling</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p6" style="font-stretch: normal; line-height: normal; margin: 9px 0px 8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article#comment" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"><img alt="unknown.gif" class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629" data-inlineimagemanipulating="true" src="https://apis.mail.aol.com/ws/v3/mailboxes/@.id==VjN-ce6z-DxIu_S2g8qZB3zEhBzmZgrr3THL4IwkE1wVplQCkvSg4OstfRBZyImsL89EhqbiNPt5J57JQHOERly2uw/messages/@.id==AB0QSBABXbycZV46iwNu6MZ3Qzc/content/parts/@.id==2/refresh?appid=AolMailNorrin&ymreqid=d41d8cd9-8f00-b204-1c2b-b90006019a00" style="outline: none;" /></a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Author affiliations: University of Kentucky Medical Center, Lexington, Kentucky, USA (R.C. Angers, T.S. Seward, D. Napier, M. Green, G.C. Telling); Colorado State University, Fort Collins, Colorado, USA (E. Hoover, T. Spraker); US Department of Agriculture, Pullman, Washington, USA (K. O’Rourke); Canadian Food Inspection Agency, Ottawa, Ontario, Canada (A. Balachandran); 1Current affiliation: Medical Research Council Laboratory of Molecular Biology, Cambridge, UK.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none;">Abstract</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the apical skin layer covering the growing antler (antler velvet) by using CWD-susceptible transgenic mice and protein misfolding cyclic amplification. Our finding of prions in antler velvet of CWD-affected elk suggests that this tissue may play a role in disease transmission among cervids. Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. The fact that CWD prion incubation times in transgenic mice expressing elk prion protein are consistently more rapid raises the possibility that residue 226, the sole primary structural difference between deer and elk prion protein, may be a major determinant of CWD pathogenesis.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article</a></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">TUESDAY, MAY 11, 2021</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusion</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CREUTZFELDT JAKOB DISEASE: A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">i was warning England and the BSE Inquiry about just this, way back in 1998, and was ask to supply information to the BSE Inquiry. for anyone that might be interested, see;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">ABOUT that deer antler spray and CWD TSE PRION... I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease. just saying...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sender: "Patricia Cantos"</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To: "Terry S Singeltary Sr. (E-mail)"</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Subject: Your submission to the Inquiry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Date: Fri, 3 Jul 1998 10:10:05 +0100 3 July 1998</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Mr Terry S Singeltary Sr. E-Mail: Flounder at <span dir="ltr" style="outline: none;">wt.net</span> Ref: E2979</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Dear Mr Singeltary, Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died. As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments. Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><span dir="ltr" style="outline: none;">http://www.bse.org.uk</span>.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on... snip...end...tss</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year previously and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. both cases confirmed. ...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">kind regards, terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS IPLEX, mad by standard process; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. also; what about potential mad cow candy bars ? see their potential mad cow candy bar list too... THESE are just a few of MANY of just this ONE COMPANY...TSS</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">''So, in sum, dietary supplements sold in the United States often contain ruminant tissues from undisclosed sources. Personally, I am rather squeamish and I don't think I would be eating prostate or testicle or pituitary, but I am also a little bit wary of consuming products with those glands, not just out of personal repugnance but simply out of a health concern.'' </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DEPARTMENT OF HEALTH AND HUMAN SERVICES FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE Friday, January 19, 2001</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">15 Open Public Hearing</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">16 DR. FREAS: We are opening the open public hearing</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 now. We have received one response to speak in this</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">18 afternoon's open public hearing. That is from Dr. Scott</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">19 Norton. If Dr. Norton is here, would you please come</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">20 forward. You can either use the podium or the microphone,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">21 whichever is your choice.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 DR. NORTON: I am Scott Norton and I am a</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">23 physician in the Washington D.C. area. I am here speaking</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">24 as a private citizen today.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">25 I first became concerned about the presence of 231</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 tissues from ruminant animals in dietary supplements about</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2 six months ago and expressed my concern in a letter that was 3 published in New England Journal of Medicine in July of Year 4 2000. 5 A couple of the products that I had looked at, and 6 examined their labels, that raised these concerns I brought 7 in right here. I will just read some of the organs that are 8 found in one that is called Male Power. Deer antler, 9 pancreas, orchic--despite what we just heard that the FDA</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">10 prefers the term "testicular tissue" to be written on the</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">11 labels, I have never seen a dietary supplement say</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">12 "testicle." They always say "orchis" or "orchic" which may</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">13 sound rather flowery to the etymologically impaired--thymus,</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">14 adrenal, heart, lymph node, prostate, spleen and pituitary.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">15 There are actually seventeen organs in that particular</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">16 product.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 There is another product that is called Brain</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">18 Nutrition that tells us that it is vitamins and minerals</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">19 essential for important brain function. It does not mention</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">20 that there is any glandulars on at least the bold print. 21 But if you look at the small print on the back, we learn</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 that it has brain extract and pituitary extract, raw, in</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">23 there.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">24 We know that many of the organs that can be found</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">25 in the dietary supplements do fall in that list of organs</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">232</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 that are suspect for contamination with TSEs, the labels, in 2 nearly all cases, identify neither the animal source nor the 3 geographic location from which the organs were derived. I 4 have seen one line that did specify from New Zealand cattle 5 but no other manufacturer will list either the species or 6 the geographic location. 7 The FDA's and the USDA's import alerts that we 8 just learned about prohibit the use of these organs in 9 foods, medicines and medical devices. But my reading of the</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">10 alert, 17-04, suggests that DSHEA does allow some loopholes</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">11 for these tissues to possible slip in.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">12 I will just read <span dir="ltr" style="outline: none;">from 17-04</span> that we heard. On the</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">13 first page, it says that, "This alert does not establish any</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">14 obligations on regulated entities." I love seeing</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">15 legislation that starts out with that caveat.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">16 Then it says, further, "The USDA regulations do</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 not apply to bovine-derived materials intended for human</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">18 consumption as finished dietary supplements." We also learn</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">19 that the prohibition, or the import alert, is limited to</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">20 bulk lots of these tissues, completed tissues, from BSE-</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">21 derived countries. It does not mention if it is not a bulk</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 import or if it is raw materials rather than finished</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">23 materials.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">24 Further, we know that it is strongly recommended</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">25 but not actually prohibited in the language here. So I have</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">233</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 not taken the assurances from that import alert that Dr. 2 Moore was trying to convey to us. 3 So, in sum, dietary supplements sold in the United 4 States often contain ruminant tissues from undisclosed 5 sources. Personally, I am rather squeamish and I don't 6 think I would be eating prostate or testicle or pituitary, 7 but I am also a little bit wary of consuming products with 8 those glands, not just out of personal repugnance but simply 9 out of a health concern.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">10 So my question to the advisory committee is this;</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">11 is my caution reasonable and, if it is, should we take</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">12 further efforts to inform, or even protect, the American</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">13 public from such exposure.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><span dir="ltr" style="outline: none;">14 I was curious about Dr.</span> Moore's remarks. I sensed</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">15 two messages. One was the initial reassurance that FDA has</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">16 the regulatory authority but then I also learned that it is</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 the manufacturer's responsibility to provide those 18 assurances, that the FDA doesn't actually inspect.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">19 I think that the FDA commissioners from Harvey</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">20 Wylie to David Kessler would say that that track record has</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">21 proven itself.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">22 Thank you very much.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">23 [Applause.]</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">24 DR. BROWN: Thanks, Dr. Norton. 25 Committee Discussion snip...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">17 But I think that we could exhibit some quite 18 reasonable concern about blood donors who are taking dietary 19 supplements that contain a certain amount of unspecified- 20 origin brain, brain-related, brain and pituitary material. 21 If they have done this for more than a sniff or something 22 like that, then, perhaps, they should be deferred as blood 23 donors. 24 That is probably worse than spending six months in 25 the U.K. 1/19/01 3681t2.rtf(845) page 501 <span dir="ltr" style="outline: none;">http://www.fda.gov/ohrms/dockets/ac/cber01.htm</span></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Advisory Committees: CBER 2001 Meeting Documents</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">see actual paper;</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div></div><a href="http://web.archive.org/web/20030830045538/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20030830045538/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Given the science and the information presented, and given the comprehensive array of Natraflex quality control and chain-of-custody procedures, we believe that you can be confident, the our velvet-antler supplements are safe.</div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://web.archive.org/web/20090120095558/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090120095558/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://web.archive.org/web/20090120094525/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_01.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090120094525/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_01.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="http://web.archive.org/web/20010805202621/http://www.fda.gov/bbs/topics/ANSWERS/2001/ANS01070.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20010805202621/http://www.fda.gov/bbs/topics/ANSWERS/2001/ANS01070.html</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="outline: none;">Date: Sun, 12 Jan 2003 12:56:44 -0600</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Sender: Bovine Spongiform Encephalopathy</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From: "Terry S. Singeltary Sr."</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Subject: Re: USA ruminant-to-ruminant feed ban warning letters ??? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">With these known facts about nutritional supplements, I think it imperative to include this potential route and source of TSE and warning in this article. Why was it not included? I lost my mother to hvCJD DOD 12/14/97, and I probably will not live long enough to know the route and source of her TSE. Exactly one year previously, to the day 12/14/96, my neighbor also lost his mother to sporadic CJD. Both of these cases were confirmed. but my neighbor's mother had been taking a nutritional supplement called IPLEX, made by Standard Process Co. Here is a listing of potentially TSE-tainted tissues: vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. The CDC came and took the pills, a few years later, I spoke with the late Dr. Gibbs and NIH/CDC and he told me that those particular pills did not show any infectivity with the testing techniques to date, but he also told me - </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1. That the testing techniques at that time may not be sufficient to pick up any 'low-level' infectivity. </div><div style="outline: none;">(so, if accumulation plays into this, this could play a big part). </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">2. She had been taking these type pills for years, could have been another batch. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">There have been other people die of CJD that were taking these type nutritional supplements. So, my point being, I believe this to warrant a potential risk factor, one not to be ignored, especially in the USA where there are many known TSEs, and where there are many unknowns due to the lack of sufficient TSE testing in USA cattle, and especially since the new findings of Collinge et al, where BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">I believe these findings to be of substantial importance: </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">DEPARTMENT OF HEALTH AND HUMAN SERVICES FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Friday, January 19, 2001 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Holiday Inn Bethesda Versailles I and II 8120 Wisconsin Avenue Bethesda, Maryland 2 PARTICIPANTS Paul W. Brown, M.D., Chairperson William Freas, Ph.D., Executive Secretary VOTING MEMBERS Ermias D. Belay, M.D. David C. Bolton, Ph.D. Donald S. Burke, M.D. Dean O. Cliver, Ph.D. Bruce M. Ewenstein, M.D., Ph.D. Peter G. Lurie, M.D. Pedro Piccardo, M.D. Stanley B. Prusiner, M.D. Raymond P. Roos, M.D. Elizabeth S. Williams, D.V.M., Ph.D. VOTING CONSULTANTS Linda A. Detwiler, D.V.M. David Gaylor, Ph.D. Paul R. McCurdy, M.D. Kenrad E. Nelson, M.D. NONVOTING CONSULTANT Susan Leitman, M.D. GUESTS Richard Davey, M.D. Louis Katz, M.D. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">snip... page 501 253 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">1 DR. BOLTON: I have an additional question about 2 that. What is the assurance that additional locally sourced 3 tracheas are not added into that manufacturing process, thus 4 boosting the yield, if you will, but being returned to the 5 U.S. as being produced from U.S.-sourced raw material? 6 DR. McCURDY: Are there data to indicate how many 7 grams, or whatever, of infected brain are likely to infect 8 an organism, either animal or man, when taken orally? 9 DR. BROWN: If I am not mistaken, and I can be 10 corrected, I think a half a gram is enough in a cow, orally; 11 in other words, one good dietary-supplement pill. 12 DR. McCURDY: What I am driving at is the question 13 we are asked is really not do we wish to regulate these 14 things coming in. I think the statements about difficulties 15 in regulating things in the future or near future for new 16 regulations were probably accurate. 17 But I think that we could exhibit some quite 18 reasonable concern about blood donors who are taking dietary 19 supplements that contain a certain amount of unspecified- 20 origin brain, brain-related, brain and pituitary material. 21 If they have done this for more than a sniff or something 22 like that, then, perhaps, they should be deferred as blood 23 donors. 24 That is probably worse than spending six months in 25 the U.K. 1/19/01 3681t2.rtf(845) page 501 http://www.fda.gov/ohrms/dockets/ac/cber01.htm There has been a Nutritional Supplement mad cow warning letter circulating around since about 1990. Every year they issue the same letter to the manufactures asking them to please be sure they source their products from BSE-FREE countries. but we all know, the statement BSE-FREE, is a joke, especially in the USA. I sat in on the 50 state emergency BSE conference call, (uninvited guest) and I know for a fact the so-called 'pharmaceutical grade' bovine source herds, bovines that were to never be fed ruminant materials, the USA cannot for certain say that indeed these cattle have never ingested ruminant feed, in fact, some probably had. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Date: Tue, 9 Jan 2001 16:49:00 -0800 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From: "Terry S. Singeltary Sr." < flounder@wt.net > </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Reply-To: Bovine Spongiform Encephalopathy < BSE-L@uni-karlsruhe.de > </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To: BSE-L@uni-karlsruhe.de Bovine Spongiform Encephalopathy < BSE-L@UNI-KARLSRUHE.DE > </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Greetings List Members, I was lucky enough to sit in on this BSE conference call today and even managed to ask a question. that is when the trouble started. I submitted a version of my notes to Sandra Blakeslee of the New York Times, whom seemed very upset, and rightly so. "They tell me it is a closed meeting and they will release whatever information they deem fit. Rather infuriating." And I would have been doing just fine, until I asked my question. I was surprised my time to ask a question came so quickly. (understand, these are taken from my notes for now. the spelling of names and such could be off.) </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[host Richard Barns] And now a question from Terry S. Singeltary of CJD Watch. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[TSS] Yes, Thank You. U.S. cattle - what kind of guarantee can you give for serum or tissue donor herds? [no answer, you could hear in the background, mumbling and "We can't. Have him ask the question again."] </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[host Richard] Could you repeat the question? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[TSS] U.S. cattle..what kind of guarantee can you give for serum or tissue donor herds? [not sure whom ask this] What group are you with? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[not sure who is speaking] Could you please disconnect Mr. Singeltary </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[TSS] You are not going to answer my question? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[not sure whom speaking] NO </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">From this point, I was still connected, got to listen and tape the whole conference. at one point someone came on, a woman, and ask again; [unknown woman] What group are you with? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">[TSS] CJD Watch and my Mom died from hvCJD We are trying to tract down CJD and other human TSE's world wide. I was invited to sit in on this from someone inside the USDA/APHIS and that is why I am here. Do you intend on banning me from this conference now? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">At this point the conference was turned back up, and I got to finish listening. They never answered or even addressed my one question, or even addressed the issue. BUT, I will try and give you a run-down for now, of the conference. IF i were another Country, I would take heed to my notes, BUT PLEASE do not depend on them. ask for transcript from: RBARNS@ORA.FDA.GOV 301-827-6906 He would be glad to give you one ;-) Rockville Maryland, Richard Barns Host </div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">U.S. 50 State Emergency BSE Conference Call <span dir="ltr" style="outline: none;"><span dir="ltr" style="outline: none;">2001</span></span></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"></a></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></span></p></div></div></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Chronic Wasting Disease CWD Maine</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">How can the average person help prevent CWD?</span></p><ol class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984ol1" style="outline: none;"><li class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984li1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Be careful with commercial feeds.</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> In theory, prions from CWD-infected deer could be present in commercial deer and elk foods if they were formulated with rendering products containing CWD-infected meat and bone meal (MBM). In 1997, the U.S. FDA banned the use of ruminant (deer, cattle, sheep, goat) MBM from commercial feeds for ruminants. Assuming 100% FDA compliance, common commercial feeds used to supplement the diets of captive/farmed or wild cervids would now be CWD-free. However, we don’t know if MBM from CWD-infected deer or elk was ever incorporated into commercial ruminant feeds distributed in Maine prior to 1997, nor do we know if commercial feeds formulated for non-ruminants (horse, swine, poultry, dog, and cat) sometimes contain MBM from CWD-infected deer or elk. To safely feed cervids, use only commercially available products formulated specifically for ruminants, or use supplement-free whole grains like oats.</span></li></ol><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><a href="https://www.maine.gov/ifw/fish-wildlife/wildlife/living-with-wildlife/diseases/chronic-wasting-disease.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.maine.gov/ifw/fish-wildlife/wildlife/living-with-wildlife/diseases/chronic-wasting-disease.html</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">“In 1997, the U.S. FDA banned the use of ruminant (deer, cattle, sheep, goat) MBM from commercial feeds for ruminants.”</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Greetings Maine Wildlife Officials on CWD,</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">I was reading over your page of information on cwd, and the above information is not correct exactly. It was just a voluntary ban for cervid.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Title:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> Scrapie as the potential origin of chronic wasting disease in white-tailed deer </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Author</span></p><table cellpadding="0" cellspacing="0" class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984t1" style="border-collapse: collapse; color: black; outline: none;"><tbody style="outline: none;"><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none;"></span><br style="outline: none;" /></p></td><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;">LAMBERT, ZOE - Oak Ridge Institute For Science And Education (ORISE)</span></p></td></tr><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none;"></span><br style="outline: none;" /></p></td><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;">WEST GREENLEE, HEATHER - Iowa State University</span></p></td></tr><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none;"></span><br style="outline: none;" /></p></td><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=57305" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">Bian, Jifeng</a></span></p></td></tr><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none;"></span><br style="outline: none;" /></p></td><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=55914" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">Cassmann, Eric</a></span></p></td></tr><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none;"></span><br style="outline: none;" /></p></td><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=44813" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">Greenlee, Justin</a></span></p></td></tr></tbody></table><table cellpadding="0" cellspacing="0" class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984t1" style="border-collapse: collapse; color: black; outline: none;"><tbody style="outline: none;"><tr style="outline: none;"><td class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none; padding: 1px 5px;" valign="top"><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Submitted to:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> Chronic Wasting Disease Symposium Proceedings </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Publication Type:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> Abstract Only </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Publication Acceptance Date:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> 3/1/2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Publication Date:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> 5/30/2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Citation:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> Lambert, Z.J., West Greenlee, H.M., Bian, J., Cassmann, E.D., Greenlee, J.J. 2023. Scrapie as the potential origin of chronic wasting disease in white-tailed deer (abstract). Chronic Wasting Disease Symposium Proceedings. 4th International Chronic Wasting Disease Symposium, May 30-June 3, 2023, Denver, Colorado.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Interpretive Summary:</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none;">Technical Abstract:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none;"> White-tailed deer (WTD) are susceptible to the scrapie agent from sheep after oronasal inoculation. However, results from western blotting these brainstems and lymph nodes are difficult to differentiate from WTD infected with chronic wasting disease (CWD). Tissues were examined via enzyme immunoassay (IDEXX), western blot, immunohistochemistry, and bioassay in cervidized mice (Tg12) in order to assess tissue phenotypes upon subsequent passage of the scrapie agent in WTD. All WTD were euthanized and necropsied following the development of clinical disease and were positive for abnormal prion protein by enzyme immunoassay. Western blotting of retinas from all WTD (second pass) resulted in a similar molecular profile as the retinas of WTD that were inoculated with the agent of scrapie from sheep (first pass). Immunohistochemical staining also was similar between inoculation groups and the initial passage from sheep, but different from WTD inoculated with the agent of CWD. Following bioassays in cervidized mice, all incubation periods were over 300 days, substantially longer than the approximately 200-day incubation period typical with CWD isolates. Based upon analysis of retinal tissues, it is possible to differentiate the agents of scrapie and CWD in WTD by both western blot and immunohistochemistry. Bioassay in cervidized mice further supports this based on incubation periods of WTD-scrapie being approximately twice that of WTD CWD</span></p></td></tr></tbody></table><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Submitted to:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> Chronic Wasting Disease Symposium Proceedings </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Publication Type:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> Abstract Only </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Publication Acceptance Date:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> 3/1/2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Publication Date:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> 5/30/2023 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Citation:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> Lambert, Z.J., West Greenlee, H.M., Bian, J., Cassmann, E.D., Greenlee, J.J. 2023. Scrapie as the potential origin of chronic wasting disease in white-tailed deer (abstract). Chronic Wasting Disease Symposium Proceedings. 4th International Chronic Wasting Disease Symposium, May 30-June 3, 2023, Denver, Colorado.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Interpretive Summary:</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;">Technical Abstract:</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> White-tailed deer (WTD) are susceptible to the scrapie agent from sheep after oronasal inoculation. However, results from western blotting these brainstems and lymph nodes are difficult to differentiate from WTD infected with chronic wasting disease (CWD). Tissues were examined via enzyme immunoassay (IDEXX), western blot, immunohistochemistry, and bioassay in cervidized mice (Tg12) in order to assess tissue phenotypes upon subsequent passage of the scrapie agent in WTD. All WTD were euthanized and necropsied following the development of clinical disease and were positive for abnormal prion protein by enzyme immunoassay. Western blotting of retinas from all WTD (second pass) resulted in a similar molecular profile as the retinas of WTD that were inoculated with the agent of scrapie from sheep (first pass). Immunohistochemical staining also was similar between inoculation groups and the initial passage from sheep, but different from WTD inoculated with the agent of CWD. Following bioassays in cervidized mice, all incubation periods were over 300 days, substantially longer than the approximately 200-day incubation period typical with CWD isolates. Based upon analysis of retinal tissues, it is possible to differentiate the agents of scrapie and CWD in WTD by both western blot and immunohistochemistry. Bioassay in cervidized mice further supports this based on incubation periods of WTD-scrapie being approximately twice that of WTD CWD.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"></a></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">https://www.nature.com/articles/srep11573</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">CWD TO HUMANS, What If, has it already happened?</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">31 TAC §§65.82, 65.85, 65.88</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Abstract</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Our results show positive prion detection in all products. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***>To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Our results show positive prion detection in all products. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">=====</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">=====</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice were inoculated into </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice were used for second passage in bank voles.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Unprecedented in human prion disease, feces of CWD-inoculated </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">tg650</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> with fecal homogenates.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p3" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s3" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none;">U of M expert warns of increasing likelihood of CWD transmission to humans</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.mprnews.org/people/cathy-wurzer" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">Cathy Wurzer</a></span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> and <a href="https://www.mprnews.org/people/gretchen-brown" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none;">Gretchen Brown</span></a>June 5, 2023 </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">1:30 PM</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Minnesota scientists have watched chronic wasting disease (CWD) — a fatal, neurological illness — kill deer and elk.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Now, they’re studying its potential to jump to humans.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The University of Minnesota’s <a href="https://www.cidrap.umn.edu/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none;">Center for Infectious Disease Research and Policy</span></a> has received more than $1.5 million in state money to start prepping for the possibility of CWD spreading to cows, pigs and possibly humans.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">He said transmission to humans has not yet been confirmed, but research suggests it is <a href="https://vet.ucalgary.ca/news/chronic-wasting-disease-may-transmit-humans-research-finds" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none;">increasingly likely</span></a> — especially as the disease continues to spread among deer and elk.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">“None of us want to believe this could happen,” he told MPR News host Cathy Wurzer. “But you know, as much as you hope it isn't going to happen, hope is not a strategy.”</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Current testing can be done <a href="https://www.mprnews.org/story/2023/03/16/state-agencies-not-sold-on-new-chronic-wasting-disease-test-option?gclid=Cj0KCQjwj_ajBhCqARIsAA37s0y2AZgeeWMW277iYJw23uHHH2jDJpN2ZbcYSOjrN7IurNYKbPaLygsaAnSGEALw_wcB" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none;">only if animals die or are killed</span></a>, and lymph nodes or brain matter is removed for testing to verify the disease.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">That means captive deer often aren’t tested until they die or show symptoms of the disease, and that’s often too late to stop the spread of the disease.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">And there aren’t yet adequate tests for humans, Osterholm said — let alone protocols in place if a human were to test positive for the disease.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Michael Osterholm, Ph.D. is a world-renowned epidemiologist who heads the center.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.mprnews.org/episode/2023/06/05/chronic-wasting-disease-in-humans-us-osterholm-prepares-for-whatif?" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.mprnews.org/episode/2023/06/05/chronic-wasting-disease-in-humans-us-osterholm-prepares-for-whatif?</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">PLoS One. 2020; 15(8): e0237410. Published online 2020 </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">Aug 20.</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> doi: 10.1371/journal.pone.0237410 PMCID: PMC7446902 PMID: </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">32817706</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Abstract </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The results demonstrate: (a) that the minimum CWD oral infectious dose is vastly lower than historical studies used to establish infection; (b) that a direct relationship exists between dose and incubation time to first prion replication detection in tonsils, irrespective of genotype; (c) that a difference was not discernible between brain vs. saliva source prions in ability to establish infection or in resultant disease course; and (d) that the CWD infection process appears to conform more to a threshold dose than an accumulative dose dynamic. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** now, let’s see what the authors said way back about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans” </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ??? </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Date: September 30, 2002 at 7:06 am PST </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">From: "Belay, Ermias" </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Sent: Monday, September 30, 2002 9:22 AM </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">404-639-3091</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">). </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Ermias Belay, M.D. Centers for Disease Control and Prevention </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">-----Original Message----- From: </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Sent: Sunday, September 29, 2002 10:15 AM To: </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">rr26k@nih.gov</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">; </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">rrace@niaid.nih.gov</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">; </span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;">ebb8@CDC.GOV</span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://pubmed.ncbi.nlm.nih.gov/11594928/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/11594928/</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">> However, to date, no CWD infections have been reported in people. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way; </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">sporadic = 54,983 hits </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">spontaneous = 325,650 hits </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></span><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"> </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">> However, to date, no CWD infections have been reported in people.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a></span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">From: Steve Dealler </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">To: BSE-L@ References: </span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Dear Terry,</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Steve Dealler </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">=============== </span></div><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Table 9 presents the results of an analysis of these data.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">snip...see full report ;</span></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydpf4564930yiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></span></p><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><div dir="ltr" style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;">2004 video</span></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;"><br style="outline: none;" /></span></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;">Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD </span><br style="outline: none;" /></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;"><br style="outline: none;" /></span></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium; outline: none;">????: CBCnews</strong><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;"><br style="outline: none;" /></span></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;"><br style="outline: none;" /></span></span></div><div style="font-family: arial; font-size: 13.3333px; outline: none;"><span style="background-color: whitesmoke; outline: none;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: none;"><a href="https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html</a></span></span></div></div><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">1997 nvCJD video</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><a href="https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html</a><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="outline: none;"><div style="outline: none;">Successful transmission of the chronic wasting disease (CWD) agent to white-tailed deer by intravenous blood transfusion </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Najiba Mammadova a b, Eric Cassmann a b, Justin J. Greenlee a Show more Add to Mendeley Share Cite <a href="https://doi.org/10.1016/j.rvsc.2020.10.009" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://doi.org/10.1016/j.rvsc.2020.10.009</a> </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Get rights and content Under a Creative Commons license open access </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Highlights </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">• The chronic wasting disease (CWD) agent efficiently transmits between white-tailed deer.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">• Blood from CWD infected deer contains infectious prions.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">• A single intravenous blood transfusion resulted in CWD transmission with an incubation of 25.6 months for the GG96 recipient.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">• The GS96 recipient had a longer incubation of 43.6 months.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Abstract Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSEs) that affects free-ranging and captive cervid species. The infectious agent of CWD may be transmitted from ingestion of prions shed in bodily fluids (e.g. feces, urine, saliva, placenta tissue) of infected animals, contaminated pastures, and/or decomposing carcasses from dead animals. Studies have also demonstrated prion infectivity in whole blood or blood fractions of CWD infected animals. To determine if CWD-infected blood contained sufficient levels of prion infectivity to cause disease, recipient deer were inoculated intravenously (IV) with blood derived from a CWD-infected white-tailed deer. We found that the CWD agent can be successfully transmitted to white-tailed deer by a single intravenous blood transfusion. The incubation period was associated with recipient prion protein genotype at codon 96 with the GG96 recipient incubating for 25.6 months and the GS96 recipient incubating for 43.6 months. This study complements and supports an earlier finding that CWD can be transmitted to deer by intravenous blood transfusion from white-tailed deer with CWD.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">SNIP...</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">While a previous and larger study showed similar results, we determined that only 100 mL of CWD-infected blood (~2.5 times less than previously shown in (Mathiason et al., 2010)) contained sufficient levels of prion infectivity to cause disease. The identification of blood-borne transmission of the CWD agent is important in reinforcing the risk of exposure to CWD via blood as well as the possibility of hematogenous transmission of the CWD agent through insect vector. </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://www.sciencedirect.com/science/article/pii/S003452882031047X/pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.sciencedirect.com/science/article/pii/S003452882031047X/pdf</a><br style="outline: none;" /></div></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">PLoS One. 2020; 15(8): e0237410. Published online 2020 Aug 20. doi: 10.1371/journal.pone.0237410 PMCID: PMC7446902 PMID: 32817706 </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">See PLoS One. 2021 June 10; 16(6): e0253356. Associated Data Data Availability Statement </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">Abstract </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">The minimum infectious dose required to induce CWD infection in cervids remains unknown, as does whether peripherally shed prions and/or multiple low dose exposures are important factors in CWD transmission. With the goal of better understand CWD infection in nature, we studied oral exposures of deer to very low doses of CWD prions and also examined whether the frequency of exposure or prion source may influence infection and pathogenesis. We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.</div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">snip...</div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">In conclusion, we have attempted to model and better understand CWD infection relative to natural exposure. The results demonstrate: </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">(a) that the minimum CWD oral infectious dose is vastly lower than historical studies used to establish infection; </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">(b) that a direct relationship exists between dose and incubation time to first prion replication detection in tonsils, irrespective of genotype; </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">(c) that a difference was not discernible between brain vs. saliva source prions in ability to establish infection or in resultant disease course; and </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;">(d) that the CWD infection process appears to conform more to a threshold dose than an accumulative dose dynamic. </div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><br style="outline: none;" /></div><div style="background-color: whitesmoke; font-family: arial; font-size: 16px; outline: none; text-align: justify;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/</a></div></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">GET THOSE DAMN DEER TESTED FOR CWD TSE PRION BEFORE TAKING THEM HOME AND FEEDING TO YOUR FAMILY AND FRIENDS !!!</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="outline: none;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">FRIDAY, NOVEMBER 17, 2023 </div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">TAHC Chronic Wasting Disease Detected in Cherokee County Deer Breeding Facility 575+ Confirmed CASES TO DATE!<br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><a href="https://chronic-wasting-disease.blogspot.com/2023/11/tahc-chronic-wasting-disease-detected.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/11/tahc-chronic-wasting-disease-detected.html</a></div></div><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">BSE TSE Prion aka mad cow disease and Scrapie of sheep and goats</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div style="outline: none;"><div style="outline: none;"><div style="color: #29303b; font-family: arial; font-size: 16px; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014) <br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="color: #29303b; font-family: arial; font-size: 16px; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine). </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAF COD_UO-P </div></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''</div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">=====</div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Transmission of Idiopathic human prion disease CJD MM1 to small ruminant mouse models (Tg338 and Tg501). </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"> Enric Vidal1,2, Samanta Giler1,2, Montse Ordóñez1,2, Hasier Eraña3,4, Jorge M. Charco3,4, Guillermo Cantero1,2, Juan C. Espinosa5 , Juan M. Torres5 , Vincent Béringue6 , Martí Pumarola7 and Joaquín Castilla3,8,9 1 Unitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia. 2 IRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia. 3 Center for Cooperative Research in Biosciences (CIC BioGUNE), Basque Research and Technology Alliance (BRTA), Prion Research Lab, Derio, Spain. 4 ATLAS Molecular Pharma S. L. Derio (Bizkaia), Spain. 5 Centro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid 28130, Spain. 6 Molecular Virology and Immunology, Institut National de la Recherche Agronomique (INRA), Université Paris-Saclay, Jouy-en-Josas, France 7 Unitat de Patologia Murina i Comparada, Departament de Medicina i Cirurgia Animals, Facultat de Veterinària, Campus de UAB, 08193 Bellaterra, Barcelona, Catalonia. 8 IKERBASQUE, Basque Foundation for Science, Bilbao 48013, Bizkaia, Spain. 9 Centro de Investigación Biomédica en Red de Enfermedades infecciosas (CIBERINFEC), Carlos III National Health Institute, Madrid, Spain. Corresponding author: enric.vidal@irta.cat Phone: 934674040 (1784) </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Aims: About 90% of Creutzfeldt-Jakob disease cases are classified as sporadic (sCJD), that is, occur infrequently, randomly and without a known cause. It is a fatal neurodegenerative disease with an incidence of 1-1.5 cases per million per year. Epidemiological studies have been so far unable to establish a causal relationship between sCJD and prion diseases in animals. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">The zoonotic potential of sheep scrapie was demonstrated in 2014 (Cassard et al., Nature Communications) through inoculation of transgenic mice overexpressing the human prion protein with scrapie isolates. The resulting prion disease was indistinguishable from that occurring after sCJD inoculation in the same model and, while these results do not demonstrate that sCJD is caused by scrapie prions, they do show that the transmission barrier between ovine and human prions is not absolute. Our aim is to further assess this zoonotic risk. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Materials and methods: we have prepared inocula from 3 sCJD cases (MM1, MV2 and VV2) and 2 VPSPr cases (MM and MV) to verify if it is possible to recover the scrapie phenotype upon inoculation in Tg338 and Tg501 ovinized mouse models. Additionally, two different inocula gCJD (E200K) and GSS (A117V) have been also included in the bioassays as controls for classical and atypical genetic human prions, respectively.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Results: No evidence of transmission was found on a first passage in Tg338 nor Tg501 ovinized mice, but on second passage, 4/10 Tg338 mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12 Tg501 mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Funded by: MINECO grant number AGL2017-88535-P and PID2021-1222010B-C22 and by Interreg POCTEFA grant number EFA148/16 (RedPRION)</div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">''but on second passage, 4/10 Tg338 mice succumbed to CJDMM1 (40% attack rate after 645 dpi) and 1/12 Tg501 mice (519dpi, 10 still alive). The remaining 2nd passages are still ongoing. Conclusions: In this poster, the neuropathological features of the resulting strain are discussed.''<br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div dir="ltr" style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Title: Transmission of scrapie prions to primate after an extended silent incubation period)</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***thus questioning the origin of human sporadic cases. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">=============== </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***thus questioning the origin of human sporadic cases*** </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">=============== </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">============== </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">PRION 2015 CONFERENCE</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">PRION 2016 TOKYO</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Saturday, April 23, 2016</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 1933-690X </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Taylor & Francis</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">FRIDAY, JANUARY 20, 2023 </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">EPIDEMIOLOGY OF SCRAPIE IN THE UNITED STATES </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://scrapie-usa.blogspot.com/2023/01/epidemiology-of-scrapie-in-united-states.html" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://scrapie-usa.blogspot.com/2023/01/epidemiology-of-scrapie-in-united-states.html</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">WEDNESDAY, FEBRUARY 03, 2021 </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Scrapie TSE Prion United States of America a Review February 2021 Singeltary et al</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://scrapie-usa.blogspot.com/2021/02/scrapie-tse-prion-united-states-of.html" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://scrapie-usa.blogspot.com/2021/02/scrapie-tse-prion-united-states-of.html</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">WEDNESDAY, MARCH 16, 2022 </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">SHEEP BY-PRODUCTS AND WHAT ABOUT Scrapie TSE PrP and Potential Zoonosis? </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html</a></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">WEDNESDAY, DECEMBER 8, 2021 </div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;">Importation of Sheep, Goats, and Certain Other Ruminants AGENCY: Animal APHIA, USDA, FINAL RULE [Docket No. APHIS–2009–0095] RIN 0579–AD10</div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><br style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" /></div><div style="outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;"><a href="https://animalhealthreportpriontse.blogspot.com/2021/12/importation-of-sheep-goats-and-certain.html" rel="nofollow" style="color: #196ad4; outline-color: currentcolor; outline-style: initial; outline-width: initial !important; outline: currentcolor;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2021/12/importation-of-sheep-goats-and-certain.html</a></div></div></div></div></div></div></div><div style="outline: none;"><br style="outline: none;" /></div></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">***> A MUST READ, GLOBAL UPDATE TSE PRION <***</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">***> TYPICAL AND ATYPICAL BSE, SCRAPIE, AND CWD, ZOONOSIS FOR HUMANS AS SPORADIC CJD <***</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">THURSDAY, NOVEMBER 9, 2023 </div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">EFSA Annual Report of the Scientific Network on BSE-TSE 2023<br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><a href="https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://efsaopinionbseanimalprotein.blogspot.com/2023/11/efsa-annual-report-of-scientific.html</a></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Professor John Collinge on tackling prion diseases sCJD accounts for around 1 in 5000 deaths worldwide<br style="outline: none;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"><br style="outline: none;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</span></div><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Professor John Collinge on tackling prion diseases</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Professor John Collinge is Director of the MRC Prion Unit and also directs the NHS National Prion Clinic at the adjacent National Hospital for Neurology and Neurosurgery.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">John Collinge What are prions, why are they important, and how might they help us develop treatments for neurodegenerative conditions like dementia?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Prions are lethal pathogens that cause neurodegenerative diseases of humans and other mammals.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide. In sharp distinction to all other infectious agents, prions lack their own DNA or RNA genome and consist of polymers of a misfolded form of a normal cellular protein (the prion protein or PrP) which form amyloid fibrils.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">These fibres grow by addition of PrP molecules at their ends and they eventually fragment producing more prion particles which continue this process and spread throughout the brain. The final proof of the once controversial “protein-only hypothesis” of prions came with the determination of the structure of infectious prions at near atomic resolution by cryogenic electron microscopy by ourselves and US colleagues in the last few years.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The normal cellular prion proteins are very similar between different species of mammals and therefore a prion infection from one species can sometimes infect another species. This is what happened with the prion disease of cattle, bovine spongiform encephalopathy (BSE) in the 1990’s which caused a new human prion disease known as variant Creutzfeldt-Jakob disease (vCJD) and led to the BSE crisis in the UK, EU and other countries.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">While human prion diseases are thankfully rare, there are common prion diseases of other species, for example scrapie in sheep and goats worldwide and chronic wasting disease in deer in North America. While prions were first thought to be unique to these rare neurological diseases, it became clear that the molecular process was of far wider relevance with for example the recognition of several different proteins in yeast that could form prions.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Most importantly with respect to neurodegeneration and dementia in humans, it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases. In Alzheimer’s disease (AD) for example, two proteins in the brain, amyloid-beta and tau can form self-propagating assemblies which spread in the brain. Indeed, we reported in two articles in Nature that the amyloid-beta pathology seen in AD can be transmissible between humans in rare circumstances causing the newly recognised condition iatrogenic cerebral amyloid angiopathy.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine. Many years ago, we demonstrated that targeting the production of the normal cellular prion protein completely halted the progression of neurodegeneration (and indeed even reversed early pathological changes) in laboratory mice. This work has underpinned multiple efforts to develop rational treatments for prion and other neurodegenerative diseases.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">What first attracted you to the area of prion diseases?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I first became involved in this field while working as a graduate student applying early molecular genetic methods to study neuropsychiatric diseases and was involved in the first description of mutations in the prion protein gene in the late 1980s in what are now known as the inherited prion diseases.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">As it was already known that brain tissue from patients who died from some of these genetic conditions could transmit disease when inoculated into laboratory animals, it seemed to me highly likely that some version of the then intensely controversial “protein-only hypothesis” was likely to be correct: this had major implications in pathobiology.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I went on to show that being heterozygous for a common human prion protein polymorphism had a profound effect on susceptibility to CJD; I considered this entirely consistent with a protein-only agent and this led to further work studying the genetics of prion disease.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">It seemed to me at the time that these early genetic insights, albeit in a rare disease, provided a powerful way in to study the fundamental basis of neurodegeneration. Of course, the evolving concerns about BSE in the early 1990’s also focussed my mind on the specific public and animal health risks posed by prions.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">You led the UK’s first clinical trial in CJD, the largest yet conducted internationally. Can you tell us about this? </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I was asked in 1997 by Medical Research Council (MRC) at the request of UK Government to establish and lead an MRC Unit to focus on understanding prion diseases and to ultimately develop treatments for them.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">At the time it was unknown how many people would develop vCJD following the widespread dietary exposure of the UK population to BSE prions and the possibility that this may eventually affect hundreds of thousands could not then be excluded.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">An early proposal (by Dr Prusiner at UCSF) for a treatment for CJD was the anti-malarial drug quinacrine based on early work in prion-infected cell cultures. We were asked by the Chief Medical Officer to establish a clinical trial and did so in collaboration with the MRC Clinical Trials Unit also based at UCL.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">While the MRC PRION-1 trial, as is was called, did not show any benefit of quinacrine, we did learn a great deal about how best to conduct a clinical trial in CJD in conjunction with patients and families affected by these terrible conditions.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">This lead on to the formation of the National Prion Monitoring Cohort (NPMC) to study the natural history of prion diseases and to develop better clinical scales and biomarkers, and earlier diagnosis, to facilitate future clinical trials. In particular, we reasoned that having a large longitudinal data set would allow us to conduct adequately powered efficacy trials by comparison of treated patients with historical controls rather that using a more classical placebo-controlled study which was understandably unacceptable to patients and their families given the rapid and invariably fatal progression of these diseases.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The NPMC has been extremely successful with the strong support of the patient community and has recruited over 1100 patients to date, by far the largest dataset worldwide, and has enabled development and validation of multiple clinical scales and blood and CSF biomarkers.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">What in your opinion have been some of the most important findings of your research to date?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Our early work established and characterised the inherited prion diseases and genetic susceptibility to acquired and sporadic prion disease, and pioneered diagnostic and presymptomatic genetic testing of neurodegenerative disease.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Many further genetic advances followed. Prions exist in multiple strain types and we developed molecular strain typing of prions which we applied in 1996 to first demonstrate that vCJD was caused by the same prion strain as cattle BSE, a finding of critical public and animal health significance at the time.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">We characterised the pathogenesis of vCJD to inform public health risk assessments, developed the first blood test for vCJD and effective means to prion sterilise surgical instruments. We proposed the now widely accepted “conformational selection hypothesis” to explain the relationship between prion strains and intermammalian transmission barriers and proposed that prion strains constitute a “cloud” under host selection rather than a molecular clone.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Importantly, we described subclinical prion infections in which animals lived a normal lifespan despite harbouring high levels of prions and went on to study the kinetics of prion propagation in vivo and showed that propagation and neurotoxicity occur in two distinct mechanistic phases with pathology only developing after prion levels had plateaued in the brain.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">We subsequently confirmed that prions themselves are not directly neurotoxic. These insights may be fundamental to understanding other diseases involving propagation and spread of assemblies of misfolded proteins, notably amyloid-beta and tau in AD.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Our discovery of human transmission of amyloid-beta pathology, mentioned above, in individuals treated many years earlier in childhood with human cadaver-derived pituitary growth hormone (c-hGH) accidentally contaminated with amyloid-beta seeds (prions) has wide implications for understanding, preventing and treating neurodegenerative diseases.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health. Iatrogenic AD is likely to be recognised in the cohort of c-hGH recipients as they age further. Our demonstration that reducing prion expression during neuroinvasive prion disease in laboratory mice prevented onset, and reverses early pathology, produced a proof of principle of therapeutically targeting prion protein.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">This led to our development of a biopharmaceutical which we have used to treat CJD. Recently, we have described the elusive structural basis of prion strain diversity: how prions can encode information in a non-Mendelian manner by determination of near atomic resolution structures of multiple prion stains by cryogenic electron microscopy. </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">In addition, we are proud of our long term field studies on the epidemic human prion disease kuru in the Eastern Highlands of Province of Papua New Guinea (PNG), in collaboration with the PNG Institute for Medical Research and the affected communities, which led to major insights including establishing the range of possible incubation periods of human prion infections (documenting cases with incubations over 50 years) and discovery of a novel prion protein variant selected by the epidemic which we demonstrated provides complete protection against prion infection and disease and the molecular structural basis of which we have recently characterised. </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">To what extent do you think we are entering a new era when it comes to developing drugs that could be used to prevent, or even reverse, neurodegenerative diseases?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Thankfully we are entering a time when disease-modifying treatments for neurodegenerative diseases are becoming feasible and indeed first-generation agents have arrived, but we cannot yet prevent, halt or reverse neurodegeneration.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Our own work validating cellular prion protein as a therapeutic target led us to develop a humanised monoclonal antibody with high affinity for cellular PrP and this has been used to treat six patients with CJD at UCLH. We consider the encouraging results justify a formal clinical trial and are seeking funding support for this at present.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Our therapeutic strategy has been to target normal cellular PrP itself, the substrate for prion propagation, and not the disease-related assemblies of misfolded PrP that accumulate during disease. We reasoned, given the diversity of these species, that drugs binding prions themselves would lead to the rapid development of resistance and indeed this has been shown to be the case with drugs developed elsewhere.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There may be important lessons here for other neurodegenerative diseases. For example, this may be critical in determining whether monoclonal antibody drugs targeting amyloid-beta fibrils or other assemblies, which also exist as structural polymorphs, have a sustained therapeutic effect or result in strain selection and evolution of resistant sub-strains as in prion diseases.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">A number of pharmaceutical and biotech companies are however developing gene targeting methods, conceptually analogous to those we demonstrated many years ago block prion pathogenesis, to reduce expression of proteins implicated in various neurodegenerative diseases. Given the complexity and diversity of AD, in which multiple proteinopathies are involved, it is likely that effective treatments are going to require a cocktail of drugs hitting multiple targets.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Another key consideration is the importance of accurate diagnosis and early treatment, not only for the obvious need to intervene before irreversible brain cell loss has occurred, but because at the stage where significant cell death (with release of toxic materials) is occurring, these secondary non-specific neurodegenerative processes may dominate and be unresponsive to the specific targeted therapies. The ultimate aim must be to identify these pathogenic processes very early (ideally pre-clinically) and intervene to delay, and eventually prevent, clinical progression or onset.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Alzheimer's disease, iatrogenic transmission, what if?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease... </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">*** Singeltary comment PLoS *** </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Posted by flounder on 05 Nov 2014 at 21:27 GMT </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Background</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Methods</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Results</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Conclusions</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="http://www.plosone.org/annotation/listThread.action?root=82860" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://www.plosone.org/annotation/listThread.action?root=82860</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://betaamyloidcjd.blogspot.com/2021/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://betaamyloidcjd.blogspot.com/2021/</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">IN CONFIDENCE</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">5 NOVEMBER 1992</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">There are also results to be made available shortly </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(1) concerning a farmer with CJD who had BSE animals, </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(2) on the possible transmissibility of Alzheimer’s and </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/nature15369</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary Comment at very bottom of this Nature publishing;</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">where have we all heard this before? it's been well documented via the BSE Inquiry. have they not learned a lesson from the last time?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">we have seen this time and time again in England (and other Country's) with the BSE mad cow TSE Prion debacle.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">That 'anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients ? </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">who got pooled extracts injected from thousands of cadavers ? were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find 'alarming' is pathetic.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Sounds like the journalists had it right in the first place: 'Alzheimer's may be a transmissible infection' in The Independent to 'You can catch Alzheimer's' in The Daily Mirror or 'Alzheimer's bombshell' in The Daily Express</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">if not for the journalist, the layperson would not know about these important findings.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer's, the price of poker goes up drastically.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">so, who makes that final decision, and how many more decades do we have to wait?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">in my opinion, it's one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it's bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer's of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer's and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Singeltary Comment at very bottom of this Nature publishing;</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369#article-comments" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/nature15369#article-comments</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nature.com/articles/nature15369</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Saturday, March 18, 2023 </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Autoclave treatment fails to completely inactivate DLB alpha-synuclein seeding activity </span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;">Prusiner et al, then and now!</span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.nejm.org/doi/full/10.1056/NEJM200105173442006" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJM200105173442006</a></span></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none;"></span><br style="outline: none;" /></p><p class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><span class="ydp3b18919byiv0252049878ydp4979a40byiv2014494137ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none; text-decoration-line: underline;"><a href="https://www.pnas.org/doi/10.1073/pnas.2220984120" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://www.pnas.org/doi/10.1073/pnas.2220984120</a></span></p><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none;"><br style="outline: none;" /></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><div dir="ltr" style="outline: none;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;">FRIDAY, JANUARY 15, 2021 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire? </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">if not, why not?</div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">CJD FOUNDATION Questionnaire</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">UK CJD Questionnaire</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a> </div></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">cjd questionnaire 1979</div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://webarchive.nationalarchives.gov.uk/ukgwa/20080102185730mp_/http://www.bseinquiry.gov.uk/files/yb/1980/01/31001001.pdf</a></div></div><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div style="outline: none;">CJD Questionnaire </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">F. MEDICATIONS, has Subject taken any medications regularly, (if yes, record the date, name of the medication, the reason for taking it, and route of administration) prompt for prescription drugs, including insulin and type. Prompt for hormone therapy or nutritional supplements including oral contraceptives and hormone replacement therapy: Prompt for homeopathic/herbal therapy: Prompt for eye drops SUMMARY OF ABOVE RESPONSES; HAS THE SUBJECT BEEN EXPOSED TO ONE OF THE MEDICATIONS OF BOVINE OR OVINE ORIGIN, AND OR ANY DESICCATED ANIMAL ORIGIN? G. Has Subject ever been tested for allergy using needles? H. Has Subject ever received a treatment involving a course of injections? (If yes, record year, name of therapy, frequency, reason)</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: none;"><br style="outline: none;" /></div></div>NOW we know that CWD will transmit to cattle, pigs and sheep, by oral routes, seems some of the old documents pertaining to Pigs and BSE might come to light...terry</div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #338fe9; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://cjdquestionnaire.blogspot.com/2007/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/</a><br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">http://cjdquestionnaire.blogspot.com/</a> </div></div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;"><div style="outline: none;"><div dir="ltr" style="outline: none;"><div style="outline: none;"><div style="outline: none;">Prion 2023</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Title: Diagnostic Journey of Patients with Creutzfeldt-Jakob Disease (CJD) in the United States: A RealWorld Evidence Study</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author list: Duncan Brown1 , Emily Kutrieb2 , Montserrat Vera Llonch1 , Rob Pulido1 , Anne Smith1 , Derek Weycker2 , Ellen Dukes2 , Brian S Appleby3-5</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Affiliations: 1 Ionis Pharmaceuticals; 2Policy Analysis Inc. (PAI); 3National Prion Disease Pathology Surveillance Center; 4Case Western Reserve University; 5University Hospitals Cleveland Medical Center</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Aims: Identification of clinical symptoms leading to a diagnosis of CJD from real-world evidence is limited. A new study using a United States (US) healthcare claims database was thus undertaken to address this evidence gap.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Materials and Methods: A retrospective cohort design and the Merative MarketScan Database (01/2012-12/2020) were employed. The study population comprised adults aged ≥18 years with ≥1 inpatient diagnosis or ≥2 outpatient diagnoses (≥3 days apart) of CJD, magnetic resonance imaging of the head or lumbar puncture, and no evidence of selected neurologic conditions after the last CJD diagnosis. Patients without healthcare coverage during the 12-month pre-diagnosis period were excluded; alternative pre-diagnosis periods (spanning 24 and 36 months, respectively) were also explored. Diagnostic journey was detailed based on diagnosis codes for selected symptoms and neurologic conditions during the pre-diagnosis period.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Results: Among the 61.8 million persons in the source population from 01/2013-12/2019, 215 CJD patients qualified for inclusion in the study population. CJD patients first presented with symptoms consistent with the diagnosis 5.0 (SD=4.0) months, on average, before the initial CJD diagnosis, and 80% had ≥3 symptoms, most commonly altered mental status (82%), gait/coordination disturbance (60%), and malaise/fatigue (44%). Most patients (63%) also had ≥1 differential (neurologic) diagnosis leading to the CJD diagnosis, most commonly cerebrovascular disease (49%), peripheral vertigo (11%), and Alzheimer’s disease (7%); mean duration from first differential diagnosis to initial CJD diagnosis was 2.4 (SD=3.1) months.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Conclusions: Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey. CJD should be considered in the differential diagnosis of those with rapidly progressing dementia or motor disturbance.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Funded by: Ionis Pharmaceuticals</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Grant number: N/A</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Acknowledgment: XXX</div></div><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none;" /></div><div dir="ltr" style="outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="outline: none;">"Study findings suggest that, in US clinical practice, CJD patients present with one or more clinical symptoms impacting motor, cognitive or other domains, and many are initially mis-diagnosed, prolonging the diagnostic journey."<br style="outline: none;" /></div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><div style="outline: none;">2001 Singeltary on CJD</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">February 14, 2001</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Terry S. Singeltary, Sr</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">Author Affiliations</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="outline: none;"><br style="outline: none;" /></div><div style="outline: none;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="outline: none;"><br style="outline: none;" /></div></div><div style="outline: none;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div></div></div><div style="font-family: arial; font-size: 16px; outline: none;"><br style="outline: none;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none;">considering 25 years, seems to be a colossal waste of time on my part, 25 years and nothing, were still talking about the same damn failures, imo $$$</div></div><div style="outline: none;"><span style="outline: none;"><br style="outline: none;" /></span></div><div style="outline: none;"><span style="outline: none;">wasted days and wasted nights...Freddy Fender</span></div><div style="outline: none;"><span style="outline: none;"><br style="outline: none;" /></span></div><div dir="ltr" style="outline: none;"><span style="outline: none;">stupid is, as stupid does, and sometimes, you just can't fix stupid $$$</span></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;"><br style="outline: none;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none;">Terry S. Singeltary Sr., flounder9@verizon.net, Bacliff, Texas, Galveston Bay, on the bottom!</div><div><br /></div></div></div></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-4968696343041095382023-10-27T11:35:00.003-05:002023-10-27T11:35:44.990-05:00 Prion disease features in Japan based on the national surveillance from 1999 to 2023<p> <span style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px;">Prion disease features in Japan based on the national surveillance from 1999 to 2023</span></p><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Authors: Tadashi Tsukamoto 1), Koki Kosami 2), Ryuusuke Ae 2), Tsuyoshi Hamaguchi 3), Nobuo Sanjo 4), Katsuya Satoh 5), Tetsuyuki Kitamoto 6), Masaki Takao 7), Masahito Yamada 8), and Hidehiro Mizusawa 1) and Prion Diseases Surveillance Committee in Japan</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Affiliations: 1 Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan 2 Department of Public Health, Jichi Medical University, Shimotsuke, Japan 3 Department of Neurology, Kanazawa Medical University, Uchinada Town, Japan 4 Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan 5 Department of Locomotive Rehabilitation Sciences, Nagasaki University Graduate School of Medicine, Nagasaki, Japan. 6 Department of Prion Protein Research, Division of CJD Science and Technology, Tohoku University Graduate School of Medicine, Sendai, Japan 7 Department of Clinical Laboratory, National Center of Neurology and Psychiatry, National Center Hospital, Tokyo, Japan. 8 Division of Neurology, Department of Internal Medicine, Kudanzaka Hospital, Tokyo, Japan.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Aim: To elucidate epidemiological features of prion disease (PrD) in Japan.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Methods: Nationwide surveillance has been conducted by Prion Disease Surveillance Committee in Japan since 1999. The committee was composed of 10 district members and specialists in epidemiology, neurosurgery, genetics, EEG, imaging, CSF biomarkers, and genetic counseling. Clinical information on suspected PrD cases was compiled and reviewed in the committee twice yearly, resulting in diagnoses of PrD and non-PrD.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Results: Information of 6936 suspected PrD patients was obtained and until February 2023 there were 4714 PrD cases [sporadic CJD 3578 cases, variant CJD 1, dura matter-associated CJD (dCJD) 93, and genetic PrD 1024 (genetic CJD 842, 174 Gerstmann-Sträussler-Scheinker disease 174, fatal familial insomnia 8)]. The annual incidence increased from 0.7 to 2.3 per million between 1999 and 2015. Genetic analysis showed a high ratio of methionine/methionine (95%) at codon 129 in sporadic CJD. Effects of polymorphisms at codon 129 and 219 to various PrDs were investigated.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Conclusions: Our surveillance confirmed the increase of PrD incidence and characteristic features of PrDs in Japan. Continuous surveillance of PrDs is very important and has contributed greatly to various types of research to overcome PrDs.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Funded by: 1) Research Committee of Surveillance and Infection Control of Prion Disease, the Ministry of Health, Labour, and Welfare of Japan</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">2) Research Committee of Prion Disease and Slow Virus Infection, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health and Labour Sciences Research Grants</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Grant number: 1) 22FC2002 2) 20FC1054</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;">Acknowledgment: We are grateful to the prion disease specialists in the prefectures, physicians, patients with prion disease, and their families for providing clinical information.</div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;" /></div></div></div><div data-setdir="false" dir="ltr" style="color: #333333; font-family: Verdana; font-size: 14px; outline: none !important;"><br style="outline: none !important;" /></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">terry</div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-57470334521914831472023-10-27T11:30:00.004-05:002023-10-27T11:30:57.598-05:00Geographic Diversity in the Incidence of Human Prion Diseases — China, 2006–2019<p><span style="background-color: white; color: #333333; font-family: Verdana; font-size: 16px; font-weight: 700;">Geographic Diversity in the Incidence of Human Prion Diseases — China, 2006–2019</span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><p style="color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"> <span style="font-family: "Times New Roman", serif; font-size: 12pt; outline: none !important;">Researchers from the State Key Laboratory for Infectious Disease Prevention and Control in China have conducted a study analyzing the features of hospitals referring cases of human prion diseases (PrDs) to the Chinese National Surveillance for Creutzfeldt-Jakob disease (CNS-CJD) between 2006 and 2019. The study found that the number of referring hospitals increased along with the annual reported PrD cases, with 42 out of 334 hospitals reporting 70% of all diagnosed cases. Furthermore, there was a geographic-related diversity, with certain administrative regions and provincial capital cities reporting significantly higher numbers of PrD cases. Human prion diseases are rare, fatal, and transmissible encephalopathies. The diagnosis requires comprehensive analysis of clinical manifestations and laboratory tests, and the main symptom is rapid progressive dementia. The study highlights the need for improved surveillance and recognition of PrDs in China.</span></p><div style="color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"> <span style="outline: none !important;"><span style="font-family: "Times New Roman"; font-size: 16px; outline: none !important;">For more information: </span></span><a href="https://weekly.chinacdc.cn/en/article/doi/10.46234/ccdcw2023.181" rel="nofollow" style="color: #196ad4; font-family: arial; font-size: 16px; outline: none !important;" target="_blank">https://weekly.chinacdc.cn/en/article/doi/10.46234/ccdcw2023.181</a></div></div><div style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"><div style="outline: none !important;"><div style="outline: none !important;">Vital Surveillances: Geographic Diversity in the Incidence of Human Prion Diseases — China, 2006–2019 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Qi Shi1, , ; Kang Xiao1; Liping Gao1; Yuezhang Wu1; Wei Zhou1; Donglin Liang1; Cao Chen1; Xiaoping Dong1,2,3, , View author affiliations Abstract Introduction Human prion diseases (PrDs) are rare, fatal encephalopathies requiring comprehensive diagnostic analysis. This study examines hospital referral patterns to the Chinese National Surveillance for Creutzfeldt-Jakob Disease (CNS-CJD) from 2006 to 2019.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods We assessed 1,970 PrD cases referred by various hospitals to CNS-CJD. Referral distributions were analyzed based on provincial-level administrative divisions (PLADs). Differences in referral numbers and confirmed cases between monitored and non-monitored PLADs were statistically evaluated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results The study included cases from 344 hospitals across 29 Chinese PLADs. Hospital referrals increased over the surveillance years: from 28.2 hospitals annually during 2006–2010, to 64 in 2011–2015, and 107 in 2016–2019. Of these, 12.2% (42/344) of hospitals reported ≥10 PrD cases, accounting for 70.0% (1,379/1,970) of total cases. Referral numbers varied across PLADs, with the top 5 of Beijing (41), Henan (26), Shanghai (21), Guangdong (21), and Jiangsu (21) leading. Additionally, 12 CJD-surveillance PLADs had more referring hospitals and PrD cases than the other 17 non-surveillance PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions Geographical variations in PrD recognition exist across Chinese PLADs, with certain regions and major cities reporting notably higher case numbers.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Human prion diseases (PrDs) encompass a suite of transmissible spongiform encephalopathies (TSEs) induced by the unwelcome prion pathogen. This set of diseases includes Kuru disease, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI) (1-2). Human PrDs can manifest in sporadic, genetic, or iatrogenic forms. Among these, sporadic CJD (sCJD) is the most prevalent form of human PrDs, constituting approximately 85%–90% of all PrD cases (3-4). The global morbidity for human PrDs is estimated at 1–2 individuals per million annually, with mortality invariably standing at 100% (2-3). Clinical manifestations of human PrDs can be diverse; however, rapid progressive dementia typically serves as the primary symptom. The definitive diagnosis of human PrDs currently relies on the neuropathological examination of biopsied brain tissue or postmortem analyses (1–2,5).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Recognition and diagnosis of human PrDs in China remained largely absent until the end of the 1980s due to its rarity. Prior to the implementation of the China National Surveillance for Creutzfeldt-Jakob Disease (CNS-CJD) in 2006, only a handful of Chinese CJD cases were reported in scholarly literature (6-7). The initiation of the CNS-CJD, however, led to an increase in diagnoses as hundreds of hospitals across China began to recognize and identify PrD cases (8-10).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nonetheless, there was notable variance in both the number of participating hospitals and diagnosed PrD cases between different provincial-level administrative divisions (PLADs) in China (9). Utilizing CNS-CJD surveillance data, we conducted an analysis of the relevant factors pertaining to participating hospitals. This analysis was undertaken to evaluate the disparities in PrD recognition capacities across different PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">METHODS CNS-CJD The development and execution of the CNS-CJD project has previously been discussed in depth (10). To summarize, the CNS-CJD initiative was officially launched in 2006. This project encompasses 12 provincial CDCs and 15 sentinel hospitals, spanning across 12 PLADs, including Beijing, Shanghai, Tianjin, Chongqing, Jilin, Shaanxi, Hubei, Guangdong, Guizhou, Anhui, Henan, and Xinjiang (9–10). Surveillance data indicated that clinical records and samples from suspect patients were procured by local hospital clinicians, while pertinent epidemiological data were gathered by provincial CDC personnel. Compiled data and samples [including cerebrospinal fluid (CSF), blood, and brain tissue] were then forwarded to the Chinese CDC’s national reference laboratory for CNS-CJD for lab testing and ultimate diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Case Definition The suspected cases of CJD referred to as CNS-CJD were identified and subtyped based on the diagnostic criteria released by the Chinese National Health Commission. This criteria framework was adapted from the diagnostic criteria for CJD developed by World Health Organization (WHO). Patient clinical and epidemiological data were gathered using specially designed questionnaires. A spectrum of clinical examination results were amassed, including magnetic resonance imaging (MRI), electroencephalography (EEG), and routine CSF biochemistry, along with laboratory tests such as CSF 14-3-3, CSF tau, CSF and skin real time-quaking induced conversion assay (RT-QuIC), and PRNP PCR and sequencing. A panel of experts consisting of neurologists, neuropathologists, epidemiologists, and laboratory staff were responsible for determining the interim or final diagnosis (9).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Data Collection This study incorporated a total of 1,970 distinct PrD cases, comprising patients with sCJD and a variety of genetic PrDs (gPrD). Hospitals that reported diagnosed cases to CNS-CJD were identified as the referring hospitals in this investigation. Separate counts were maintained for the annual and cumulative totals of PrD cases reported from each hospital. The distribution of referring hospital numbers and their diagnosed PrD cases were established based on the varied PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Statistical Analysis The differences in the numbers of the referring hospitals and the diagnosed PrD cases between surveillance and non-surveillance PLADs were assessed by two-tailed Student’s t test using the SPSS 22.0 (International Business Machines Corporation, Armonk, New York, USA) statistical package. The data were presented as mean±standard deviation (SD).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">RESULTS The Number of Referring Hospitals for Human PrDs Increased Over the Duration of the Surveillance Years This study enrolled a total of 1,970 human PrD cases of CNS-CJD from 2006 to 2019, encompassing sCJD, FFI, and various genotypes of gCJD and GSS. These PrD cases derived from 344 distinct hospitals across 29 of the 31 PLADs in Chinese mainland. Remarkably, 89% (308/344) of these referring hospitals represent Grade III class A medical institutions, the highest level according to Chinese standards for hospital classification. The annual tally of both the referring hospitals and the diagnosed PrD cases was compiled for the years 2006 through 2019. Concurrent with the uptick in diagnosed PrD cases was an increase in the number of referring hospitals throughout these surveillance years (Figure 1). The average number of referring hospitals during the first five years (2006–2010) and the second five years (2011–2015) were 28.2 and 64 respectively, this number increased to 107 in the most recent four years (2016–2019). This suggests that an increasing number of hospitals have been identifying and diagnosing human PrD cases over the surveillance period.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A Significant Number of PrD Cases Reported from a Small Subset of Hospitals The referring hospitals were categorized based on the number of PrD cases they diagnosed (Table 1). Among these, 12.2% (42/344) reported and diagnosed ten PrD cases within the study period, collectively representing 70.0% (1,379/1,970) of all PrD cases. Leading the group was Beijing Xuanwu Hospital, which diagnosed the highest number of PrD cases (163). Moreover, ten additional hospitals referred between 40 to 99 PrD cases, distributed among Beijing (4 hospitals), Henan (2), Guangdong (1), Jilin (1), Shanghai (1), and Sichuan (1). An additional 31 hospitals reported between 10 to 39 cases. Despite this, the majority of the hospitals (263, accounting for 76.5%) reported merely one or a handful of PrD cases (less than 5).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Referral Hospitals in China Unevenly Distributed Among the PLADs We evaluated the diversity of the referring hospital distributions within the PLADs, based on the number of referring hospitals and the PrD cases reported in each PLAD. Table 2 indicates that Beijing had the highest number of referring hospitals and diagnosed PrD cases. During the study period, 41 hospitals from Beijing reported 546 PrD cases, constituting 11.9% of the referring hospitals and 27.7% of the total reported PrD cases. Five other PLADs reported equal to or more than 20 referring hospitals: Henan (26), Shanghai (21), Guangdong (21), Jiangsu (21), and Hebei (20). An additional seven PLADs had referring hospital numbers ranging from 10 to 19. The reported cases from these 13 PLADs represented 82.6% of all PrD cases (1,628 out of 1,970). While there was a strong correlation between the number of referring hospitals and diagnosed PrD cases, diversities were observed in several PLADs. For instance, Jiangsu and Hubei had a larger number of referring hospitals but a relatively small number of PrD cases, whereas Jilin, with fewer referring hospitals, reported a higher number of PrD cases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 2 further delineates the distribution of hospitals in various PLADs in China that referred 10 or more PrD cases. Of the 29 PLADs reporting PrD cases, 19 had a range of hospitals referring at least 10 PrD cases. The most significant numbers of such hospitals were located in Beijing (11) and Shanghai (5), which reported 472 and 107 PrD cases, respectively. Two provinces, Shandong and Shaanxi, each had three hospitals referring 10 or more PrD cases, and five PLADs — Chongqing, Henan, Guangdong, Hebei, and Zhejiang — each had two hospitals doing the same. Among these, two hospitals in Henan reported notably higher case numbers, with 152 cases. In contrast, 10 PLADs had only one hospital referring 10 or more PrD cases, with the most cases reported in Jilin (49), Sichuan (47), and Fujian (28). These data highlight substantial variation in both the number of referring hospitals and those referring 10 or more PrD cases. Given its unparalleled medical resources, Beijing demonstrates its superior capacity for recognizing and diagnosing PrDs among the varied PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Majority of Referring Hospitals Located in Provincial Capital Cities The disparities in the number of referral hospitals and reported PrD cases between provincial capital cities and other cities received further analysis. Excluding four municipalities (Beijing, Shanghai, Tianjin, and Chongqing), 146 hospitals from the 25 provincial capital cities reported 947 PrD cases, while 117 hospitals from other cities reported 209 cases (Table 3). Although the overall ratio of referral hospitals in provincial capital cities to those in other cities was not significantly different (55.5% vs. 44.5%, P=0.359), the ratio of diagnosed PrD cases showed a significant disparity (81.9% vs. 18.1%, P=0.001). On average, the number of referral hospitals and PrD cases in the 25 provincial capital cities were 5.84 (range: 1–17 hospitals, median: 5) and 37.88 (range: 1–169 cases, median: 21), respectively. Considering the actual number of other prefecture-level cities (281 cities excluding the capital cities) in those 25 PLADs, the average number of referral hospitals was 0.41 (range: 0–17), and the average number of PrD cases was 0.74 (range: 0–36 cases). This discrepancy underlines the predominant role of provincial capital cities in identifying PrD patients.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A Greater Number of Referring Hospitals Found in the Surveillance PLADs Compared to the Non-surveillance PLADs Evaluation of the numerical differences between referring hospitals and diagnosed PrD cases in both surveillance and non-surveillance PLADs was undertaken. A mean of 15.3 referring hospitals were counted in the 12 surveillance PLADs (range 6.0–41.0), above the average of the 17 non-surveillance PLADs (9.9 hospitals, range 2.0–21.0); however, the observed discrepancy did not reach statistical significance (P=0.073). Conversely, diagnosed PrD cases in surveillance PLADs presented a noticeable average increase, with 117.4 cases (range 31.0–516.0) compared to the 34.9 cases (range 2.0–98.0) of non-surveillance PLADs (P=0.028) (Figure 2A).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Assessing the historic data spanning 2006–2019, calculations of hospitals referring 10 cases and their corresponding PrD diagnoses were performed. Surveillance PLADs averaged 2.4 hospitals referring a minimum of 10 cases (range 0.0–11.0) and their corresponding average PrD diagnoses were 86.4 (range 0.0–472.0), significantly greater compared to non-surveillance PLADs. Non-surveillance PLADs averaged at 0.8 for hospitals referring 10 cases (range 0.0–3.0, P=0.042), with an average of 15.9 PrD diagnoses (range 0.0–78.0, P=0.036) (Figure 2B).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Moreover, within the timeframe of investigation, it was recorded that in 47.1% of non-surveillance PLADs (8/17) and 16.7% (2/12) of surveillance PLADs, no hospital had diagnosed 10 cases. The results highlight a comparatively robust capacity in the recognition and diagnosis of PrDs within the surveillance PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DISCUSSION In the present study, we have analyzed the annual- and geographic-distributions and case reporting frequencies of the hospitals diagnosed and referred human PrDs in China in the past 15 years. Accompanying with the increase of the annual diagnosed PrD cases, more and more hospitals referred PrD cases to CNS-CJD actively, particularly in the latest four year that the numbers of referring hospitals are over 100 continually. Notably, some local and small hospitals (below Grade III) started to report PrD cases actively in recent years. It implies that the awareness and recognition of human PrDs in the medical institutions in China has been gradually improved in the past 15 years.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is a notable disparity in the frequency of case reporting across referring hospitals, characterized by a majority of diagnosed PrD cases originating from a limited number of these institutions. The majority of PrD cases are referred by a select few — primarily university hospitals boasting robust neurology departments — amongst the top 20 referring hospitals. Conversely, the majority (over 75%) of referring hospitals reported a relatively low number of PrD cases (less than 5), with 168 (48.8%) of these hospitals having only reported a single case in the last 15 years. These findings suggest a significant variation in the recognition capacity for human PrDs among hospitals in China.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our findings suggest a significant geographic disparity in the distribution of both referring hospitals and PrD cases. Expectedly, the eastern region of China record a higher number of referring hospitals and PrD cases than their western counterparts. The four municipalities directly governed by the central government — Beijing, Shanghai, Chongqing, and Tianjin — report high rates of referring hospitals and PrD cases. Notably, Beijing, the capital city, reports a significantly higher number with 4 out of the top 10 hospitals reporting the most cases situated there. Conversely, PLADs such as Inner Mongolia, Hainan, Ningxia, and Heilongjiang, located in the border regions and characterized by lower population density and underdeveloped economies, report fewer referring hospitals and/or PrD cases. Notably, the PLADs of Qinghai and Xizang have no medical institutions actively reporting any PrD cases. This geographic disparity, to some extent, reflects the variable provincial-level capacity for recognizing human PrDs in China.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A prominent trend observed is the elevated detection of human PrD cases heavily concentrated in larger, central cities. Aside from the four municipalities, the incidence of reported PrD cases in provincial capital cities significantly surpasses the cumulative incidents from other cities. Aside from these four municipalities, the overwhelming majority of hospitals with at least ten reported PrD cases in PLADs are situated in provincial capitals. This disparity in PrD detection between major, central cities and smaller, outlying regions is strongly linked to variances in access to medical resources and economic proficiency. Predominantly, large, reputable hospitals — particularly university-affiliated hospitals — are located within provincial capital cities.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We have found a significant difference in the PrD recognition capacity between CJD surveillance and non-surveillance PLADs, especially notable during the first and second five-year periods of surveillance. In addition to the available medical resources, we attribute this disparity to the more established CJD surveillance activities, annual human PrD training courses and workshops, as well as relatively active academic exchanges concerning human PrDs and other neurodegenerative diseases, organized by various local entities. This significantly bolsters PrD recognition and diagnostic capacity within the surveillance PLADs. This is exceedingly pronounced in Beijing and Shanghai where all Grade III hospitals have been incorporated into their local CJD surveillance systems (11). Moreover, an increasing number of provincial CDCs and medical institutions from non-surveillance PLADs are being included in the annual workshops and other activities hosted by CNS-CJD in recent years. We believe this is a positive step towards enhancing PrD recognition capacity in these non-surveillance PLADs.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Despite significant improvements in PrD recognition capacity in China, largely due to the implementation of CNS-CJD since 2006, the yearly case count remains underestimated compared to data from numerous countries in Europe and North America (12–14). This is the case even in large, developed central cities. Even though the China CDC implemented a diagnostic protocol for CJD surveillance in 2006, and the National Health Commission issued diagnostic criteria for CJD in 2017, most PrD patients only receive their final diagnoses after being transferred multiple times between hospitals, typically from smaller local facilities to larger central institutions. As PrDs represent a rare set of neurological diseases lacking specific clinical manifestations, misdiagnoses are frequent (15). Increased awareness and recognition of human PrDs are warranted, particularly in smaller cities. Key strategies for improvement include the widespread promotion and implementation of CJD diagnostic criteria among clinicians, the sequential development of training programs on prions and PrDs for clinicians, public health personnel, and laboratory staff, and an emphasis on related scientific literacy and health education for the general public.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conflicts of interest No conflicts of interest.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funding: Supported by the Grant (2021SKLID101, 2019SKLID603) from the State Key Laboratory for Infectious Disease Prevention and Control, China CDC Author Affiliations 1. National Key-Laboratory of Intelligent Tracking and Forecasting for Infectious Disease, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2. Center for Biosafety Mega-Science, Chinese Academy of Sciences, Wuhan City, Hubei Province, China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. China Academy of Chinese Medical Sciences, Beijing, China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Corresponding authors: Qi Shi, shiqi@ivdc.chinacdc.cn; Xiaoping Dong, dongxp238@sina.com Online Date: October 27 2023 doi: 10.46234/ccdcw2023.181 References</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"><a href="https://weekly.chinacdc.cn/en/article/doi/10.46234/ccdcw2023.181" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://weekly.chinacdc.cn/en/article/doi/10.46234/ccdcw2023.181</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; color: #333333; font-family: Verdana; font-size: 14px; line-height: 25px; margin: 0px 0px 10px; outline: none !important; padding: 0px;"><div style="outline: none !important;"><div style="outline: none !important;">A Comparative Study of the Diagnostic Value of Real Time-Quaking Induced Conversion Assay in Multi-Site Skin and Cerebrospinal Fluid for CreutzfeldtJakob Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Liyong Wu1 , Zhongyun Chen1 , Qi Shi2 , Xiaoping Dong2</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China. 2 State Key Laboratory for Infectious Disease Prevention and Control, NHC Key Laboratory of Medical Virology and Viral Diseases, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China, Beijing 102206, China.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: This study aims to compare the diagnostic differences between real-timequaking induced conversion (RT-QuIC) in multi-site skin and cerebrospinal fluid (CSF) for Creutzfeldt-Jakob disease (CJD) and evaluate their diagnostic value in the early stages of the disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: CJD patients admitted to Xuanwu Hospital of Capital Medical University between September 2021 and April 2023 were included. Skin tissue samples (3 mm2) were collected from behind the ear, upper arm, back, and thigh using a skin punch. CSF was collected when possible. The RT-QuIC assay was performed at the Prion Disease Laboratory of the Chinese Center for Disease Control and Prevention.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: The study included 58 CJD patients, with a male-to-female ratio of 1:0.6 and a mean age of onset at 61.8 years. Among them, 50 cases were diagnosed as sporadic CJD and 8 cases as genotypic CJD. The mean time from onset to biopsy was 5.3 months, with 35 patients biopsied within 3 months. The skin RT-QuIC had a higher overall positive rate (93.1%) compared to CSF (68.8%) (P<0.05). Some patients had positive skin but negative CSF RT-QuIC results, and vice versa. Skin biopsy sites showed similar positivity rates. Among patients with sporadic CJD, the skin RT-QuIC had a higher positivity rate (96.0%) compared to CSF (69.8%). For genotypic CJD, the positivity rates for skin and CSF RT-QuIC were lower but not statistically significant. Patients with an onset-to-biopsy time of less than 3 months had higher positivity rates for both skin and CSF RT-QuIC compared to those with longer intervals.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: In Chinese CJD patients, cutaneous RT-QuIC has a higher positive rate than CSF, and simultaneous testing can be complementary. A multi-site skin biopsy may improve the diagnostic rate of skin positivity. RT-QuIC shows important diagnostic value in the early detection of CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: National Natural Science Foundation of China Grant Number: 8227146 and 81971011</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: None Theme: Biomarkers for prion and other neurodegenerative diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unraveling the Unique Genetic and Clinical Signatures of Prion Diseases in China: Insights from a Pioneering Single-Center Investigation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Liyong Wu, Zhongyun Chen, Yu Kong</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: This study aimed to analyze the genetic, clinical, and ancillary examination characteristics of prion diseases in China, focusing on a single center.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: Patients diagnosed with prion diseases and admitted to Xuanwu Hospital of Capital Medical University between January 2012 and July 2023 were included. Data on genetic profiles, clinical manifestations, and ancillary examinations (MRI, EEG, cerebrospinal fluid biomarkers, and RT-QuIC) were collected. Using the clinical diagnostic criteria of MM2C and MM2T types proposed in 2020 and 2018, approximate pathological speculations were made for the included sporadic Creutzfeldt Jakob disease (sCJD) patients.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: A total of 287 prion patients were included, with a male-to-female ratio of 1:0.9, mean age of onset of 60.1 years, and median disease duration of 1.1 years. Genetic prion diseases accounted for 13.6% of cases, with 248 sCJD, 24 genetic CJD (gCJD), 12 fatal familial insomnia (FFI), and 3 Gerstmann-Straussler-Scheinker syndrome (GSS) cases. gCJD cases included 9 E200K, 8 T188K, 2 V180, and one G114V, 7-OPRI, R148H, T193I, and V210I. FFI and GSS cases had D178N-129M and P102L genotypes, respectively. Common findings in CJD patients were high DWI signals, periodic sharp wave complexes on EEG, positive cerebrospinal fluid biomarkers (14-3- 3 protein, total tau), positive skin/cerebrospinal fluid RT-QuIC, and PRNP-129M haplotype. Among 235 sCJD patients, 25.1% were probable MM2 type CJD, including 22.6% MM2C and 2.6% MM2T cases. Heidenhain's variant of CJD accounted for 8.0% and included mutations in PRNP (5 cases), T188K (3 cases), E200K (1 case), and V210I (1 case).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: This study reveals distinct genetic distributions, clinical features, and pathological classifications of prion diseases in China compared to Caucasian populations.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: National Natural Science Foundation of China Grant Number: 8227146 and 81971011</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: None Theme: Biomarkers for prion and other neurodegenerative diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Fatal familial insomnia in China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Min Chu, Zhongyun Chen, Liyong Wu</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xuanwu Hospital, Capital Medical University, Beijing, China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: To summarize the clinical and genetic features of patients with Fatal familial insomnia (FFI) in China.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Materials and Methods: We collected clinical and genetic data from 13 FFI patients admitted to Xuanwu Hospital in Beijing, and 49 Chinese FFI patients / 85 from Western countries reported in the literature who with detailed information. Clinical and genetic features of Chinese FFI patients were summarized and compared with that of Western countries.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Among the 13 patients at Xuanwu Hospital, 61.5% (8/13) had a positive family history, with an average age of 49.23 ± 13.10 years. The male-to-female ratio was 0.86:1. Eleven patients died with a mean survival time of 11.8 ± 3.08 months. All patients (13/13) exhibited sleep disturbances, rapidly progressive dementia, and autonomic symptoms. No high signal and PSWCs was found on Diffusion-weighted imaging (DWI) and electroencephalography (EEG) (13/13). Skin biopsy and CSF RT-QuIC were done in 2 patients, and all were positive. CSF neurofilament light (NFL) levels were measured in 2 patients, and all were elevated. Cortisol/melatonin circadian rhythm disappeared in 100% (4/4) of the patients. Among all Chinese patients, a shorter disease duration (11.46 ± 5.97 vs. 14.43 ± 9.78 months) and a higher rate of positive CSF 1433 protein (56.7% vs. 18.2%) were found than Westerners. The genotype of 129 MM and MV was 1/61 and 61/21 in Chinese and Westerners. Currently, there are no specific interventions for FFI. Agomelatine and a ventilator might help.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion: FFI in China has a shorter disease duration, a higher positivity rate of 14-33 protein in cerebrospinal fluid, and a significantly lower proportion of the MV genotype at codon 129 than Westerners. Given the rarity of FFI, future studies should continue to expand the sample size to clarify the unique characteristics of the Chinese FFI population.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: National Natural Science Foundation of China</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant Number: 8227146 and 81971011</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgment: None</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Theme: Biomarkers for prion and other neurodegenerative diseases</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html</a></div><div dir="ltr" style="outline: none !important;"><br /></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">terry</div><div><br /></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-72016226934239845962023-09-11T10:17:00.004-05:002023-09-21T13:32:05.911-05:00Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide<div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor John Collinge on tackling prion diseases sCJD accounts for around 1 in 5000 deaths worldwide<br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”</span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor John Collinge on tackling prion diseases</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor John Collinge is Director of the MRC Prion Unit and also directs the NHS National Prion Clinic at the adjacent National Hospital for Neurology and Neurosurgery.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">John Collinge What are prions, why are they important, and how might they help us develop treatments for neurodegenerative conditions like dementia?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Prions are lethal pathogens that cause neurodegenerative diseases of humans and other mammals.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide. In sharp distinction to all other infectious agents, prions lack their own DNA or RNA genome and consist of polymers of a misfolded form of a normal cellular protein (the prion protein or PrP) which form amyloid fibrils.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">These fibres grow by addition of PrP molecules at their ends and they eventually fragment producing more prion particles which continue this process and spread throughout the brain. The final proof of the once controversial “protein-only hypothesis” of prions came with the determination of the structure of infectious prions at near atomic resolution by cryogenic electron microscopy by ourselves and US colleagues in the last few years.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The normal cellular prion proteins are very similar between different species of mammals and therefore a prion infection from one species can sometimes infect another species. This is what happened with the prion disease of cattle, bovine spongiform encephalopathy (BSE) in the 1990’s which caused a new human prion disease known as variant Creutzfeldt-Jakob disease (vCJD) and led to the BSE crisis in the UK, EU and other countries.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">While human prion diseases are thankfully rare, there are common prion diseases of other species, for example scrapie in sheep and goats worldwide and chronic wasting disease in deer in North America. While prions were first thought to be unique to these rare neurological diseases, it became clear that the molecular process was of far wider relevance with for example the recognition of several different proteins in yeast that could form prions.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Most importantly with respect to neurodegeneration and dementia in humans, it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases. In Alzheimer’s disease (AD) for example, two proteins in the brain, amyloid-beta and tau can form self-propagating assemblies which spread in the brain. Indeed, we reported in two articles in Nature that the amyloid-beta pathology seen in AD can be transmissible between humans in rare circumstances causing the newly recognised condition iatrogenic cerebral amyloid angiopathy.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There is accumulating evidence also for iatrogenic AD. Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine. Many years ago, we demonstrated that targeting the production of the normal cellular prion protein completely halted the progression of neurodegeneration (and indeed even reversed early pathological changes) in laboratory mice. This work has underpinned multiple efforts to develop rational treatments for prion and other neurodegenerative diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">What first attracted you to the area of prion diseases?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I first became involved in this field while working as a graduate student applying early molecular genetic methods to study neuropsychiatric diseases and was involved in the first description of mutations in the prion protein gene in the late 1980s in what are now known as the inherited prion diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">As it was already known that brain tissue from patients who died from some of these genetic conditions could transmit disease when inoculated into laboratory animals, it seemed to me highly likely that some version of the then intensely controversial “protein-only hypothesis” was likely to be correct: this had major implications in pathobiology.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I went on to show that being heterozygous for a common human prion protein polymorphism had a profound effect on susceptibility to CJD; I considered this entirely consistent with a protein-only agent and this led to further work studying the genetics of prion disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">It seemed to me at the time that these early genetic insights, albeit in a rare disease, provided a powerful way in to study the fundamental basis of neurodegeneration. Of course, the evolving concerns about BSE in the early 1990’s also focussed my mind on the specific public and animal health risks posed by prions.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">You led the UK’s first clinical trial in CJD, the largest yet conducted internationally. Can you tell us about this? </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I was asked in 1997 by Medical Research Council (MRC) at the request of UK Government to establish and lead an MRC Unit to focus on understanding prion diseases and to ultimately develop treatments for them.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">At the time it was unknown how many people would develop vCJD following the widespread dietary exposure of the UK population to BSE prions and the possibility that this may eventually affect hundreds of thousands could not then be excluded.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">An early proposal (by Dr Prusiner at UCSF) for a treatment for CJD was the anti-malarial drug quinacrine based on early work in prion-infected cell cultures. We were asked by the Chief Medical Officer to establish a clinical trial and did so in collaboration with the MRC Clinical Trials Unit also based at UCL.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">While the MRC PRION-1 trial, as is was called, did not show any benefit of quinacrine, we did learn a great deal about how best to conduct a clinical trial in CJD in conjunction with patients and families affected by these terrible conditions.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">This lead on to the formation of the National Prion Monitoring Cohort (NPMC) to study the natural history of prion diseases and to develop better clinical scales and biomarkers, and earlier diagnosis, to facilitate future clinical trials. In particular, we reasoned that having a large longitudinal data set would allow us to conduct adequately powered efficacy trials by comparison of treated patients with historical controls rather that using a more classical placebo-controlled study which was understandably unacceptable to patients and their families given the rapid and invariably fatal progression of these diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The NPMC has been extremely successful with the strong support of the patient community and has recruited over 1100 patients to date, by far the largest dataset worldwide, and has enabled development and validation of multiple clinical scales and blood and CSF biomarkers.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">What in your opinion have been some of the most important findings of your research to date?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Our early work established and characterised the inherited prion diseases and genetic susceptibility to acquired and sporadic prion disease, and pioneered diagnostic and presymptomatic genetic testing of neurodegenerative disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Many further genetic advances followed. Prions exist in multiple strain types and we developed molecular strain typing of prions which we applied in 1996 to first demonstrate that vCJD was caused by the same prion strain as cattle BSE, a finding of critical public and animal health significance at the time.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">We characterised the pathogenesis of vCJD to inform public health risk assessments, developed the first blood test for vCJD and effective means to prion sterilise surgical instruments. We proposed the now widely accepted “conformational selection hypothesis” to explain the relationship between prion strains and intermammalian transmission barriers and proposed that prion strains constitute a “cloud” under host selection rather than a molecular clone.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Importantly, we described subclinical prion infections in which animals lived a normal lifespan despite harbouring high levels of prions and went on to study the kinetics of prion propagation in vivo and showed that propagation and neurotoxicity occur in two distinct mechanistic phases with pathology only developing after prion levels had plateaued in the brain.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">We subsequently confirmed that prions themselves are not directly neurotoxic. These insights may be fundamental to understanding other diseases involving propagation and spread of assemblies of misfolded proteins, notably amyloid-beta and tau in AD.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Our discovery of human transmission of amyloid-beta pathology, mentioned above, in individuals treated many years earlier in childhood with human cadaver-derived pituitary growth hormone (c-hGH) accidentally contaminated with amyloid-beta seeds (prions) has wide implications for understanding, preventing and treating neurodegenerative diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health. Iatrogenic AD is likely to be recognised in the cohort of c-hGH recipients as they age further. Our demonstration that reducing prion expression during neuroinvasive prion disease in laboratory mice prevented onset, and reverses early pathology, produced a proof of principle of therapeutically targeting prion protein.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">This led to our development of a biopharmaceutical which we have used to treat CJD. Recently, we have described the elusive structural basis of prion strain diversity: how prions can encode information in a non-Mendelian manner by determination of near atomic resolution structures of multiple prion stains by cryogenic electron microscopy. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In addition, we are proud of our long term field studies on the epidemic human prion disease kuru in the Eastern Highlands of Province of Papua New Guinea (PNG), in collaboration with the PNG Institute for Medical Research and the affected communities, which led to major insights including establishing the range of possible incubation periods of human prion infections (documenting cases with incubations over 50 years) and discovery of a novel prion protein variant selected by the epidemic which we demonstrated provides complete protection against prion infection and disease and the molecular structural basis of which we have recently characterised. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To what extent do you think we are entering a new era when it comes to developing drugs that could be used to prevent, or even reverse, neurodegenerative diseases?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Thankfully we are entering a time when disease-modifying treatments for neurodegenerative diseases are becoming feasible and indeed first-generation agents have arrived, but we cannot yet prevent, halt or reverse neurodegeneration.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Our own work validating cellular prion protein as a therapeutic target led us to develop a humanised monoclonal antibody with high affinity for cellular PrP and this has been used to treat six patients with CJD at UCLH. We consider the encouraging results justify a formal clinical trial and are seeking funding support for this at present.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Our therapeutic strategy has been to target normal cellular PrP itself, the substrate for prion propagation, and not the disease-related assemblies of misfolded PrP that accumulate during disease. We reasoned, given the diversity of these species, that drugs binding prions themselves would lead to the rapid development of resistance and indeed this has been shown to be the case with drugs developed elsewhere.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There may be important lessons here for other neurodegenerative diseases. For example, this may be critical in determining whether monoclonal antibody drugs targeting amyloid-beta fibrils or other assemblies, which also exist as structural polymorphs, have a sustained therapeutic effect or result in strain selection and evolution of resistant sub-strains as in prion diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">A number of pharmaceutical and biotech companies are however developing gene targeting methods, conceptually analogous to those we demonstrated many years ago block prion pathogenesis, to reduce expression of proteins implicated in various neurodegenerative diseases. Given the complexity and diversity of AD, in which multiple proteinopathies are involved, it is likely that effective treatments are going to require a cocktail of drugs hitting multiple targets.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Another key consideration is the importance of accurate diagnosis and early treatment, not only for the obvious need to intervene before irreversible brain cell loss has occurred, but because at the stage where significant cell death (with release of toxic materials) is occurring, these secondary non-specific neurodegenerative processes may dominate and be unresponsive to the specific targeted therapies. The ultimate aim must be to identify these pathogenic processes very early (ideally pre-clinically) and intervene to delay, and eventually prevent, clinical progression or onset.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ucl.ac.uk/brain-sciences/dementia-ucl-priority/professor-john-collinge-tackling-prion-diseases</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Alzheimer's disease, iatrogenic transmission, what if?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease... </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** Singeltary comment PLoS *** </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Posted by flounder on 05 Nov 2014 at 21:27 GMT </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Background</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Methods</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Results</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Conclusions</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.plosone.org/annotation/listThread.action?root=82860" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.plosone.org/annotation/listThread.action?root=82860</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://betaamyloidcjd.blogspot.com/2021/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://betaamyloidcjd.blogspot.com/2021/</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">IN CONFIDENCE</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">5 NOVEMBER 1992</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There are also results to be made available shortly </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(1) concerning a farmer with CJD who had BSE animals, </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(2) on the possible transmissibility of Alzheimer’s and </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/nature15369</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary Comment at very bottom of this Nature publishing;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">where have we all heard this before? it's been well documented via the BSE Inquiry. have they not learned a lesson from the last time?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">we have seen this time and time again in England (and other Country's) with the BSE mad cow TSE Prion debacle.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">That 'anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients ? </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">who got pooled extracts injected from thousands of cadavers ? were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find 'alarming' is pathetic.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Sounds like the journalists had it right in the first place: 'Alzheimer's may be a transmissible infection' in The Independent to 'You can catch Alzheimer's' in The Daily Mirror or 'Alzheimer's bombshell' in The Daily Express</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">if not for the journalist, the layperson would not know about these important findings.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer's, the price of poker goes up drastically.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">so, who makes that final decision, and how many more decades do we have to wait?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">in my opinion, it's one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it's bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer's of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer's and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary Comment at very bottom of this Nature publishing;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369#article-comments" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/nature15369#article-comments</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/nature15369</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Saturday, March 18, 2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Autoclave treatment fails to completely inactivate DLB alpha-synuclein seeding activity </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Prusiner et al, then and now!</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.nejm.org/doi/full/10.1056/NEJM200105173442006" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJM200105173442006</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.pnas.org/doi/10.1073/pnas.2220984120" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.pnas.org/doi/10.1073/pnas.2220984120</a></span></p><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;">2001 Singeltary on CJD</div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><br style="outline: none !important;" /></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">February 14, 2001</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Terry S. Singeltary, Sr</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Author Affiliations</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Terry S. Singeltary, retired (medically), CJD WATCH</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Submitted March 26, 2003</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">August 10, 2009</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Greetings,</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">But, while sub-clinical, how many can one exposed human infect? </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">please see history, and the ever evolving TSE science to date ;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Saturday, June 13, 2009</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary 2000</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">BMJ 2000; 320 doi: </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">https://doi.org/10.1136/bmj.320.7226.8/b</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> (Published 01 January 2000) Cite this as: BMJ 2000;320:8</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">02 January 2000 Terry S Singeltary retired</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Rapid Response: </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Something else I find odd, page 16;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">A few more factors to consider, page 15;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To be continued...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Terry S. Singeltary Sr. Bacliff, Texas USA</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Competing interests: No competing interests</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Tracking spongiform encephalopathies in North America</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Xavier Bosch</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Available online 29 July 2003. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Volume 3, Issue 8, August 2003, Page 463 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Volume 3, Number 8 01 August 2003</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Newsdesk</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Tracking spongiform encephalopathies in North America</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Xavier Bosch</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary 2007</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">by Philip Yam </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Revisiting Sporadic CJD</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">www.madcow.org</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">223</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">www.vegsource.com</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">224 CHAPTER 14</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Laying Odds 225</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">226 CHAPTER 14</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">A Case for Undercounting</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Laying Odds 227</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a onein-a-million lottery, it’s more like one-in-2.5-million for AfricanAmericans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">SNIP...SEE FULL TEXT;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Singeltary Submission SEAC 2007</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a></span></p><div style="outline: none !important;"><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><br style="outline: none !important;" /></div></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">2023</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">United States of America - Bovine spongiform encephalopathy - Immediate notification<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">GENERAL INFORMATION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">COUNTRY/TERRITORY OR ZONE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">COUNTRY/TERRITORY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ANIMAL TYPE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TERRESTRIAL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DISEASE CATEGORY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Listed disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EVENT ID 5067</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DISEASE Bovine spongiform encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CAUSAL AGENT Bovine spongiform encephalopathy prion, atypical strain, L-type</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">GENOTYPE / SEROTYPE / SUBTYPE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">START DATE 2023/05/15</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REASON FOR NOTIFICATION Recurrence of an eradicated disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DATE OF LAST OCCURRENCE 2018/08/28</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONFIRMATION DATE 2023/05/18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EVENT STATUS On-going</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">END DATE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SELF-DECLARATION NO</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REPORT INFORMATION REPORT NUMBER Immediate notification</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REPORT ID IN_160986</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REPORT REFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REPORT DATE 2023/05/23</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">REPORT STATUS Validated</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NO EVOLUTION REPORT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EPIDEMIOLOGY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SOURCE OF EVENT OR ORIGIN OF INFECTION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Spontaneous mutation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unknown or inconclusive</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EPIDEMIOLOGICAL COMMENTS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">As part of the United States’ targeted surveillance program for bovine spongiform encephalopathy (BSE), a case of atypical BSE was identified in a nine year old beef type cow. This atypical BSE case was classified as L-type. In over 25 years of surveillance, the six native cases detected in the United States have all been atypical cases. The identified animal did not enter any food supply channels and at no time presented a risk to human health. Specified risk material removal and ruminant-to-ruminant feed bans continue to be effectively applied.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">QUANTITATIVE DATA SUMMARY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MEASURING UNIT</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Animal</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Species Susceptible Cases Deaths Killed and Disposed of Slaughtered/ Killed for commercial use Vaccinated</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cattle (DOMESTIC)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NEW-1-1--TOTAL-1-1--</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DIAGNOSTIC DETAILS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CLINICAL SIGNS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">YES</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">METHOD OF DIAGNOSTIC</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnostic test</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Test name Laboratory Species sampled Number of outbreaks sampled First result date Latest result date Result</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Immunohistochemistry (IHC) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 2023/05/22 2023/05/22 Positive</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Antigen capture enzyme-linked immunosorbent assay (AC-ELISA) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023/05/18 2023/05/18 Positive</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Antigen detection Western blot (Ag Western blot) National Veterinary Services Laboratories (NVSL), Ames, Iowa Cattle 1 2023/05/18 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2023/05/18 Positive</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONTROL MEASURES AT EVENT LEVEL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONTROL MEASURES AT EVENT LEVEL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DOMESTIC ANIMALS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WILD ANIMALS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Official disposal of carcasses, by-products and waste</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Applied</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Screening</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Applied</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Traceability</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Applied</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NEW OUTBREAKS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">OB_118941 - TENNESSEE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">OUTBREAK REFERENCE -</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">START DATE 2023/05/15</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">END DATE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DETAILED CHARACTERISATION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">FIRST ADMINISTRATIVE DIVISION Tennessee</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SECOND ADMINISTRATIVE DIVISION Davidson</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THIRD ADMINISTRATIVE DIVISION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EPIDEMIOLOGICAL UNIT Farm</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">LOCATION Tennessee</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Latitude, Longitude 36.165 , -86.784</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(Approximate location) OUTBREAKS IN CLUSTER</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MEASURING UNIT Animal</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">AFFECTED POPULATION DESCRIPTION</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nine year old beef type cow. Note: Tracing efforts to date place this animal’s origin in the State of Tennessee. Location coordinates are approximate to the Tennessee State Capitol.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Species Susceptible Cases Deaths Killed and Disposed of Slaughtered/Killed for commercial use Vaccinated</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cattle (DOMESTIC)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NEW-1-1--TOTAL-1-1--</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">METHOD OF DIAGNOSTIC</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Diagnostic test</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONTROL MEASURES DIFFERENT FROM EVENT LEVEL MEASURES NOT IMPLEMENTED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ADDITIONAL MEASURES</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/5067</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">snip...see full text and more here;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Wednesday, May 24, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Atypical BSE detected St. Gallen Bern, 13.07.2023 case 2 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.blv.admin.ch/blv/de/home/dokumentation/nsb-news-list.msg-id-96688.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.blv.admin.ch/blv/de/home/dokumentation/nsb-news-list.msg-id-96688.html</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">WAHIS, WOAH, OIE, REPORT Switzerland BSE 2023/03/08 case 1 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4962" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4962</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Wednesday, May 24, 2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">WAHIS, WOAH, OIE, USA BSE</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/5067" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/5067</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Monday, March 20, 2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">WAHIS, WOAH, OIE, REPORT UK BSE</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4977" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4977</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">BRAZIL BSE START DATE 2023/01/18</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4918" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4918</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">SPAIN BSE START DATE 2023/01/21</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4888" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4888</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">NETHERLANDS BSE START DATE 2023/02/01</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p2" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877p1" style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydpecec48f4yiv1300318425ydpa9bd8ecdyiv9679883877s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wahis.woah.org/#/in-review/4876" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wahis.woah.org/#/in-review/4876</a></span></p><div style="font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><br style="outline: none !important;" /></div></div></div><div style="outline: none !important;">'Spontaneous mutation'<br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">***> PLEASE NOTE!</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">spontaneous/sporadic CJD in 85%+ of all human TSE, or spontaneous BSE in cattle, is a pipe dream, dreamed up by USDA/OIE et al, that has never been proven. let me repeat, NEVER BEEN PROVEN FOR ALL HUMAN OR ANIMAL TSE I.E. ATYPICAL BSE OR SPORADIC CJD! please see;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="color: black; outline: none !important;">OIE Conclusions on transmissibility of atypical BSE among cattle</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><a href="https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">34 Scientific Commission/September 2019</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">3. Atypical BSE</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">4. Definitions of meat-and-bone meal (MBM) and greaves</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;">The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion<br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article</a><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/</a></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/</a><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><div style="outline: none !important;">Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Affiliations expand</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PMID: 21266763</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://pubmed.ncbi.nlm.nih.gov/21266763/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/21266763/</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full text;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://www.niid.go.jp/niid/images/JJID/64/81.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.niid.go.jp/niid/images/JJID/64/81.pdf</a><br style="outline: none !important;" /></div></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;">This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.</div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094</a><br style="outline: none !important;" /></div><div style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><div style="outline: none !important;">Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: <a href="https://doi.org/10.3389/fvets.2019.00430" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.3389/fvets.2019.00430</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=363305</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=360665</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=373668</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://bmcvetres.biomedcentral.com/articles/10.1186/s12917-020-02611-0</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1985</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506002258/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506001031/http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506024922/http://www.bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf</a></div></div></div><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Friday, May 19, 2023 | 04:12pm NASHVILLE — The Tennessee State Veterinarian is confirming a case of atypical bovine spongiform encephalopathy (BSE) in a cow with ties to Tennessee.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The cow appeared unwell after arriving at a packing company in South Carolina. In alignment with the United States Department of Agriculture’s BSE surveillance program, the animal was isolated and euthanized. It did not enter the food supply. Preliminary investigation has determined the cow originated in southeast Tennessee.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">“We are working closely with our federal partners and animal health officials in South Carolina for this response,” State Veterinarian Dr. Samantha Beaty said. “That includes determining prior owners and locations where the affected cow lived in Tennessee and tracing siblings and offspring for testing.”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE is a chronic degenerative disease affecting the central nervous system of cattle. It is caused by an abnormal prion protein. The atypical form occurs spontaneously at very low levels in all cattle populations, particularly in older animals. Atypical BSE poses no known risk to human health. It is different from the classical form of BSE, which has not been detected in the U.S. since 2003.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE is not contagious and therefore is not spread through contact between cattle or with other species. There is no treatment for or vaccine to prevent BSE. The U.S. has a strong surveillance program in place for early detection and to prevent suspect cattle from entering the food supply chain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cattle owners are always advised to monitor their herds for health. Cattle affected by BSE may display changes in temperament, abnormal posture, poor coordination, decreased milk production, or loss of condition without noticeable loss of appetite. Owners should report any herd health concerns to their veterinarian or to the State Veterinarian’s office at <span dir="ltr" style="outline: none !important;">615-837-5120</span>.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Tennessee Department of Agriculture Animal Health Division is responsible for promoting animal health in Tennessee. The State Veterinarian’s office seeks to prevent the spread of disease through import and movement requirements, livestock traceability, disaster mitigation, and the services of the C.E. Kord Animal Health Diagnostic Laboratory. The division collaborates with other health-related stakeholders, academic institutions, and extension services to support One Health, an initiative to improve health for people and animals.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tn.gov/agriculture/news/2023/5/19/state-veterinarian-alerts-cattle-owners-to-disease-detection.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">USDA Announces Atypical L-Type Bovine Spongiform Encephalopathy BSE Detection<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div style="outline: none !important;">The U.S. Department of Agriculture (USDA) is announcing an atypical case of Bovine Spongiform Encephalopathy (BSE), a neurologic disease of cattle, in an approximately five-year-old or older beef cow at a slaughter plant in South Carolina. This animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the United States. Given the United States’ negligible risk status for BSE, we do not expect any trade impacts as a result of this finding. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USDA Animal and Plant Health Inspection Service’s (APHIS) National Veterinary Services Laboratories (NVSL) confirmed that this cow was positive for atypical L-type BSE. The animal was tested as part of APHIS’s routine surveillance of cattle that are deemed unsuitable for slaughter. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> This is the nation’s 7th detection of BSE. Of the six previous U.S. cases, the first, in 2003, was a case of classical BSE in a cow imported from Canada; the rest have been atypical (H- or L-type) BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The World Organization for Animal Health (WOAH) recognizes the United States as negligible risk for BSE. As noted in the WOAH guidelines for determining this status, atypical BSE cases do not impact official BSE risk status recognition as this form of the disease is believed to occur spontaneously in all cattle populations at a very low rate. Therefore, this finding of an atypical case will not change the negligible risk status of the United States, and should not lead to any trade issues. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The United States has a longstanding system of interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials - or the parts of an animal that would contain BSE should an animal have the disease - from all animals presented for slaughter. The second safeguard is a strong feed ban that protects cattle from the disease. Another important component of our system - which led to this detection - is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">More information about this disease is available in the BSE factsheet.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"># </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May 2, 2023, i submitted this to the USDA et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Docket No. APHIS–<span dir="ltr" style="outline: none !important;">2023–0027</span> Notice of Request for Revision to and Extension of Approval of an Information Collection; National Veterinary Services Laboratories; Bovine Spongiform Encephalopathy Surveillance Program Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ONLY by the Grace of God, have we not had a documented BSE outbreak, that and the fact the USDA et al are only testing 25K cattle for BSE, a number too low to find mad cow disease from some 28.9 million beef cows in the United States as of Jan. 1, 2023, down 4% from last year. The number of milk cows in the United States increased to 9.40 million. U.S. calf crop was estimated at 34.5 million head, down 2% from 2021. Jan 31, 2023. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ALL it would take is one BSE positive, yet alone a handful of BSE cases, this is why the Enhanced BSE was shut down, and the BSE testing shut down to 25k, and the BSE GBRs were replaced with BSE MRRs, after the 2003 Christmas Mad cow, the cow that stole Christmas, making it legal to trade BSE, imo. </div></div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div dir="ltr" style="outline: none !important;"><a href="https://www.regulations.gov/comment/APHIS-2023-0027-0002" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/APHIS-2023-0027-0002</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">see full submission;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf</a></div></div></div></div><br style="outline: none !important;" /></div><div style="outline: none !important;"><div dir="ltr" style="color: black; outline: none !important;"><span style="color: #141414; font-family: Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif; outline: none !important;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</span><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><span style="color: #141414; font-family: Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="color: black; outline: none !important;"><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a><span style="color: #141414; font-family: Helvetica, Roboto, Oxygen, Ubuntu, Cantarell, sans-serif; outline: none !important;"><br style="outline: none !important;" /></span></div><div dir="ltr" style="color: black; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: black; outline: none !important;"><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;">BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">1st a bit of history </div></div></div><div style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p4" style="font-size: 30.2px; font-stretch: normal; line-height: normal; margin: 0px 0px 3px; outline: none !important;"><span style="font-family: UICTFontTextStyleBody; font-size: 30.24px; font-weight: bold; outline: none !important;">Chronic Wasting Disease in Texas</span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none !important;">A Real Disease with Proven Impacts</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Produced by a coalition of concerned hunters, landowners, & conservationists (last update 08/2023)</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Since 2012, CWD has been detected in wild deer in just 7 counties in Texas and is only established in the western panhandle and far west Texas.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In that same period of time, captive deer breeders have exposed almost half of Texas counties to CWD. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://bit.ly/3xL16Gm" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://bit.ly/3xL16Gm</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">As of August 2023, 116 Texas counties have received possibly infected breeder deer that cannot be located, putting more than 140,000 landowners at risk of the disease. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://bit.ly/3xL16Gm" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://bit.ly/3xL16Gm</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">“It is interesting to note that, in 2001, the State of Texas shifted its deer management strategies toward the same leanings that Kroll has suggested for Wisconsin. In Texas, the change was brought about via heavy lobbying from the high-fence deer ranching industry. This pressure helped convince the Texas Parks and Wildlife to change their regulations and allow private landowners to select the own deer biologists.”</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.texasmonthly.com/story/which-side-fence-are-you" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.texasmonthly.com/story/which-side-fence-are-you</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">2012 “For 10 years, Texas has had an aggressive Chronic Wasting Disease prevention and monitoring program. Wildlife agency regulations prohibit importing deer into the state, and the agency has tested more than 26,000 hunter-taken deer and 7,400 animals from the captive-deer industry. None of those deer tested positive.”</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.chron.com/news/houston-texas/article/Brain-eating-disease-found-in-Texas-deer-3697731.php" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.chron.com/news/houston-texas/article/Brain-eating-disease-found-in-Texas-deer-3697731.php</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Texas CWD Surveillance Positives</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/huntwild/wild/diseases/cwd/positive-cases/listing-cwd-cases-texas.phtml#texasCWD</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Counties where CWD Exposed Deer were Released</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/documents/257/CWD-Trace-OutReleaseSites.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Number of CWD Exposed Deer Released by County</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"><a href="https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/documents/258/CWD-Trace-OutReleaseSites-NbrDeer.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Chronic Wasting Disease CWD Captive Herds updated April 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"><a href="https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Chronic Wasting Disease CWD Captive Herds updated April 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"><a href="https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">CWD poses a significant threat to the future of hunting in Texas. Deer population declines of 45 and 50 percent have been documented in Colorado and Wyoming. A broad infection of Texas deer populations resulting in similar population impacts would inflict severe economic damage to rural communities and could negatively impact land markets. Specifically, those landowners seeking to establish a thriving herd of deer could avoid buying in areas with confirmed CWD infections. As they do with anthrax-susceptible properties, land brokers may find it advisable to inquire about the status of CWD infections on properties that they present for sale. Prospective buyers should also investigate the status of the wildlife on prospective properties. In addition, existing landowners should monitor developments as TPWD crafts management strategies to identify and contain this deadly disease. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Dr. Gilliland (c-gilliland@tamu.edu) is a research economist with the Texas Real Estate Research Center at Texas A&M University.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.recenter.tamu.edu/articles/tierra-grande/oh-deer-2314" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.recenter.tamu.edu/articles/tierra-grande/oh-deer-2314</a></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s6" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important; text-decoration-line: underline;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">TPWD Executive Order No. 23-003 CWD Emergency Rules Adopted for Movement of Breeder Deer </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Executive Orders</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Executive Order No. 23-003</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Date: July 24, 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The Executive Director finds that additional discoveries of CWD in free-ranging white-tailed deer within deer breeding facilities regulated under Parks and Wildlife Code, Chapter 43, Subchapter L and regulations adopted pursuant to that subchapter (31 TAC Chapter 65, Subchapters B and T) constitute an immediate danger to the white-tailed deer and mule deer resources of Texas and that the adoption of rules on an emergency basis with fewer than 30 days’ notice is necessary to address an immediate danger.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://tpwd.texas.gov/publications/executive_orders/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/publications/executive_orders/</a></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">15 minute mark video shows sick deer with cwd, and this deer DIED FROM CWD, IT'S DOCUMENTED, commentator says ''so if anyone every tells you, that a deer has never died from CWD, think of this picture, because the Wisconsin Veterinary Lab told us, what when they looked at her sample under a microscope, she was the hottest animal they had ever seen, and that's in terms of the fluorescents that comes off the slide when the look at it, so, a lot of Prion in her system.''</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">see much more about 2 hours long...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.youtube.com/watch?v=O3CAI-EwlgM" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.youtube.com/watch?v=O3CAI-EwlgM</a></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">TEXAS BREEDER DEER ESCAPEE WITH CWD IN THE WILD, or so the genetics would show?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">OH NO, please tell me i heard this wrong, a potential Texas captive escapee with cwd in the wild, in an area with positive captive cwd herd?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">apparently, no ID though. tell me it ain't so please...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">23:00 minute mark</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">''Free Ranging Deer, Dr. Deyoung looked at Genetics of this free ranging deer and what he found was, that the genetics on this deer were more similar to captive deer, than the free ranging population, but he did not see a significant connection to any one captive facility that he analyzed, so we believe, Ahhhhhh, this animal had some captive ahhh, whatnot.''</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://youtu.be/aoPDeGL6mpQ?t=1384" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://youtu.be/aoPDeGL6mpQ?t=1384</a></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Commission Agenda Item No. 5 Exhibit B</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">DISEASE DETECTION AND RESPONSE RULES</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">PROPOSAL PREAMBLE</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">1. Introduction. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> A third issue is the accuracy of mortality reporting. Department records indicate that for each of the last five years an average of 26 deer breeders have reported a shared total of 159 escapes. Department records for the same time period indicate an average of 31 breeding facilities reported a shared total of 825 missing deer (deer that department records indicate should be present in the facility, but cannot be located or verified). </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://tpwd.texas.gov/business/feedback/meetings/2022/1104/agenda/item.phtml?item=5" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tpwd.texas.gov/business/feedback/meetings/2022/1104/agenda/item.phtml?item=5</a></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">On January 21, 2017 a tornado took down thousands of feet of fence for a 420-acre illegal deer enclosure in Lamar County that had been subject to federal and state investigation for illegally importing white-tailed deer into Mississippi from Texas (a CWD positive state). Native deer were free to move on and off the property before all of the deer were able to be tested for CWD. Testing will be made available for a period of three years for CWD on the property and will be available for deer killed within a 5-mile radius of the property on a voluntary basis. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.mdwfp.com/media/254796/2016-17-deer-report.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.mdwfp.com/media/254796/2016-17-deer-report.pdf</a><br style="outline: none !important;" /></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><div style="outline: none !important;"><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Deep Throat to Singeltary BSE Mad Cow 2001 to 2023</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">I remember what “deep throat” told me about Scrapie back around 2001, I never forgot, and it seems it’s come to pass;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Confidential!!!!</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">---end personal email---end...tss </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">(I never knew who this person was, but got me into the U.S. BSE Emergency 50 State conference call back 2001, and we corresponded for years about BSE TSE Prion, have not heard from in over a decade, but they were on the inside looking out. You can believe this or not, but this was real, i don’t make this stuff up…plus my endeavors to get those 1 million cattle tested for BSE failed. There was an ENHANCED BSE SURVEILLANCE put forth after 2003, we pushed for it, but it was abruptly shut down after the atypical BSE cases were popping up…a bit of history for anyone interested…terry)</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss) </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at..... </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!! And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall... </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!" </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.) </span></p><div style="outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp2c8370d6yiv4469501343s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div></div>END...TSS</div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;">and so it was...</div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Rapid recontamination of a farm building occurs after attempted prion removal</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">First published: 19 January 2019 <a href="https://doi.org/10.1136/vr.105054" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1136/vr.105054</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">snip...</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">***>This is very likely to have parallels with control efforts for CWD in cervids.</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://pubmed.ncbi.nlm.nih.gov/30602491/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/30602491/</a> </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Front. Vet. Sci., 14 September 2015 | <a href="https://doi.org/10.3389/fvets.2015.00032" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.3389/fvets.2015.00032</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">172. Establishment of PrPCWD extraction and detection methods in the farm soil</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Conclusions: Our studies showed that PrPCWD persist in 0.001% CWD contaminated soil for at least 4 year and natural CWD-affected farm soil. When cervid reintroduced into CWD outbreak farm, the strict decontamination procedures of the infectious agent should be performed in the environment of CWD-affected cervid habitat.</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">THE tse prion aka mad cow type disease is not your normal pathogen. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">you cannot cook the TSE prion disease out of meat. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">the TSE prion agent also survives Simulated Wastewater Treatment Processes. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">you can bury it and it will not go away. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">it’s not your ordinary pathogen you can just cook it out and be done with. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">***> that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8 </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">***> Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Laboratory of Central Nervous System Studies, National Institute of </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Neurological Disorders and Stroke, National Institutes of Health, </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Bethesda, MD 20892. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">PMID: <span dir="ltr" style="outline: none !important;">8006664</span> [PubMed - indexed for MEDLINE] </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="http://www.pnas.org/content/97/7/3418.full" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.pnas.org/content/97/7/3418.full</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Detection of protease-resistant cervid prion protein in water from a CWD-endemic area </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">THURSDAY, FEBRUARY 28, 2019 </div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">BSE infectivity survives burial for five years with only limited spread</div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf</a></div><div style="font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Volume 15, Number </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">5—May</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> 2009</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s7" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic; outline: none !important;">Research</span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s7" style="font-family: UICTFontTextStyleItalicBody; font-size: 18.36px; font-style: italic; outline: none !important;"><br style="outline: none !important;" /></span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none !important;">Chronic Wasting Disease Prions in Elk Antler Velvet</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Rachel C. Angers<a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article#fn1" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">1</a>, Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O’Rourke, Aru Balachandran, and Glenn C. Telling</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p6" style="font-stretch: normal; line-height: normal; margin: 9px 0px 8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article#comment" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"><img alt="unknown.gif" class="yiv1585712922ydp9f180146yiv7216723629" src="https://apis.mail.aol.com/ws/v3/mailboxes/@.id==VjN-ce6z-DxIu_S2g8qZB3zEhBzmZgrr3THL4IwkE1wVplQCkvSg4OstfRBZyImsL89EhqbiNPt5J57JQHOERly2uw/messages/@.id==AHVXZ6RoFdN_ZQoJNAt5gKjaTwI/content/parts/@.id==2/refresh?appid=AolMailNorrin&ymreqid=d41d8cd9-8f00-b204-1ce2-c20003019b00" style="outline: none !important; text-indent: -9999px;" /></a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Author affiliations: University of Kentucky Medical Center, Lexington, Kentucky, USA (R.C. Angers, T.S. Seward, D. Napier, M. Green, G.C. Telling); Colorado State University, Fort Collins, Colorado, USA (E. Hoover, T. Spraker); US Department of Agriculture, Pullman, Washington, USA (K. O’Rourke); Canadian Food Inspection Agency, Ottawa, Ontario, Canada (A. Balachandran); 1Current affiliation: Medical Research Council Laboratory of Molecular Biology, Cambridge, UK.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p5" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s4" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none !important;">Abstract</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the apical skin layer covering the growing antler (antler velvet) by using CWD-susceptible transgenic mice and protein misfolding cyclic amplification. Our finding of prions in antler velvet of CWD-affected elk suggests that this tissue may play a role in disease transmission among cervids. Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. The fact that CWD prion incubation times in transgenic mice expressing elk prion protein are consistently more rapid raises the possibility that residue 226, the sole primary structural difference between deer and elk prion protein, may be a major determinant of CWD pathogenesis.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/15/5/08-1458_article</a></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">TUESDAY, MAY 11, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusion</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CREUTZFELDT JAKOB DISEASE: A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">i was warning England and the BSE Inquiry about just this, way back in 1998, and was ask to supply information to the BSE Inquiry. for anyone that might be interested, see;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ABOUT that deer antler spray and CWD TSE PRION... I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease. just saying...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sender: "Patricia Cantos"</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: "Terry S Singeltary Sr. (E-mail)"</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: Your submission to the Inquiry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Fri, 3 Jul 1998 10:10:05 +0100 3 July 1998</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Mr Terry S Singeltary Sr. E-Mail: Flounder at <span dir="ltr" style="outline: none !important;">wt.net</span> Ref: E2979</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Mr Singeltary, Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died. As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments. Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><span dir="ltr" style="outline: none !important;">http://www.bse.org.uk</span>.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on... snip...end...tss</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year previously and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. both cases confirmed. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">kind regards, terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS IPLEX, mad by standard process; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. also; what about potential mad cow candy bars ? see their potential mad cow candy bar list too... THESE are just a few of MANY of just this ONE COMPANY...TSS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''So, in sum, dietary supplements sold in the United States often contain ruminant tissues from undisclosed sources. Personally, I am rather squeamish and I don't think I would be eating prostate or testicle or pituitary, but I am also a little bit wary of consuming products with those glands, not just out of personal repugnance but simply out of a health concern.'' </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DEPARTMENT OF HEALTH AND HUMAN SERVICES FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE Friday, January 19, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">15 Open Public Hearing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">16 DR. FREAS: We are opening the open public hearing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 now. We have received one response to speak in this</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">18 afternoon's open public hearing. That is from Dr. Scott</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">19 Norton. If Dr. Norton is here, would you please come</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">20 forward. You can either use the podium or the microphone,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">21 whichever is your choice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 DR. NORTON: I am Scott Norton and I am a</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">23 physician in the Washington D.C. area. I am here speaking</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">24 as a private citizen today.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">25 I first became concerned about the presence of 231</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 tissues from ruminant animals in dietary supplements about</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2 six months ago and expressed my concern in a letter that was 3 published in New England Journal of Medicine in July of Year 4 2000. 5 A couple of the products that I had looked at, and 6 examined their labels, that raised these concerns I brought 7 in right here. I will just read some of the organs that are 8 found in one that is called Male Power. Deer antler, 9 pancreas, orchic--despite what we just heard that the FDA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">10 prefers the term "testicular tissue" to be written on the</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">11 labels, I have never seen a dietary supplement say</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">12 "testicle." They always say "orchis" or "orchic" which may</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">13 sound rather flowery to the etymologically impaired--thymus,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">14 adrenal, heart, lymph node, prostate, spleen and pituitary.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">15 There are actually seventeen organs in that particular</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">16 product.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 There is another product that is called Brain</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">18 Nutrition that tells us that it is vitamins and minerals</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">19 essential for important brain function. It does not mention</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">20 that there is any glandulars on at least the bold print. 21 But if you look at the small print on the back, we learn</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 that it has brain extract and pituitary extract, raw, in</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">23 there.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">24 We know that many of the organs that can be found</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">25 in the dietary supplements do fall in that list of organs</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">232</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 that are suspect for contamination with TSEs, the labels, in 2 nearly all cases, identify neither the animal source nor the 3 geographic location from which the organs were derived. I 4 have seen one line that did specify from New Zealand cattle 5 but no other manufacturer will list either the species or 6 the geographic location. 7 The FDA's and the USDA's import alerts that we 8 just learned about prohibit the use of these organs in 9 foods, medicines and medical devices. But my reading of the</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">10 alert, 17-04, suggests that DSHEA does allow some loopholes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">11 for these tissues to possible slip in.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">12 I will just read <span dir="ltr" style="outline: none !important;">from 17-04</span> that we heard. On the</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">13 first page, it says that, "This alert does not establish any</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">14 obligations on regulated entities." I love seeing</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">15 legislation that starts out with that caveat.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">16 Then it says, further, "The USDA regulations do</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 not apply to bovine-derived materials intended for human</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">18 consumption as finished dietary supplements." We also learn</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">19 that the prohibition, or the import alert, is limited to</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">20 bulk lots of these tissues, completed tissues, from BSE-</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">21 derived countries. It does not mention if it is not a bulk</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 import or if it is raw materials rather than finished</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">23 materials.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">24 Further, we know that it is strongly recommended</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">25 but not actually prohibited in the language here. So I have</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">233</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 not taken the assurances from that import alert that Dr. 2 Moore was trying to convey to us. 3 So, in sum, dietary supplements sold in the United 4 States often contain ruminant tissues from undisclosed 5 sources. Personally, I am rather squeamish and I don't 6 think I would be eating prostate or testicle or pituitary, 7 but I am also a little bit wary of consuming products with 8 those glands, not just out of personal repugnance but simply 9 out of a health concern.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">10 So my question to the advisory committee is this;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">11 is my caution reasonable and, if it is, should we take</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">12 further efforts to inform, or even protect, the American</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">13 public from such exposure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><span dir="ltr" style="outline: none !important;">14 I was curious about Dr.</span> Moore's remarks. I sensed</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">15 two messages. One was the initial reassurance that FDA has</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">16 the regulatory authority but then I also learned that it is</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 the manufacturer's responsibility to provide those 18 assurances, that the FDA doesn't actually inspect.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">19 I think that the FDA commissioners from Harvey</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">20 Wylie to David Kessler would say that that track record has</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">21 proven itself.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">22 Thank you very much.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">23 [Applause.]</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">24 DR. BROWN: Thanks, Dr. Norton. 25 Committee Discussion snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">17 But I think that we could exhibit some quite 18 reasonable concern about blood donors who are taking dietary 19 supplements that contain a certain amount of unspecified- 20 origin brain, brain-related, brain and pituitary material. 21 If they have done this for more than a sniff or something 22 like that, then, perhaps, they should be deferred as blood 23 donors. 24 That is probably worse than spending six months in 25 the U.K. 1/19/01 3681t2.rtf(845) page 501 <span dir="ltr" style="outline: none !important;">http://www.fda.gov/ohrms/dockets/ac/cber01.htm</span></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Advisory Committees: CBER 2001 Meeting Documents</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see actual paper;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><a href="http://web.archive.org/web/20030830045538/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030830045538/http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090120100414/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_03.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Given the science and the information presented, and given the comprehensive array of Natraflex quality control and chain-of-custody procedures, we believe that you can be confident, the our velvet-antler supplements are safe.</div><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20090120095558/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090120095558/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://web.archive.org/web/20090120094525/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_01.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090120094525/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t1_01.pdf</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="http://web.archive.org/web/20010805202621/http://www.fda.gov/bbs/topics/ANSWERS/2001/ANS01070.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20010805202621/http://www.fda.gov/bbs/topics/ANSWERS/2001/ANS01070.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Date: Sun, 12 Jan 2003 12:56:44 -0600</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sender: Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: Re: USA ruminant-to-ruminant feed ban warning letters ??? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">With these known facts about nutritional supplements, I think it imperative to include this potential route and source of TSE and warning in this article. Why was it not included? I lost my mother to hvCJD DOD 12/14/97, and I probably will not live long enough to know the route and source of her TSE. Exactly one year previously, to the day 12/14/96, my neighbor also lost his mother to sporadic CJD. Both of these cases were confirmed. but my neighbor's mother had been taking a nutritional supplement called IPLEX, made by Standard Process Co. Here is a listing of potentially TSE-tainted tissues: vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. The CDC came and took the pills, a few years later, I spoke with the late Dr. Gibbs and NIH/CDC and he told me that those particular pills did not show any infectivity with the testing techniques to date, but he also told me - </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1. That the testing techniques at that time may not be sufficient to pick up any 'low-level' infectivity. </div><div style="outline: none !important;">(so, if accumulation plays into this, this could play a big part). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2. She had been taking these type pills for years, could have been another batch. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There have been other people die of CJD that were taking these type nutritional supplements. So, my point being, I believe this to warrant a potential risk factor, one not to be ignored, especially in the USA where there are many known TSEs, and where there are many unknowns due to the lack of sufficient TSE testing in USA cattle, and especially since the new findings of Collinge et al, where BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I believe these findings to be of substantial importance: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DEPARTMENT OF HEALTH AND HUMAN SERVICES FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Friday, January 19, 2001 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Holiday Inn Bethesda Versailles I and II 8120 Wisconsin Avenue Bethesda, Maryland 2 PARTICIPANTS Paul W. Brown, M.D., Chairperson William Freas, Ph.D., Executive Secretary VOTING MEMBERS Ermias D. Belay, M.D. David C. Bolton, Ph.D. Donald S. Burke, M.D. Dean O. Cliver, Ph.D. Bruce M. Ewenstein, M.D., Ph.D. Peter G. Lurie, M.D. Pedro Piccardo, M.D. Stanley B. Prusiner, M.D. Raymond P. Roos, M.D. Elizabeth S. Williams, D.V.M., Ph.D. VOTING CONSULTANTS Linda A. Detwiler, D.V.M. David Gaylor, Ph.D. Paul R. McCurdy, M.D. Kenrad E. Nelson, M.D. NONVOTING CONSULTANT Susan Leitman, M.D. GUESTS Richard Davey, M.D. Louis Katz, M.D. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip... page 501 253 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1 DR. BOLTON: I have an additional question about 2 that. What is the assurance that additional locally sourced 3 tracheas are not added into that manufacturing process, thus 4 boosting the yield, if you will, but being returned to the 5 U.S. as being produced from U.S.-sourced raw material? 6 DR. McCURDY: Are there data to indicate how many 7 grams, or whatever, of infected brain are likely to infect 8 an organism, either animal or man, when taken orally? 9 DR. BROWN: If I am not mistaken, and I can be 10 corrected, I think a half a gram is enough in a cow, orally; 11 in other words, one good dietary-supplement pill. 12 DR. McCURDY: What I am driving at is the question 13 we are asked is really not do we wish to regulate these 14 things coming in. I think the statements about difficulties 15 in regulating things in the future or near future for new 16 regulations were probably accurate. 17 But I think that we could exhibit some quite 18 reasonable concern about blood donors who are taking dietary 19 supplements that contain a certain amount of unspecified- 20 origin brain, brain-related, brain and pituitary material. 21 If they have done this for more than a sniff or something 22 like that, then, perhaps, they should be deferred as blood 23 donors. 24 That is probably worse than spending six months in 25 the U.K. 1/19/01 3681t2.rtf(845) page 501 http://www.fda.gov/ohrms/dockets/ac/cber01.htm There has been a Nutritional Supplement mad cow warning letter circulating around since about 1990. Every year they issue the same letter to the manufactures asking them to please be sure they source their products from BSE-FREE countries. but we all know, the statement BSE-FREE, is a joke, especially in the USA. I sat in on the 50 state emergency BSE conference call, (uninvited guest) and I know for a fact the so-called 'pharmaceutical grade' bovine source herds, bovines that were to never be fed ruminant materials, the USA cannot for certain say that indeed these cattle have never ingested ruminant feed, in fact, some probably had. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Tue, 9 Jan 2001 16:49:00 -0800 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr." < flounder@wt.net > </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy < BSE-L@uni-karlsruhe.de > </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: BSE-L@uni-karlsruhe.de Bovine Spongiform Encephalopathy < BSE-L@UNI-KARLSRUHE.DE > </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Greetings List Members, I was lucky enough to sit in on this BSE conference call today and even managed to ask a question. that is when the trouble started. I submitted a version of my notes to Sandra Blakeslee of the New York Times, whom seemed very upset, and rightly so. "They tell me it is a closed meeting and they will release whatever information they deem fit. Rather infuriating." And I would have been doing just fine, until I asked my question. I was surprised my time to ask a question came so quickly. (understand, these are taken from my notes for now. the spelling of names and such could be off.) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[host Richard Barns] And now a question from Terry S. Singeltary of CJD Watch. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[TSS] Yes, Thank You. U.S. cattle - what kind of guarantee can you give for serum or tissue donor herds? [no answer, you could hear in the background, mumbling and "We can't. Have him ask the question again."] </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[host Richard] Could you repeat the question? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[TSS] U.S. cattle..what kind of guarantee can you give for serum or tissue donor herds? [not sure whom ask this] What group are you with? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[not sure who is speaking] Could you please disconnect Mr. Singeltary </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[TSS] You are not going to answer my question? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[not sure whom speaking] NO </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From this point, I was still connected, got to listen and tape the whole conference. at one point someone came on, a woman, and ask again; [unknown woman] What group are you with? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">[TSS] CJD Watch and my Mom died from hvCJD We are trying to tract down CJD and other human TSE's world wide. I was invited to sit in on this from someone inside the USDA/APHIS and that is why I am here. Do you intend on banning me from this conference now? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At this point the conference was turned back up, and I got to finish listening. They never answered or even addressed my one question, or even addressed the issue. BUT, I will try and give you a run-down for now, of the conference. IF i were another Country, I would take heed to my notes, BUT PLEASE do not depend on them. ask for transcript from: RBARNS@ORA.FDA.GOV 301-827-6906 He would be glad to give you one ;-) Rockville Maryland, Richard Barns Host </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><p class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">U.S. 50 State Emergency BSE Conference Call <span dir="ltr" style="outline: none !important;"><span dir="ltr" style="outline: none !important;">2001</span></span></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"></a></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp36890145yiv0316037590ydpcf2aa9bdyiv8106196362s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a></span></p></div></div></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Chronic Wasting Disease CWD Maine</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">How can the average person help prevent CWD?</span></p><ol class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984ol1" style="outline: none !important;"><li class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984li1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Be careful with commercial feeds.</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> In theory, prions from CWD-infected deer could be present in commercial deer and elk foods if they were formulated with rendering products containing CWD-infected meat and bone meal (MBM). In 1997, the U.S. FDA banned the use of ruminant (deer, cattle, sheep, goat) MBM from commercial feeds for ruminants. Assuming 100% FDA compliance, common commercial feeds used to supplement the diets of captive/farmed or wild cervids would now be CWD-free. However, we don’t know if MBM from CWD-infected deer or elk was ever incorporated into commercial ruminant feeds distributed in Maine prior to 1997, nor do we know if commercial feeds formulated for non-ruminants (horse, swine, poultry, dog, and cat) sometimes contain MBM from CWD-infected deer or elk. To safely feed cervids, use only commercially available products formulated specifically for ruminants, or use supplement-free whole grains like oats.</span></li></ol><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://www.maine.gov/ifw/fish-wildlife/wildlife/living-with-wildlife/diseases/chronic-wasting-disease.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.maine.gov/ifw/fish-wildlife/wildlife/living-with-wildlife/diseases/chronic-wasting-disease.html</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">“In 1997, the U.S. FDA banned the use of ruminant (deer, cattle, sheep, goat) MBM from commercial feeds for ruminants.”</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Greetings Maine Wildlife Officials on CWD,</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">I was reading over your page of information on cwd, and the above information is not correct exactly. It was just a voluntary ban for cervid.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Title:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> Scrapie as the potential origin of chronic wasting disease in white-tailed deer </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Author</span></p><table cellpadding="0" cellspacing="0" class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984t1" style="border-collapse: collapse; color: black; outline: none !important;"><tbody style="outline: none !important;"><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none !important;"></span><br style="outline: none !important;" /></p></td><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;">LAMBERT, ZOE - Oak Ridge Institute For Science And Education (ORISE)</span></p></td></tr><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none !important;"></span><br style="outline: none !important;" /></p></td><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;">WEST GREENLEE, HEATHER - Iowa State University</span></p></td></tr><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none !important;"></span><br style="outline: none !important;" /></p></td><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none !important; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=57305" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">Bian, Jifeng</a></span></p></td></tr><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none !important;"></span><br style="outline: none !important;" /></p></td><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none !important; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=55914" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">Cassmann, Eric</a></span></p></td></tr><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p8" style="font-family: Helvetica; font-size: 12px; font-stretch: normal; line-height: normal; margin: 0px; min-height: 13.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s8" style="outline: none !important;"></span><br style="outline: none !important;" /></p></td><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p10" style="color: #e4af0a; font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s10" style="font-family: UICTFontTextStyleBody; outline: none !important; text-decoration-line: underline;"><a href="https://www.ars.usda.gov/people-locations/person?person-id=44813" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">Greenlee, Justin</a></span></p></td></tr></tbody></table><table cellpadding="0" cellspacing="0" class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984t1" style="border-collapse: collapse; color: black; outline: none !important;"><tbody style="outline: none !important;"><tr style="outline: none !important;"><td class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984td1" style="border-color: rgb(170, 170, 170); border-style: solid; border-width: 1px; outline: none !important; padding: 1px 5px; word-break: normal;" valign="top"><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Submitted to:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> Chronic Wasting Disease Symposium Proceedings </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Publication Type:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> Abstract Only </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Publication Acceptance Date:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> 3/1/2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Publication Date:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> 5/30/2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Citation:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> Lambert, Z.J., West Greenlee, H.M., Bian, J., Cassmann, E.D., Greenlee, J.J. 2023. Scrapie as the potential origin of chronic wasting disease in white-tailed deer (abstract). Chronic Wasting Disease Symposium Proceedings. 4th International Chronic Wasting Disease Symposium, May 30-June 3, 2023, Denver, Colorado.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Interpretive Summary:</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p9" style="font-size: 17px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s11" style="font-family: UICTFontTextStyleEmphasizedBody; font-weight: bold; outline: none !important;">Technical Abstract:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s9" style="font-family: UICTFontTextStyleBody; outline: none !important;"> White-tailed deer (WTD) are susceptible to the scrapie agent from sheep after oronasal inoculation. However, results from western blotting these brainstems and lymph nodes are difficult to differentiate from WTD infected with chronic wasting disease (CWD). Tissues were examined via enzyme immunoassay (IDEXX), western blot, immunohistochemistry, and bioassay in cervidized mice (Tg12) in order to assess tissue phenotypes upon subsequent passage of the scrapie agent in WTD. All WTD were euthanized and necropsied following the development of clinical disease and were positive for abnormal prion protein by enzyme immunoassay. Western blotting of retinas from all WTD (second pass) resulted in a similar molecular profile as the retinas of WTD that were inoculated with the agent of scrapie from sheep (first pass). Immunohistochemical staining also was similar between inoculation groups and the initial passage from sheep, but different from WTD inoculated with the agent of CWD. Following bioassays in cervidized mice, all incubation periods were over 300 days, substantially longer than the approximately 200-day incubation period typical with CWD isolates. Based upon analysis of retinal tissues, it is possible to differentiate the agents of scrapie and CWD in WTD by both western blot and immunohistochemistry. Bioassay in cervidized mice further supports this based on incubation periods of WTD-scrapie being approximately twice that of WTD CWD</span></p></td></tr></tbody></table><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Submitted to:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> Chronic Wasting Disease Symposium Proceedings </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Publication Type:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> Abstract Only </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Publication Acceptance Date:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> 3/1/2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Publication Date:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> 5/30/2023 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Citation:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> Lambert, Z.J., West Greenlee, H.M., Bian, J., Cassmann, E.D., Greenlee, J.J. 2023. Scrapie as the potential origin of chronic wasting disease in white-tailed deer (abstract). Chronic Wasting Disease Symposium Proceedings. 4th International Chronic Wasting Disease Symposium, May 30-June 3, 2023, Denver, Colorado.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Interpretive Summary:</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;">Technical Abstract:</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> White-tailed deer (WTD) are susceptible to the scrapie agent from sheep after oronasal inoculation. However, results from western blotting these brainstems and lymph nodes are difficult to differentiate from WTD infected with chronic wasting disease (CWD). Tissues were examined via enzyme immunoassay (IDEXX), western blot, immunohistochemistry, and bioassay in cervidized mice (Tg12) in order to assess tissue phenotypes upon subsequent passage of the scrapie agent in WTD. All WTD were euthanized and necropsied following the development of clinical disease and were positive for abnormal prion protein by enzyme immunoassay. Western blotting of retinas from all WTD (second pass) resulted in a similar molecular profile as the retinas of WTD that were inoculated with the agent of scrapie from sheep (first pass). Immunohistochemical staining also was similar between inoculation groups and the initial passage from sheep, but different from WTD inoculated with the agent of CWD. Following bioassays in cervidized mice, all incubation periods were over 300 days, substantially longer than the approximately 200-day incubation period typical with CWD isolates. Based upon analysis of retinal tissues, it is possible to differentiate the agents of scrapie and CWD in WTD by both western blot and immunohistochemistry. Bioassay in cervidized mice further supports this based on incubation periods of WTD-scrapie being approximately twice that of WTD CWD.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"></a></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=401927</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s5" style="font-family: UICTFontTextStyleEmphasizedBody; font-size: 18.36px; font-weight: bold; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.nature.com/articles/srep11573 ">https://www.nature.com/articles/srep11573 </a><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032">https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032</a><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">CWD TO HUMANS, What If, has it already happened?</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">PART 2. TPWD CHAPTER 65. DIVISION 1. CWD</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">31 TAC §§65.82, 65.85, 65.88</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The Texas Parks and Wildlife Commission in a duly noticed meeting on May 25, 2023 adopted amendments to 31 TAC §§65.82, 65.85, and §65.88, concerning Disease Detection and Response, without changes to the proposed text as published in the April 21, 2023, issue of the Texas Register (48 TexReg 2048). The rules will not be republished.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Currently, there is scientific evidence to suggest that CWD has zoonotic potential; however, no confirmed cases of CWD have been found in humans.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57">https://www.sos.texas.gov/texreg/archive/June302023/Adopted%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html#57</a><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">17 DETECTION OF CHRONIC WASTING DISEASE PRIONS IN PROCESSED MEATS.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Rebeca Benavente1, Francisca Bravo1,2, Paulina Soto1,2, J. Hunter Reed3, Mitch Lockwood3, Rodrigo Morales1,2</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">1Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, USA. 2Universidad Bernardo O’Higgins, Santiago, Chile. 3Texas Parks and Wildlife, Austin, USA</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Abstract</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The zoonotic potential of chronic wasting disease (CWD) remains unknown. Currently, there are no known natural cases of CWD transmission to humans but increasing evidence suggests that the host range of CWD is not confined only to cervid species. Alarmingly, recent experimental evidence suggests that certain CWD isolates can induce disease in non-human primates. While the CDC strongly recommends determining CWD status in animals prior to consumption, this practice is voluntary. Consequently, it is plausible that a proportion of the cervid meat entering the human food chain may be contaminated with CWD. Of additional concern is that traditional diagnostic techniques used to detect CWD have relatively low sensitivity and are only approved for use in tissues other than those typically ingested by humans. In this study, we analyzed different processed meats derived from a pre-clinical, CWD-positive free-ranging elk. Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Our results show positive prion detection in all products. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***>To confirm the resilience of CWD-prions to traditional cooking methods, we grilled and boiled the meat products and evaluated them for any remnant PMCA seeding activity. Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Products tested included filets, sausages, boneless steaks, burgers, ham steaks, seasoned chili meats, and spiced meats. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> CWD-prion presence in these products were assessed by PMCA using deer and elk substrates. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Our results show positive prion detection in all products. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Results confirmed the presence of CWD-prions in these meat products suggesting that infectious particles may still be available to people even after cooking.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Our results strongly suggest ongoing human exposure to CWD-prions and raise significant concerns of zoonotic transmission through ingestion of CWD contaminated meat products. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">=====</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Samia Hannaoui1,2, Ginny Cheng1,2, Wiebke Wemheuer3, Walter Schulz-Schaeffer3, Sabine Gilch1,2, Hermann Schatzl1,2 1University of Calgary, Calgary, Canada. 2Calgary Prion Research Unit, Calgary, Canada. 3Institute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Further passage to cervidized mice revealed transmission with a 100% attack rate. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including the oral one. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">****> The disease manifested as atypical in macaques and initial transgenic mouse transmissions, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">***> Epidemiologic surveillance of prion disease among cervid hunters and people likely to have consumed venison contaminated with chronic wasting disease</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">=====</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">https://intcwdsympo.files.wordpress.com/2023/06/final-agenda-with-abstracts.pdf?force_download=true</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice were inoculated into </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice were used for second passage in bank voles.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice and bank voles. Intriguingly, protease-resistant PrP in the brain of </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Unprecedented in human prion disease, feces of CWD-inoculated </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">tg650</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> with fecal homogenates.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p3" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s3" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none !important;">U of M expert warns of increasing likelihood of CWD transmission to humans</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p3" style="font-size: 23.8px; font-stretch: normal; line-height: normal; margin: 0px 0px 4px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s3" style="font-family: UICTFontTextStyleBody; font-size: 23.76px; font-weight: bold; outline: none !important;"><br /></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.mprnews.org/people/cathy-wurzer" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">Cathy Wurzer</a></span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> and <a href="https://www.mprnews.org/people/gretchen-brown" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none !important;">Gretchen Brown</span></a>June 5, 2023 </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">1:30 PM</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Minnesota scientists have watched chronic wasting disease (CWD) — a fatal, neurological illness — kill deer and elk.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br /></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Now, they’re studying its potential to jump to humans.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br /></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The University of Minnesota’s <a href="https://www.cidrap.umn.edu/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none !important;">Center for Infectious Disease Research and Policy</span></a> has received more than $1.5 million in state money to start prepping for the possibility of CWD spreading to cows, pigs and possibly humans.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">He said transmission to humans has not yet been confirmed, but research suggests it is <a href="https://vet.ucalgary.ca/news/chronic-wasting-disease-may-transmit-humans-research-finds" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none !important;">increasingly likely</span></a> — especially as the disease continues to spread among deer and elk.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">“None of us want to believe this could happen,” he told MPR News host Cathy Wurzer. “But you know, as much as you hope it isn't going to happen, hope is not a strategy.”</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Current testing can be done <a href="https://www.mprnews.org/story/2023/03/16/state-agencies-not-sold-on-new-chronic-wasting-disease-test-option?gclid=Cj0KCQjwj_ajBhCqARIsAA37s0y2AZgeeWMW277iYJw23uHHH2jDJpN2ZbcYSOjrN7IurNYKbPaLygsaAnSGEALw_wcB" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="outline: none !important;">only if animals die or are killed</span></a>, and lymph nodes or brain matter is removed for testing to verify the disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">That means captive deer often aren’t tested until they die or show symptoms of the disease, and that’s often too late to stop the spread of the disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">And there aren’t yet adequate tests for humans, Osterholm said — let alone protocols in place if a human were to test positive for the disease.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Michael Osterholm, Ph.D. is a world-renowned epidemiologist who heads the center.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.mprnews.org/episode/2023/06/05/chronic-wasting-disease-in-humans-us-osterholm-prepares-for-whatif?" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.mprnews.org/episode/2023/06/05/chronic-wasting-disease-in-humans-us-osterholm-prepares-for-whatif?</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">PLoS One. 2020; 15(8): e0237410. Published online 2020 </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">Aug 20.</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> doi: 10.1371/journal.pone.0237410 PMCID: PMC7446902 PMID: </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">32817706</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Abstract </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The results demonstrate: (a) that the minimum CWD oral infectious dose is vastly lower than historical studies used to establish infection; (b) that a direct relationship exists between dose and incubation time to first prion replication detection in tonsils, irrespective of genotype; (c) that a difference was not discernible between brain vs. saliva source prions in ability to establish infection or in resultant disease course; and (d) that the CWD infection process appears to conform more to a threshold dose than an accumulative dose dynamic. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** now, let’s see what the authors said way back about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans” </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">From: TSS Subject: CWD aka MAD DEER/ELK TO HUMANS ??? </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Date: September 30, 2002 at 7:06 am PST </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">From: "Belay, Ermias" </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Sent: Monday, September 30, 2002 9:22 AM </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">404-639-3091</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">). </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Ermias Belay, M.D. Centers for Disease Control and Prevention </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">-----Original Message----- From: </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Sent: Sunday, September 29, 2002 10:15 AM To: </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">rr26k@nih.gov</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">; </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">rrace@niaid.nih.gov</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">; </span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;">ebb8@CDC.GOV</span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://pubmed.ncbi.nlm.nih.gov/11594928/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/11594928/</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">> However, to date, no CWD infections have been reported in people. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven. if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way; </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">sporadic = 54,983 hits </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">spontaneous = 325,650 hits </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></span><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"> </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">> However, to date, no CWD infections have been reported in people.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a></span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">From: Steve Dealler </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">To: BSE-L@ References: </span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Dear Terry,</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Steve Dealler </span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"><br style="outline: none !important;" /></span></div><div style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">=============== </span></div><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Table 9 presents the results of an analysis of these data.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;">snip...see full report ;</span></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p2" style="font-stretch: normal; line-height: normal; margin: 0px; min-height: 23.8px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s1" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important;"></span><br style="outline: none !important;" /></p><p class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984p1" style="font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><span class="yiv1585712922ydp9f180146yiv7216723629ydp8aa3ee25yiv7404456984s2" style="font-family: UICTFontTextStyleBody; font-size: 18.36px; outline: none !important; text-decoration-line: underline;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></span></p><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div dir="ltr" style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;">2004 video</span></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;"><br style="outline: none !important;" /></span></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;">Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD </span><br style="outline: none !important;" /></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;"><br style="outline: none !important;" /></span></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium; outline: none !important;">????: CBCnews</strong><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;"><br style="outline: none !important;" /></span></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;"><br style="outline: none !important;" /></span></span></div><div style="color: black; font-family: arial; font-size: 13.3333px; outline: none !important;"><span style="background-color: whitesmoke; outline: none !important;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: medium; font-weight: 700; outline: none !important;"><a href="https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html</a></span></span></div></div><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;">1997 nvCJD video</div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><a href="https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Successful transmission of the chronic wasting disease (CWD) agent to white-tailed deer by intravenous blood transfusion </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Najiba Mammadova a b, Eric Cassmann a b, Justin J. Greenlee a Show more Add to Mendeley Share Cite <a href="https://doi.org/10.1016/j.rvsc.2020.10.009" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1016/j.rvsc.2020.10.009</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Get rights and content Under a Creative Commons license open access </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Highlights </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">• The chronic wasting disease (CWD) agent efficiently transmits between white-tailed deer.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">• Blood from CWD infected deer contains infectious prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">• A single intravenous blood transfusion resulted in CWD transmission with an incubation of 25.6 months for the GG96 recipient.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">• The GS96 recipient had a longer incubation of 43.6 months.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSEs) that affects free-ranging and captive cervid species. The infectious agent of CWD may be transmitted from ingestion of prions shed in bodily fluids (e.g. feces, urine, saliva, placenta tissue) of infected animals, contaminated pastures, and/or decomposing carcasses from dead animals. Studies have also demonstrated prion infectivity in whole blood or blood fractions of CWD infected animals. To determine if CWD-infected blood contained sufficient levels of prion infectivity to cause disease, recipient deer were inoculated intravenously (IV) with blood derived from a CWD-infected white-tailed deer. We found that the CWD agent can be successfully transmitted to white-tailed deer by a single intravenous blood transfusion. The incubation period was associated with recipient prion protein genotype at codon 96 with the GG96 recipient incubating for 25.6 months and the GS96 recipient incubating for 43.6 months. This study complements and supports an earlier finding that CWD can be transmitted to deer by intravenous blood transfusion from white-tailed deer with CWD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">While a previous and larger study showed similar results, we determined that only 100 mL of CWD-infected blood (~2.5 times less than previously shown in (Mathiason et al., 2010)) contained sufficient levels of prion infectivity to cause disease. The identification of blood-borne transmission of the CWD agent is important in reinforcing the risk of exposure to CWD via blood as well as the possibility of hematogenous transmission of the CWD agent through insect vector. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.sciencedirect.com/science/article/pii/S003452882031047X/pdf" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.sciencedirect.com/science/article/pii/S003452882031047X/pdf</a><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">PLoS One. 2020; 15(8): e0237410. Published online 2020 Aug 20. doi: 10.1371/journal.pone.0237410 PMCID: PMC7446902 PMID: 32817706 </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">See PLoS One. 2021 June 10; 16(6): e0253356. Associated Data Data Availability Statement </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">Abstract </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">The minimum infectious dose required to induce CWD infection in cervids remains unknown, as does whether peripherally shed prions and/or multiple low dose exposures are important factors in CWD transmission. With the goal of better understand CWD infection in nature, we studied oral exposures of deer to very low doses of CWD prions and also examined whether the frequency of exposure or prion source may influence infection and pathogenesis. We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.</div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">snip...</div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">In conclusion, we have attempted to model and better understand CWD infection relative to natural exposure. The results demonstrate: </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">(a) that the minimum CWD oral infectious dose is vastly lower than historical studies used to establish infection; </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">(b) that a direct relationship exists between dose and incubation time to first prion replication detection in tonsils, irrespective of genotype; </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">(c) that a difference was not discernible between brain vs. saliva source prions in ability to establish infection or in resultant disease course; and </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;">(d) that the CWD infection process appears to conform more to a threshold dose than an accumulative dose dynamic. </div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><br style="outline: none !important;" /></div><div style="background-color: whitesmoke; color: black; font-family: arial; font-size: 16px; outline: none !important; text-align: justify;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/" rel="nofollow noopener noreferrer" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446902/</a></div></div></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;">GET THOSE DAMN DEER TESTED FOR CWD TSE PRION BEFORE TAKING THEM HOME AND FEEDING TO YOUR FAMILY AND FRIENDS !!!</div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="background-color: white; color: #1d2228; font-family: Helvetica, Arial, sans-serif; font-size: 18.4px; font-stretch: normal; line-height: normal; margin: 0px; outline: none !important;">Terry S. Singeltary Sr.</div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-79298926913178440332023-07-05T11:54:00.004-05:002023-07-05T11:54:39.781-05:00A novel subtype of sporadic Creutzfeldt–Jakob disease with PRNP codon 129MM genotype and PrP plaques<p><span style="background-color: white; font-family: arial; font-size: 16px;">Acta Neuropathol. 2023; 146(1): 121–143.</span></p><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Published online 2023 May 8. doi: 10.1007/s00401-023-02581-1</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">PMCID: PMC10166463</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">PMID: 37156880</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">A novel subtype of sporadic Creutzfeldt–Jakob disease with PRNP codon 129MM genotype and PrP plaques</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Rabeah Bayazid,1 Christina Orru’,6 Rabail Aslam,1 Yvonne Cohen,1 Amelia Silva-Rohwer,1,5 Seong-Ki Lee,2 Rossana Occhipinti,2 Qingzhong Kong,1,5 Shashirekha Shetty,1,5 Mark L. Cohen,1,5 Byron Caughey,6 Lawrence B. Schonberger,7 Brian S. Appleby,1,3,4,5 and Ignazio Calicorresponding author1,5</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The presence of amyloid kuru plaques is a pathological hallmark of sporadic Creutzfeldt–Jakob disease (sCJD) of the MV2K subtype. Recently, PrP plaques (p) have been described in the white matter of a small group of CJD (p-CJD) cases with the 129MM genotype and carrying resPrPD type 1 (T1). Despite the different histopathological phenotype, the gel mobility and molecular features of p-CJD resPrPD T1 mimic those of sCJDMM1, the most common human prion disease. Here, we describe the clinical features, histopathology, and molecular properties of two distinct PrP plaque phenotypes affecting the gray matter (pGM) or the white matter (pWM) of sCJD cases with the PrP 129MM genotype (sCJDMM). Prevalence of pGM- and pWM-CJD proved comparable and was estimated to be ~ 0.6% among sporadic prion diseases and ~ 1.1% among the sCJDMM group. Mean age at onset (61 and 68 years) and disease duration (~ 7 months) of pWM- and pGM-CJD did not differ significantly. PrP plaques were mostly confined to the cerebellar cortex in pGM-CJD, but were ubiquitous in pWM-CJD. Typing of resPrPD T1 showed an unglycosylated fragment of ~ 20 kDa (T120) in pGM-CJD and sCJDMM1 patients, while a doublet of ~ 21–20 kDa (T121−20) was a molecular signature of pWM-CJD in subcortical regions. In addition, conformational characteristics of pWM-CJD resPrPD T1 differed from those of pGM-CJD and sCJDMM1. Inoculation of pWM-CJD and sCJDMM1 brain extracts to transgenic mice expressing human PrP reproduced the histotype with PrP plaques only in mice challenged with pWM-CJD. Furthermore, T120 of pWM-CJD, but not T121, was propagated in mice. These data suggest that T121 and T120 of pWM-CJD, and T120 of sCJDMM1 are distinct prion strains. Further studies are required to shed light on the etiology of p-CJD cases, particularly those of T120 of the novel pGM-CJD subtype.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Here, we describe the detailed disease phenotype and distinctive molecular features of PrPD associated with the pWM-CJD group and novel pGM-CJD subtype. These findings are important as they point toward the distinction of two human prion diseases by divergent PrP plaque phenotypes, and highlight the importance of a careful histopathological examination, with special attention to the cerebellar cortex.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Beyond sporadic CJD, PrP plaques, including florid plaques and other less common types of plaques, have been described in iCJD, kuru, and variant CJD in humans, or in bovine spongiform encephalopathy (BSE) and chronic wasting disease (CWD) in animals [13, 16, 30, 43, 72]. These prion diseases are readily transmissible to certain transgenic mice and to humans [17, 19, 34, 69]. Furthermore, there are concerns about whether CWD prions can infect humans, as humans are exposed to CWD in several states [29]. Plaques are also observed in genetic prion diseases, such as Gerstmann-Sträussler-Scheinker syndrome (GSS) [18].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Discussion Prevalence of United States p-CJD We have characterized the clinico-histopathological and molecular features of 21 US p-CJD cases. Taking into account the 14 p-CJD individuals of the retrospective study, the prevalence of each p-CJD subtype is 1.13% among definite sCJDMM cases, or 0.59% among all sporadic prion diseases. The 0.59% prevalence is higher than the 0.17% of iCJD (P = 0.18), but lower than sporadic fatal insomnia (sFI) (1.35%, P = 0.059), sCJDVV1 (1.52%, P = 0.027), and variably protease-sensitive prionopathy (VPSPr) (1.78%, P = 0.0078) [23, 46, 54, 56]. The identification of 7 additional pWM-CJD cases suggests that this subtype is the most common of the two. Furthermore, the pWM-CJD subtype has been previously described by European and Japanese laboratories [4, 26, 33, 63], whereas a gap exists between our description of pGM-CJD and the lack of similar reports in the context of idiopathic CJD [35].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Our study indicates that pGM-CJD should be searched in cases of the -MM2 and -MM1-2 sCJD subtypes. On the contrary, 10 pWM-CJD cases (71%) belonged to the -MM1 subtype, and only two were diagnosed as -MM2 or -MM1-2 [8]. These prevalences are consistent with those of non-US pWM-CJD cases [4, 26, 33, 63].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Clinical and histopathological features of US- and non-US pWM-CJD patients Data from our case series suggests that there may be slight clinical differences between p-CJD and conventional sCJDMM cases. p-CJD cases may present more commonly with cognitive and psychiatric symptoms and less commonly with cerebellar symptoms compared to sCJDMM cases. Additionally, positive CSF 14–3-3 analyses were less frequent in p-CJD cases, and these cases may be less likely to have basal ganglia involvement on brain MRI. These slight differences fall within the expected clinical heterogeneity of CJD and did not appear to affect the clinical diagnoses of these subjects [1]. Interpretation of the clinical phenotype of p-CJD is limited by the amount and type of clinical information that is collected by the NPDPSC. Although clinical phenotypes can sometimes vary across different human prion strains, they are unlikely to be a reliable indicator of strain differences in isolation. Examples are the prominent neuropsychiatric symptoms observed in both vCJD and young onset sCJD and MRI findings suggestive of sCJD in a case of vCJD that was heterozygous at codon 129 [2, 47]. Clinical features have been shown to be heterogeneous in non-US pWM-CJD cases [63]. While age at onset of US and non-US (~ 65 years) pWM-CJD cases does not differ significantly, disease duration was 3-times longer in non-US cases (21 ± 12 months; P < 0.02) [4, 26, 33, 63]. The significantly different disease duration is probably due to more extensive medical care in Asia.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The US pWM-CJD histotype mimicked that of non-US pWM-CJD cases [4, 26, 33, 63]. We and others have recently shown that CAA is a major feature of Aβ pathology in patients with iCJD, but not of age-matched sCJD cases [10, 28, 32, 59]. Although the US p-CJD cases were significantly older than US sCJDMM cases [10], CAA prevalence did not differ between the two diseases. These results point to an age-dependent Aβ pathology in US p-CJD.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">US pGM-CJD, sporadic CJD and iCJD cases with PrP plaque pathology: a review of the literature PrP plaques populated the cerebellar cortex in pGM-CJD with the exception of one case in which rare PrP plaques affected the occipital cortex. The spread of PrP plaques to the occipital lobe does not seem to be the result of a protracted disease duration since death occurred two months after clinical presentation. Two pGM-CJD cases showed target-like PrP formations. Whether these “loose plaques” contains PrP fibrils remains to be determined [67]. Also, the presence of only rare diffuse Aβ plaques in these patients is against the hypothesis that target-like PrP is the result of an enhancement of PrP around or within Aβ plaques [10].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">We have searched in the literature for the presence of PrP plaques in the gray matter of patients with sporadic prion disease linked to codon 129MM genotype; three cases were found. In the first report, a 54-year-old neurosurgeon with an 18-month disease course and sleep disturbances harbored PrP plaques in the cerebral cortex. Inoculation of brain homogenates from this patient to chimpanzee and squirrel monkeys lead to prion disease. However, the human histotype was not fully reproduced by these primates as kuru-type plaques were not detected in the brain of the affected animals [64]. In another study, a 75-year-old woman with an 11-month clinical course, underwent neurosurgery without dura mater about 14 years before the onset of clinical symptoms. At autopsy, “congophilic amyloid plaques” were noted in the cerebellar cortex [31]. Although both patients were originally diagnosed as being sporadic, a subsequent study suggested these two cases had an iatrogenic prion disease. This conclusion, which stems from a known iatrogenic risk factor in one of the cases (neurosurgery), was supported by an in vivo study [35]. The third case is that of a 40-year-old woman with no known history of iatrogenic exposure who was alive at the time of the brain biopsy, which occurred ~ 2.5 years after the appearance of clinical disease. This patient presented with dementia, showed a “conspicuous” number of PrP plaques in the occipital cortex, and was 129MM [45]. To our knowledge, these cases were free of florid plaques.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In our pGM-CJD case 5, clusters of florid plaques were noted in the cerebellum. Our pGM-CJD case 5, is a 57-year-old male with sensory symptoms (numbness, tingling, and pain in the fingers) at presentation. Case 5 did not have a history of venison consumption, blood transfusion, travel to any BSE-exposed countries, or known surgical history. It should be emphasized that disease onset with sensory symptoms can be a clinical presentation observed in vCJD; however, case 5’s brain MRI demonstrated restricted diffusion abnormalities in the cortex and caudate that is typical for sCJD. Additionally, the illness duration of 2 months is shorter than typically reported in vCJD. The resPrPD glycotype of case 5 resembles that of sCJD.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The presence of florid plaques has been described in a 70-year-old Slovenian female who presented with psychiatric symptoms at the age of ~ 68 years, and had traveled to the UK at a time of BSE pandemic. The patient was of the 129MV genotype and harbored resPrPD T2. Despite the presence of florid plaques and several clinical features overlapping with vCJD, the authors concluded that the patient’s atypical phenotype was likely due to the known heterogeneity of the -MV2 subtype [6, 49, 57]. The resPrPD glycotype of this patient mimicked that of sCJD, characterized by the predominance of the monoglycosylated resPrPD isoform. By contrast, the over-representation of the diglycosylated resPrPD isoform is a feature of vCJD, and is independent of the codon 129 genotype [47, 71]. Florid plaques have been reported in patients with iCJD linked to the 129MM genotype (iCJDMM) [13, 40, 42, 66]. We have described a US growth hormone iCJDMM (GH-iCJDMM) case with a complex PrP plaque pathology. In addition, this patient showed laminar spongiform degeneration, PrP immunostaining with diffuse, plaque-like and perineuronal patterns, and pericellular PrP [13]. Thus, the presence of PrP plaques is the only common histopathological feature of the US GH-iCJDMM and pGM-CJD. Moreover, PrP plaque pathology of the US GH-iCJDMM case was significantly more severe.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Finally, conflicted results are shown in the literature regarding the gel mobility of the unglycosylated resPrPD of iCJDMM cases with PrP plaques. In one case report, resPrPD of DM-iCJD migrated about 1 kDa more than the ~ 21 kDa of sCJDMM1 [42]. Similar results were observed in one US GH-iCJD [10, 13], two atypical iCJD [35], Japanese DM-iCJD [34], and one UK GH-iCJD case[60]. In two other studies, resPrPD of iCJD and sCJDMM1 showed similar gel mobility [20, 66]. Notably, 10 of 11 French iCJDMM cases harbored “Type 1” and only one case “type i” (or type “intermediate”, corresponding to a resPrPD fragment of ~ 20 kDa) [20]. Since the buffer pH and the use of other stringent experimental conditions are important tools in assessing the gel mobility of resPrPD, unified experimental conditions should be used to characterize the molecular features of atypical cases that are suspected of an iatrogenic etiology (Fig. S10).</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Molecular features of T1 and T2 of US p-CJD The electrophoretic profile of resPrPD T1 and its anatomical distribution are major differences of p-CJD subtypes. Although T121−20 was noted in ~ 55% of the cases, its prevalence is likely to be higher if a more extensive sampling of subcortical brain regions is carried out. T121−20 was occasionally detected in the cerebral cortex, but never found in the cerebellum. These data suggest a tropism of PrPD for different neuronal cell types, and high accessibility of T121 to subcortical regions, where PrPC is likely to be converted to PrPD T121 at a higher rate than T120. This hypothesis is supported by the fact that our buffer pH (8.0) favors the formation of T120 [8, 9, 50]. Furthermore, it seems unlikely that T2 affects T1 distribution in subcortical regions, as the amount of T2 distribution in the two p-CJD subtypes was virtually identical. The fact that T120 of pGM-CJD and sCJDMM1 share similar GdnHCl1/2 values based on the CSSA, does not necessarily indicate that T120 in these two diseases belong to the same prion strain. This can be demonstrated by the similar GdnHCl1/2 indexes of T120 and T2 associated with sCJDVV1 and -VV2, respectively [14]. In previous studies, RT-QuIC assays have revealed some significant differences in the means of kinetic values obtained from certain sCJD subtypes [21]. In this study, we saw modest differences in the overall mean times to threshold between pWM-CJD T1 and pGM-CJD T2 brain specimens, but the relevance of such differences is unclear because they could be explained by relative seed concentration and distinct seed structures. A second major molecular difference, is the higher proportion of T2 and sCJDMM2-like histopathological features in pGM-CJD, which exceeded that of pWM-CJD by ~ 3- and twofold, respectively. Despite the significantly higher proportion of T2 in pGM-CJD, T120 is better represented than T2 overall, and the bulk of PrP plaque pathology is in the cerebellum, which harbors only T120 by western blot in most cases. Case 7, with an overall T2 representation of only 3%, deserves a separate analysis. Unlike the other pGM-CJD cases, rare PrP plaques populated only the granular layer. This may indicate that the presence of T2 is required for a more severe and/or widespread distribution of PrP plaques, and that T2 aggregates are preferentially sequestered in the amyloid core [68]. Furthermore, cerebellar T1 aggregates may be less compact and readily disaggregate following proteolytic digestion and standard denaturation procedures [37].</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Do T121, T120 and T2 of pWM-CJD have strains properties? Transmission studies are a gold standard to gain insights on disease mechanism of neurodegeneration. From our bioassay we can reach three conclusions. First, only pWM-CJD T120 propagates in TgHuPrPGly+/+, generating amyloid plaques. Although we did not perform a second passage, T120 of pWM-CJD and sCJDMM1 likely represent different prion strains. The lack of T2 in these mice indicates that T120 is a faster replicating prion strain [8, 52] (Table (Table1).1). Second, T121 could be a different human strain, as it did not propagate in the host. One possible, but perhaps unlikely, explanation is that T121 in the cerebral cortex (where the brain homogenates was injected), may be unable to convert PrPC into PrPD; or that conversion by T121 is inefficient and occurs at a rate that is several orders of magnitudes lower than that of T120. Recently, we have shown that T121 associated with sCJDVV1 faithfully propagates in the TgHuPrP-129VV mouse brain and that inoculation of T121−20 to the same mouse line generates T121 [11]. However, attempts to transmit T121 to TgHuPrP-129MM mice failed on first and second passages. Altogether, the present data and those of Cali et al. [11] suggest that T121 of pWM-CJD and sCJDVV1 have distinct molecular features, as pWM-CJD T121 does not replicate in TgHuPrP-129MM mice, whereas sCJDVV1 T121 faithfully propagates to TgHuPrP mice with the same genotype (129VV). It would be important to assess whether T121 pWM-CJD faithfully transmits disease to TgHuPrP-129VV mice. Lastly, pWM-CJD injected into TgHuPrPGly+/– mice generated T2 and PrP plaques. These data suggest that the lack of glycosylated PrP isoforms, even in one allele, is sufficient to favor T2 propagation, and therefore overturn strain selection by the host. The incomplete glycosylation may also explain the more complex PrP plaque pathology. Although the formation of PrP plaques in TgHuPrPGly+/– mice may be the result of the partial glycosylation, the presence of immature plaques in the cerebellar white matter argues for a role of T2 (present in the inoculum) in PrP plaque formation. A minority of TgHuPrPGly+/– mice were accessible to T120 of sCJDMM1 but the incubation time was longer than in TgHuPrPGly+/+ mice. Overall, our data suggest that glycans play a protective role in these mice [65, 73]. Although the partial absence of glycans accelerates T2 replication at the expenses of T120, it should be emphasized that T1 in sCJDMM patients is significantly better represented than T2.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Conclusions</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">We have characterized the clinical, histopathological, and molecular properties of two human prion diseases with distinct PrP plaque pathology and divergent PrPD molecular features. While pWM-CJD cases are readily identifiable due to the presence of PrP plaques on a white background, pGM-CJD cases may be more difficult to detect. If pGM-CJD is a sporadic prion disease, this phenotype should be identified by other prion Surveillance Centers. Nevertheless, the lack of major reports on pGM-CJD by other countries is puzzling as both plaque histotypes have similar prevalences in the United States. If these cases are the result of acquired prion disease, their route of transmission is not apparent and not due to any recognized or currently hypothesized acquired prion disease/risk factors. While one major goal of this study is to contribute to the identification of atypical or novel histotypes (or novel prion diseases), it is important to identify new markers of iatrogenic disease. Meanwhile, it seems appropriate to classify pGM-CJD and pWM-CJD as sporadic prion diseases. Additionally, in vitro and in vivo experiments are needed to further dissect the molecular features of p-CJD PrPD with the aim of gaining insights on the mechanisms governing these disorders.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10166463/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10166463/</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">1999</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">''I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age.''</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">US scientists develop a possible test for BSE</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">BMJ 1999; 319 doi: <a href="https://doi.org/10.1136/bmj.319.7220.1312b" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1136/bmj.319.7220.1312b</a> (Published 13 November 1999)</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Cite this as: BMJ 1999;319:1312</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Rapid Response:</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Re: vCJD in the USA * BSE in U.S.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">15 November 1999</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S Singeltary</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">NA</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">medically retired</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease. Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Sporadic, simply means, they do not know.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Since 1990 the U.S. has raised 1,250,880,700 cattle;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endoscopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">P.O. Box </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Bacliff, Texas 77518 USA</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">flounder@wt.net</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Competing interests: No competing interests</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">February 14, 2001</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary, Sr</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Author Affiliations</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://jamanetwork.com/journals/jama/article-abstract/1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jama/article-abstract/1031186</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary, retired (medically), CJD WATCH</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Submitted March 26, 2003</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">August 10, 2009</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Greetings,</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">please see history, and the ever evolving TSE science to date ;</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Saturday, June 13, 2009</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Singeltary 2000</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7226.8/b (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">02 January 2000 Terry S Singeltary retired</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rapid Response: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Something else I find odd, page 16;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A few more factors to consider, page 15;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To be continued...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Terry S. Singeltary Sr. Bacliff, Texas USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Competing interests: No competing interests</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #338fe9; outline: none !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tracking spongiform encephalopathies in North America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xavier Bosch</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Available online 29 July 2003. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Volume 3, Issue 8, August 2003, Page 463 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Volume 3, Number 8 01 August 2003</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Newsdesk</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tracking spongiform encephalopathies in North America</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Xavier Bosch</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Singeltary 2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">by Philip Yam </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Revisiting Sporadic CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow.org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">223</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on www.vegsource.com and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">224 CHAPTER 14</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laying Odds 225</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">226 CHAPTER 14</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A Case for Undercounting</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Laying Odds 227</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a onein-a-million lottery, it’s more like one-in-2.5-million for AfricanAmericans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SNIP...SEE FULL TEXT;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div></div></div></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;">Singeltary Submission SEAC 2007</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Alzheimer's disease, iatrogenic transmission, what if?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full</a> </div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">*** Singeltary comment PLoS *** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Posted by flounder on 05 Nov 2014 at 21:27 GMT </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.plosone.org/annotation/listThread.action?root=82860" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.plosone.org/annotation/listThread.action?root=82860</a></div></div></div></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">MONDAY, APRIL 24, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/2023-cdc-reports-cjd-tse-prion-5-cases.html</a> </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prpsc.proboards.com/thread/114/2023-cdc-cjd-prion-cases" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/114/2023-cdc-cjd-prion-cases</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">WEDNESDAY, FEBRUARY 8, 2023</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">WEDNESDAY, JANUARY 25, 2023</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">FRIDAY, DECEMBER 02, 2022 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Wednesday, May 24, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">SATURDAY, MAY 20, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed</a></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Monday, May 22, 2023 </div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">BSE TSE Prion MAD COW TESTING IN THE USA COMPARED TO OTHER COUNTRIES?</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><a href="https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://specifiedriskmaterial.blogspot.com/2023/05/bse-tse-prion-mad-cow-testing-in-usa.html</a><br style="outline: none !important;" /></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Tuesday, May 30, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">World Organisation for Animal Health 90th General Session of the World Assembly of Delegates BSE TSE Prion 2023<br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/05/world-organisation-for-animal-health.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://woahoie.blogspot.com/2023/05/world-organisation-for-animal-health.html</a></div></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Tue, 9 Jan 2001 16:49:00 -0800</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Terry S. Singeltary Sr."</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: BSE-L@uni-karlsruhe.de </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr.</div><div style="background-color: white; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-38091566652935532962023-04-24T15:17:00.001-05:002023-04-24T16:59:36.203-05:002023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older<div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older</span><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The risk of CJD increases with age; the 2016–2020 average annual rate in the United States was about 5 cases per million in persons 55 years of age or older.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.cdc.gov/prions/cjd/occurrence-transmission.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.cdc.gov/prions/cjd/occurrence-transmission.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 1999</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">US scientists develop a possible test for BSE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Cite this as: BMJ 1999;319:1312</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">15 November 1999</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Terry S Singeltary</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">medically retired</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rapid Response:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Re: vCJD in the USA * BSE in U.S.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Since 1990 the U.S. has raised 1,250,880,700 cattle;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Terry S. Singeltary Sr.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Bacliff, Texas 77518 USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">flounder@wt.net</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Competing interests: No competing interests </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2000</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7226.8/b (Published 01 January 2000) Cite this as: BMJ 2000;320:8</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">02 January 2000 Terry S Singeltary retired</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rapid Response: </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Something else I find odd, page 16;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A few more factors to consider, page 15;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To be continued...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Terry S. Singeltary Sr. Bacliff, Texas USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Competing interests: No competing interests</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #338fe9; outline: currentcolor;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: currentcolor;">Singeltary 2001</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">To the Editor: </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.. </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Terry S. Singeltary, Sr Bacliff, Tex </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. </div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><a href="http://jama.jamanetwork.com/article.aspx?articleid=1031186" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://jama.jamanetwork.com/article.aspx?articleid=1031186</a> </div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2003</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Tracking spongiform encephalopathies in North America</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Xavier Bosch</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Available online 29 July 2003. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Volume 3, Issue 8, August 2003, Page 463 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Volume 3, Number 8 01 August 2003</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Newsdesk</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Tracking spongiform encephalopathies in North America</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Xavier Bosch</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD) the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunters two of whom were friends who died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div style="outline: currentcolor;"><a href="http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2003</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">January 28, 2003; 60 (2) VIEWS & REVIEWS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Terry S. Singeltary, retired (medically) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Published March 26, 2003</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">26 March 2003</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Terry S. Singeltary, retired (medically) CJD WATCH</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states</a></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2007</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">by Philip Yam </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Revisiting Sporadic CJD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">It’s not hard to get Terry Singeltary going. “I have my conspiracy theories,” admitted the 49-year-old Texan.1 Singeltary is probably the nation’s most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union’s Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow.org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">223</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people’s health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary has similar inclinations, but unlike these men, he doesn’t have the professional credentials behind him. He is an 11th-grade dropout, a machinist who retired because of a neck injury sustained at work. But you might not know that from the vast stores of information in his mind and on his hard drive. Over the years, he has provided unacknowledged help to reporters around the globe, passing on files to such big-time players as The New York Times, Newsweek, and USA Today. His networking with journalists, activists, and concerned citizens has helped medical authorities make contact with suspected CJD victims. He has kept scientists informed with his almost daily posting of news items and research abstracts on electronic newsgroups, including the bulletin board on www.vegsource.com and the BSE-listserv run out of the University of Karlsruhe, Germany. His combative, blunt, opinionated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others—especially when he repeats his conviction that “the government has lied to us, the feed industry has lied to us—all over a buck.” As evidence, Singeltary cites the USDA’s testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 million cattle, because the incidence of BSE may be as low as one in a million, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary got into the field of transmissible spongiform encephalopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version—the Heidenhain variant—that first causes the patient to go blind and then to deteriorate rapidly. She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: “It’s something you never forget.” Her uncontrollable muscle twitching became so bad “that it took three of us to hold her one time,” Singeltary recalled. “She did everything but levitate in bed and spin her head.” Doctors originally diagnosed Alzheimer’s disease, but a postmortem neuropathological exam demanded by Singeltary revealed the true nature of her death.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">224 CHAPTER 14</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Classifying a disease as “sporadic” is another way for doctors to say they don’t know the cause. Normal prion proteins just turn rogue in the brain for no apparent reason. The term “sporadic” is often particularly hard for the victims’ families to accept, especially when the patient was previously in robust health. Maybe it was something in the water, they wonder, or in the air, or something they ate—the same questions CJD researchers tried to answer decades ago. The names “sporadic CJD” and “variant CJD” also confuse the public and raise suspicions that U.S. authorities are hiding something when they say there have been no native variant CJD cases in the country.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary suspected an environmental cause in his mother’s demise—a feeling reinforced a year later when a neighbor died of sporadic CJD. For years, the neighbor had been taking nutritional supplements that contained cow brain extracts. Researchers from the National Institutes of Health collected samples of the supplement, Singeltary recounted, and inoculated suspensions into mice. The mice remained healthy—which only means that those supplement samples tested were prion-free.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Scientists have made several attempts during the past few decades to find a connection between sporadic CJD and the environment. Often, these studies take the form of asking family members about CJD victims—their diet, occupation, medical history, hobbies, pets, and so forth—and comparing them with non-CJD subjects. Such case-control CJD studies have produced some intriguing—and sometimes contradictory—results. In 1985, Carleton Gajdusek and his NIH colleagues reported a correlation between CJD and eating a lot of roast pork, ham, hot dogs, and lamb, as well as rare meats and raw oysters.2 Yet they also recognized that the findings were preliminary and that more studies were needed.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Following up, Robert Will of the U.K. National CJD Surveillance Unit and others pooled this data with those from two other case-control studies on CJD (one from Japan and one from the U.K.). In particular, they figured the so-called odds ratio—calculated by dividing the frequency of a possible factor in the patient group by the frequency of the factor in the control group. An odds ratio greater than 1 means that the factor may be significant. In their study, Will and his collaborators found an increase of CJD in people who have worked as health professionals (odds ratio of 1.5) and people who have had contact with cows</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Laying Odds 225</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1.7) and sheep (1.6). Unfortunately, those connections were not statistically significant: The numbers of pooled patients (117) and control subjects (333) were so small that the researchers felt the odds ratios needed to reach 2.5 to 8 (depending on the assumptions) before they could be deemed statistically significant. The only statistically significant correlations they found were between CJD and a family history of either CJD (19.1) or other psychotic disease (9.9), although the latter might simply be correlated because psychotic disease may be an early symptom of undiagnosed CJD.3 In contrast with earlier findings, the team concluded that there was no association between sporadic CJD and the consumption of organ meats, including brains (0.6).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Although these case-control studies shed a certain amount of light on potential risk factors for CJD, it’s impossible to draw firm conclusions. Obtaining data that produces statistically meaningful results can be difficult because of the rarity of CJD and hence the shortage of subjects. Human memory is quite fragile, too, so patients’ families may not accurately recall the lifestyle and dietary habits of their loved ones over the course of a decade or more. Consequently, researchers must cope with data that probably contain significant biases. In a review paper on CJD, Joe Gibbs of the NIH and Richard T. Johnson of Johns Hopkins University concluded that “the absence of geographic differences in incidence is more convincing evidence against major dietary factors, since large populations eschew pork and some consume no meat or meat products.” A CJD study of lifelong vegetarians, they proposed, could produce some interesting data.4</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The inconclusive results of case-control studies do not completely rule out the environment as a possible cause of CJD. “Dr. Prusiner’s theory does fit much of the data of spontaneous generation of [malformed] PrP somewhere in the brain,” Will remarked—that is, the idea that sporadic CJD just happens by itself falls within the realm of the prion theory. Still, “it’s very odd, if you look at all the forms of human prion diseases there are, all of them are transmissible in the laboratory and could be due to some sort of infectious agent.”5 One of the great difficulties, he explained, is that “given that this is a disease of an extraordinarily long incubation period, are we really confident that we can exclude childhood exposure that is transmitted from person to person, as people move around? It’s difficult to be sure about that.” There might a “carrier state” that leaves people healthy yet still able to</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">226 CHAPTER 14</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">infect others. If so, “you would never be able to identify what’s causing the spread of the disease,” concluded Will, who hasn’t stopped looking for a possible environmental link. He has some preliminary data based on studies that trace CJD victims’ lives well before the time symptoms began—up to 70 years; they suggest some degree of geographic clustering, but no obvious candidates for a source of infection.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A Case for Undercounting</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The difficulty in establishing causal links in sporadic prion diseases—if there are any in the first place—underlines the importance of thorough surveillance. The U.K. has an active program, and when a victim of CJD is reported, one of Robert Will’s colleagues visits and questions the victim’s family. “No one has looked for CJD systematically in the U.S.,” the NIH’s Paul Brown noted. “Ever.”6 The U.S., through the Centers for Disease Control and Prevention, has generally maintained a more passive system, collecting information from death certificates from the National Center for Health Statistics. Because CJD is invariably fatal, mortality data is considered to be an effective means of tabulating cases. The CDC assessed the accuracy of such data by comparing the numbers with figures garnered through an active search in 1996: Teams covering five regions of the U.S. contacted the specialists involved and reviewed medical records for CJD cases between 1991 and 1995. Comparing the actively garnered data with the death certificate information showed that “we miss about 14 percent,” said CDC epidemiologist Lawrence Schonberger. “That’s improving. Doctors are becoming more knowledgeable,” thanks to increased scientific and media attention given to prion diseases.7</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The active surveillance study of 1996, however, only looked at cases in which physicians attributed the deaths to CJD. Misdiagnosed patients or patients who never saw a neurologist were not tabulated— thus CJD may be grossly underreported. Many neurological ailments share symptoms, especially early on. According to various studies, autopsies have found that CJD is misdiagnosed as other ills, such as dementia or Alzheimer’s disease, 5 to 13 percent of the time. The CDC finds that around 50,000Americans die from Alzheimer’s each year</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Laying Odds 227</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(about 4 million have the disease, according to the Alzheimer’s Association). Therefore, one could argue that thousands of CJD cases are being missed. (On the flip side, CJD could be mistakenly diagnosed as Alzheimer’s disease or dementia, but the number of CJD patients is so small that they wouldn’t dramatically skew the statistics for other neurological ills.)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In part to address the issue of misdiagnosis, CJD families have asked the CDC to place the disease on the national list of officially notifiable illnesses, which tends to include more contagious conditions such as AIDS, tuberculosis, hepatitis, and viral forms of encephalitis. Currently, only some states impose this requirement. CDC officials have discounted the utility of such an approach, arguing that it would duplicate the mortality data, which is more accurate than early diagnoses of CJD, anyway. Moreover, mandatory reporting of CJD cases does not necessarily guarantee the end to missed cases.8</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">One clue suggests that the passive system is undercounting CJD in the U.S.: racial difference. The number of black CJD victims is about 38 percent that of white victims. Rather than sporadic CJD being a onein-a-million lottery, it’s more like one-in-2.5-million for AfricanAmericans. Access to medical care might be one reason. Schonberger recounted that the CDC had asked other countries with substantial black populations to submit CJD figures for comparison but found that the surveillance in those countries was inadequate. “We haven’t been able to find any comparable literature on this issue, so it’s still up in the air,” Schonberger said. On the other hand, Alzheimer’s disease is more common among black people than whites, with an estimated higher prevalence ranging from 14 percent to almost 100 percent, according to a February 2002 report by the Alzheimer’s Association. Are some black CJD cases being misdiagnosed as Alzheimer’s?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Answering critics like Terry Singeltary, who feels that the U.S. undercounts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population. As Schonberger pointed out, no doctor would misdiagnose a 30-year-old CJD patient as having Alzheimer’s. The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SNIP...SEE FULL TEXT;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''The average age of the first 100 variant CJD victims was 29; should the epidemiology of vCJD change—if older people start coming down with it—then there would be problems. “The adequacy of our overall CJD surveillance would be greatly reduced should the proportion of older individuals affected by variant CJD substantially increase,” Schonberger explained.9''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://ndl.ethernet.edu.et/bitstream/123456789/38386/1/516.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">THE PATHOLOGICAL PROTEIN by Philip Yam</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.amazon.com/s?k=THE+PATHOLOGICAL+PROTEIN&i=stripbooks&crid=LA5IKGMF6PI9&sprefix=the+pathological+protein%2Cstripbooks%2C96&ref=nb_sb_noss_1" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.amazon.com/s?k=THE+PATHOLOGICAL+PROTEIN&i=stripbooks&crid=LA5IKGMF6PI9&sprefix=the+pathological+protein%2Cstripbooks%2C96&ref=nb_sb_noss_1</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary Submission SEAC 2007</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This was 22 years to the day Mom died from the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD, when i made this submission to SEAC and this was their reply to my questions of concern about cjd in the USA, my how things have changed...terry</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20091010132933/http://www.seac.gov.uk/minutes/99.pdf</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2009</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009<br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">August 10, 2009</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Greetings,</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">please see history, and the ever evolving TSE science to date ;</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Saturday, June 13, 2009</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 16px; outline: currentcolor;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a></div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2010</div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Human Prion Diseases in the United States</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Robert C. Holman ,Ermias D. Belay,Krista Y. Christensen,Ryan A. Maddox,Arialdi M. Minino,Arianne M. Folkema,Dana L. Haberling,Teresa A. Hammett,Kenneth D. Kochanek,James J. Sejvar,Lawrence B. Schonberger</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Published: January 1, 2010</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">https://doi.org/10.1371/journal.pone.0008521</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">re-Human Prion Diseases in the United States</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted by flounder on 01 Jan 2010 at 18:11 GMT</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I kindly disagree with your synopsis for the following reasons ;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Singeltary 2014</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Alzheimer's disease, iatrogenic transmission, what if?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://journals.plos.org/plosone/article/comment?id=info:doi/10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://journals.plos.org/plosone/article/comments?id=10.1371/journal.pone.0111492</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://journals.plos.org/plosone/article/comment?id=10.1371/annotation/933cc83a-a384-45c3-b3b2-336882c30f9d</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.frontiersin.org/articles/10.3389/fnagi.2016.00005/full</a> </div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">*** Singeltary comment PLoS *** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted by flounder on 05 Nov 2014 at 21:27 GMT </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.plosone.org/annotation/listThread.action?root=82860" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.plosone.org/annotation/listThread.action?root=82860</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">IN CONFIDENCE</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">5 NOVEMBER 1992</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">There are also results to be made available shortly </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">(1) concerning a farmer with CJD who had BSE animals, </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">(2) on the possible transmissibility of Alzheimer’s and </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf" rel="nofollow" style="color: blue; outline: currentcolor;" target="_blank">https://web.archive.org/web/20170126060344/http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow" style="color: blue; outline: currentcolor;" target="_blank">https://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf" rel="nofollow" style="color: blue; outline: currentcolor;" target="_blank">https://web.archive.org/web/20040315075058/www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Singeltary 2015</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow" style="color: blue; outline: currentcolor;" target="_blank">https://www.nature.com/articles/nature15369</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Singeltary Comment at very bottom of this Nature publishing;</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">where have we all heard this before? it's been well documented via the BSE Inquiry. have they not learned a lesson from the last time?</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">we have seen this time and time again in England (and other Country's) with the BSE mad cow TSE Prion debacle.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">That 'anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients ? </div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">who got pooled extracts injected from thousands of cadavers ? were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find 'alarming' is pathetic.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Sounds like the journalists had it right in the first place: 'Alzheimer's may be a transmissible infection' in The Independent to 'You can catch Alzheimer's' in The Daily Mirror or 'Alzheimer's bombshell' in The Daily Express</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">if not for the journalist, the layperson would not know about these important findings.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer's, the price of poker goes up drastically.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">so, who makes that final decision, and how many more decades do we have to wait?</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">in my opinion, it's one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it's bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer's of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. There are also results to be made available shortly (1) concerning a farmer with CJD who had BSE animals, (2) on the possible transmissibility of Alzheimer's and (3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;">Singeltary Comment at very bottom of this Nature publishing;</div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://www.nature.com/articles/nature15369#article-comments" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/nature15369#article-comments</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><a href="https://www.nature.com/articles/nature15369" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/nature15369</a><br style="outline: currentcolor;" /></div><div style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; font-size: 13.3333px; outline: currentcolor;"><div style="outline: currentcolor;">Saturday, March 18, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Autoclave treatment fails to completely inactivate DLB alpha-synuclein seeding activity </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://alpha-synuclein.blogspot.com/2023/03/autoclave-treatment-fails-to-completely.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Prusiner et al, then and now!</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.nejm.org/doi/full/10.1056/NEJM200105173442006" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nejm.org/doi/full/10.1056/NEJM200105173442006</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.pnas.org/doi/10.1073/pnas.2220984120" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.pnas.org/doi/10.1073/pnas.2220984120</a></div></div></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;">MONDAY, APRIL 24, 2023 </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;">Prion Disease on the Rise in the U.S., Now the question is, why?<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;">''5 cases per million in persons 55 years of age or older.''<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/prion-disease-on-rise-in-us-now.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/prion-disease-on-rise-in-us-now.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined¹ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Updated quarterly. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Last updated on: January 11th, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 1999 & earlier 383 232 202 27 3 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2000 145 102 90 12 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2001 209 118 110 8 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2002 241 144 124 18 2 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2003 259 160 137 21 2 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2004 315 180 163 16 0 1³ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2005 330 179 157 21 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2006 365 179 159 17 1 2⁴ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2007 374 210 191 19 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2008 384 221 205 16 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2009 397 231 210 20 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2010 402 246 218 28 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2011 392 238 214 24 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2012 413 244 221 23 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2013 416 258 223 34 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2014 355 208 185 21 1 1⁵ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2015 401 263 243 20 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2016 395 277 248 29 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2017 375 266 247 19 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2018 308 221 202 18 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2019 433 280 259 21 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2020 366 252 227 24 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2021 343 248 223 22 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2022 307 199 165 13 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> TOTAL 83086 51567 46238 4919 14 4 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Year CSF Only and RT-QuIC Positive10 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2015 241 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2016 360 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2017 406 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2018 431 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2019 538 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2020 494 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2021 516 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2022 492 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TOTAL 3478 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Listed based on the year of death or, if not available, on the year of referral; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2Cases with suspected prion disease for which brain tissue was submitted; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3Disease acquired in the United Kingdom; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">4Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">5Disease possibly acquired in a Middle Eastern or Eastern European country; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">6Includes 25 cases in which the diagnosis is pending (1 from 2020, 2 from 2021 and 21 from 2022), and 20 inconclusive cases; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">7Includes 24 (3 from 2021 and 21 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">8The sporadic cases include 4504 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">9Total does not include 301 Familial cases diagnosed by blood test only. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">10Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">For a downloadable PDF version of our quarterly table, please click the link below: </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> NPDPSC Table of Cases Examined </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> © 2023 Case Western Reserve University 10900 Euclid Ave. Cleveland, Ohio 44106 216.368.2000 Legal Notice | Privacy Policy PATHOLOGY Campus Location: Wolstein Research Building 5129 2103 Cornell Road Cleveland, OH 44106 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Mailing Address: 10900 Euclid Ave. Cleveland, OH 44106-7288 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Phone: 216.368.3611 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Email: pathology@case.edu </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Tables of Cases Examined | Pathology | School of Medicine | Case Western Reserve University case.edu </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> National Prion Disease Pathology Surveillance Center Cases Examined1 (September 20, 2022) Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 1999 & earlier 383 232 202 27 3 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2000 145 102 90 12 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2001 209 118 110 8 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2002 241 144 124 18 2 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2003 259 160 137 21 2 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2004 315 180 163 16 0 13 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2005 328 179 157 21 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2006 365 179 159 17 1 24 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2007 374 210 191 19 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2008 384 221 205 16 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2009 397 231 210 20 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2010 401 246 218 28 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2011 392 238 214 24 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2012 413 244 221 23 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2013 416 258 223 34 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2014 355 208 185 21 1 15 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2015 401 263 243 20 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2016 395 277 248 29 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2017 375 266 247 19 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2018 308 221 202 18 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2019 434 281 259 22 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2020 365 252 227 24 1 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2021 343 248 223 22 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2022 213 124 98 9 0 0 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> TOTAL 82116 50827 45568 4889 14 4 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Year CSF Only & RT-QuIC Positive10 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2015 140 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2016 183 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2017 227 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2018 266 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2019 311 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2020 310 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2021 341 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2022 262 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> TOTAL 2040 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 1 Listed based on the year of death or, if not available, on year of referral; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 3 Disease acquired in the United Kingdom; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 5 Disease possibly acquired in a Middle Eastern or Eastern European country; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 6 Includes 28 cases in which the diagnosis is pending (1 from 2020, 3 from 2021 and 24 from 2022), and 20 inconclusive cases; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 7 Includes 20 (3 from 2021 and 17 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 8 The sporadic cases include 4437 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> 9 Total does not include 300 Familial cases diagnosed by blood only. 10 Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;">WEDNESDAY, JANUARY 25, 2023 </div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html</a> </div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://vpspr.blogspot.com/</a></div></div><div dir="ltr" style="outline: currentcolor;"><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">FRIDAY, APRIL 07, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan <***</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><div style="outline: currentcolor;">Tuesday APRIL 05, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SUNDAY, MAY 08, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, MAY 24, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, MAY 10, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> FRIDAY, DECEMBER 02, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">SUNDAY, DECEMBER 11, 2022 </div><div dir="ltr" style="outline: currentcolor;"><br /></div><div dir="ltr" style="outline: currentcolor;">SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://seac992007.blogspot.com/2022/12/seac-spongiform-encephalopathy-advisory.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://seac992007.blogspot.com/2022/12/seac-spongiform-encephalopathy-advisory.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> Friday, DECEMBER 24, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html</a> </div></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">USA CJD TSE Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined May 1, 2018</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SATURDAY, JULY 22, 2017</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Why the U.S. Needs to Continue Prion Disease Surveillance, instead of reducing funding to zero</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;">National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr<br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">USA CJD TSE Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined May 1, 2018</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SATURDAY, JULY 22, 2017</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Why the U.S. Needs to Continue Prion Disease Surveillance, instead of reducing funding to zero</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a 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dir="ltr" style="outline: currentcolor;">(1)</span></li></ul></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="font-family: arial; outline: currentcolor;">Thursday, April 6, 2023 </div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; outline: currentcolor;">WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1. </div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html</a></div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">WEDNESDAY, MARCH 29, 2023 </span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;"><br style="outline: currentcolor;" /></span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">The use of animal by-products in a circular bioeconomy: Time for a TSE road map 3? </span></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html</a><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">ZOONOSIS, ZOONOTIC, TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PrP UPDATE 2023</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><p style="background-color: #fff3db; color: #29303b; font-family: Georgia, "New sans-serif"; line-height: 1.5em; margin: 0px 0px 0.6em; outline: currentcolor; padding: 0px;"><span style="background-color: white; color: #1d2228; font-family: arial; outline: currentcolor;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</span></p><div style="font-family: arial; outline: currentcolor;"><div style="outline: currentcolor;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifin1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022 / Published online: 22 August 2022 © The Author(s) 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerst-mann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent pas-sage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable. Here, humanized mice inoculated with CWD deer isolates had an atypical onset of the disease with myoclonus (93.75%), before presenting typical clinical signs, generating prions that presented with either atypical biochemical signature (#321 and #3063), shed in feces (#327), or were undetectable by the classical detection methods. The fact that we could not establish a strong correlation between disease manifestation in 3tg650 mice inoculated with Wisc-1- or 116AG-CWD and the presence of abnormal PrP (Western blot, IHC or RT-QuIC) might be explained by the presence of heterogeneous prions in the brains of infected mice with different seeding properties in vitro. Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43]. We could not establish a correlation between the presence of PrPSc deposits in mouse #328 and the absence of resist-ant PrP in Western blot even though prions present in the brain of this animal seeded the conversion of rPrP sub-strate very well (Fig. 1). Mouse #321 was not available for IHC; therefore, we cannot draw a conclusion about abnormal PrP deposits in the brain of this animal, but we can conclude that there is a correlation between level of prion seeding activity (Fig. 1) and the detection of atypical PrPres fragments (Fig. 2) present in the brain of this animal. We can only speculate that transmission of Wisc-1 to tg650 mice generated prions with characteristics that render them less prone to be unveiled with conventional methods. However, 77.7% of these mice showed prion seeding activity despite the absence of PK-resistant PrPSc for most of them, suggesting that protease-sensitive prions could be involved in these transmissions like it was shown in human prion diseases [54]. Further analyses such as conformation-dependent immunoassay [54] and in vivo sub-passages will be required to resolve these questions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the dis-ease within humans might become endemic. In contrast to bank voles inoculated with fecal homogenates from mouse #327, so far, we could not detect a PK-resistant PrPSc fragment in the brain homogenates of fecal homogenate-inoculated tg650 mice. The presence of PrPres in these mice will allow us to determine if the molecular signature of hCWD prions from the brain (mouse #321 and #3063) vs feces are the same. Previously, Beringue et al. found that extraneural prions, compared to neural prions, helped more to over-come the species barrier to foreign prions, in addition, different strain types emerged from such serial transmission [11]. Our data also suggest that prions found in the periphery may hold higher zoonotic potential than prions found in neural tissues. In fact, upon second passage, 50% of the tg650 mice inoculated with fecal homogenates from mouse #327 had succumbed with terminal disease compared to only 20% of brain/spinal cord homogenates inoculated-tg650 mice suggesting that hCWD prions found in feces transmit disease more efficiently. Our results also suggest that epidemiological studies [25] may have missed sub-clinical and atypical infections that are/might be transmissible, undetected by the gold standard tests, i.e., Western blot, ELISA, and IHC.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ideally, an oral inoculation of mice with CWD prions would have mimicked best the natural route of exposure to acquired prion diseases like CWD. However, in our case, an oral inoculation would have been impossible considering the lifespan of the rodent models. It took us an extended amount of time, over two years and a half, to have a better idea of the extent of CWD transmission in the tg650 model, even with intracerebral inoculation, usually the fastest way to induce prion disease in a rodent model. Taking this into consideration, our study is the strongest proof-of-principle that CWD might be transmissible to humans. The overall risk for zoonotic transmission of CWD is likely lower than that for BSE; however, rather than predicting the absolute zoonotic risk for CWD, our study indicates the possibility of atypical features in humans. Furthermore, our findings provide striking insights into how CWD might manifest in humans and the impact it may have on human health. We have used Wisc-1/CWD1, one of the most common CWD strains, notably white-tailed deer prions, which have been shown to be more prone to generate human prions in vitro [47]. This implies a high risk of exposure to this strain, e.g., through consumption or handling of infected carcasses, in contrast to rarer CWD strains, and therefore, an actual risk for human health. Fecal shedding of infectious prions, if it occurs in humans, is particularly concerning because of potential human-to-human transmission and adaptation of hCWD. Overall, our findings suggest that CWD surveil-lance in humans should encompass a wider spectrum of tissues/organs tested and include new criteria in the diagnosis of potential patients.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://link.springer.com/epdf/10.1007/s00401-022-02482-9?sharing_token=Vrc9lVnCx6nATJCPtcFbwPe4RwlQNchNByi7wbcMAY4BjPjkpxdzH-iFThODxSPKAK1QgQ-_2ZGPNoYIZyD3IDH8BQbGxEd1NHRwsyD-plz3_gnMpuGB-13C4DkxsBjdAJb3TEqBq0mBLdO-wl3E0flYPQbhh654WM_ijkAKt_U=&fbclid=IwAR0urg8a9eojZqpU6GePUgt5fQxsWqxsxwTzF_E_MktS3jf11wDxZP9Jy10" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://link.springer.com/epdf/10.1007/s00401-022-02482-9?sharing_token=Vrc9lVnCx6nATJCPtcFbwPe4RwlQNchNByi7wbcMAY4BjPjkpxdzH-iFThODxSPKAK1QgQ-_2ZGPNoYIZyD3IDH8BQbGxEd1NHRwsyD-plz3_gnMpuGB-13C4DkxsBjdAJb3TEqBq0mBLdO-wl3E0flYPQbhh654WM_ijkAKt_U=&fbclid=IwAR0urg8a9eojZqpU6GePUgt5fQxsWqxsxwTzF_E_MktS3jf11wDxZP9Jy10</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">SEE A FEW HIGHLIGHTS;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><span style="background-color: #fcfcfc; color: #333333; font-family: Georgia, Palatino, serif; font-size: 18px; outline: currentcolor;">''Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model.''</span><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">''Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission.'' ''These findings have strong implications for public health and CWD management.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions.'' </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage.'' </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for ''infection. ''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43].''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''This implies a high risk of exposure to this strain, e.g., through consumption or handling of infected carcasses, in contrast to rarer CWD strains, and therefore, an actual risk for human health.'' </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Fecal shedding of infectious prions, if it occurs in humans, is particularly concerning because of potential human-to-human transmission and adaptation of hCWD.'' </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Overall, our findings suggest that CWD surveillance in humans should encompass a wider spectrum of tissues/organs tested and include new criteria in the diagnosis of potential patients.</div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">***> PLEASE NOTE;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">''Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defned by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profle and the N-terminal cleavage site.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable. Here, humanized mice inoculated with CWD deer isolates had an atypical onset of the disease with myoclonus (93.75%), before presenting typical clinical signs, generating prions that presented with either atypical biochemical signature (#321 and #3063), shed in feces (#327), or were undetectable by the classical detection methods. The fact that we could not establish a strong correlation between disease manifestation in tg650 mice inoculated with Wisc-1- or 116AG-CWD and the presence of abnormal PrP (Western blot, IHC or RTQuIC) might be explained by the presence of heterogeneous prions in the brains of infected mice with diferent seeding properties in vitro. Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43].''</div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">VPSPr, GSS, and CWD zoonosis, concerns there from, where did i hear this concern before?</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">wow, just wow, i don't know where to start with Canada and CJD and strange neurological conditions mounting. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">seems the New Brunswick cases just sill not go away, and CJD cases in Canada keep rising.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report Update January 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(PLEASE NOTE, ''The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians.'' HAS BEEN USED 'ad nauseam' YEAR, AFTER YEAR, AFTER YEAR, OF RISKING CJD CASES.)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(PLEASE NOTE, OCTOBER OF 2022 THERE WERE 65 CJD CASES REPORTED, TWO MONTHS LATER, DECEMBER 2022, CJD CASES JUMPED TO 154 CASES REPORTED...tss)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(PLEASE NOTE, STILL, CANADA DOES NOT REPORT VPSPr CJD TSE Prion Cases to the public, see links below for this explanation and discussion. tss)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">THIS problem must be addressed immediately imo.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">QUOTE;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">end</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://vpspr.blogspot.com/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</div></div></div></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">SNIP...SEE FULL TEXT;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">SUNDAY, APRIL 9, 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/04/transmission-of-cervid-prions-to.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/04/transmission-of-cervid-prions-to.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="font-family: arial; outline: currentcolor;" /></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada. Grant number: 1R01NS121016-01; 201,600,023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Eric Cassmanna, Xu Qib, Qingzhong Kongb, and Justin Greenleea</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aNational Animal Disease Center, Agricultural Research Service, US Department of Agriculture, Ames, IA, USA bDepartments of Pathology, Neurology, National Center for Regenerative Medicine, and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer host.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Pooled brain material (GG96) from a CWD positive herd was used to oronasally inoculate two white-tailed deer with wild-type prion protein genotype and intracranially inoculate a raccoon. Brain homogenates (10% w/v) from the raccoon and the two white-tailed deer were used to intracranially inoculate separate groups of transgenic mice that express human prion protein with methionine (M) at codon 129 (Tg40h). Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Humanized transgenic mice inoculated with the raccoon passaged CWD agent from white-tailed deer exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPScwas detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPScalso was detected in brain tissue by western blot and immunohistochemistry. No PrPScwas detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from white-tailed deer did not have detectable PrPScusing conventional immunoassay techniques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: The host range of the CWD agent from white-tailed deer was expanded in our experimental model after one passage through raccoons.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Quazetta Brown, Lexi Frese, Rylie Frese, Kevin Hassall, Leisa Mandell, and Trudy Tatum for providing excellent technical support to this project.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stable and highly zoonotic cervid prion strain is possible</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, and Qingzhong Kong Department of Pathology, Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in some areas. Multiple in vitro conversion experiments and in vivo animal studies suggest that the CWD-to-human transmission barrier is not unbreakable. A major public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: We inoculated a few sCJD brain samples into cervidized transgenic mice, which were intended as negative controls for bioassays of brain tissues from sCJD cases who had hunted or consumed vension from CWD-endemic states. Some of these mice became infected and their brain tissues were further examined by serial passages in humanized or cervidized mice.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a ‘cervidized’ CJD strain that we termed CJDElkPrP. We observed 100% transmission of CJDElkPrPin transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrPtwo more times in the Tg12 mice. We found that such second and third passage CJDElkPrPprions also led to 100% infection in the Tg40h mice. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice, despite that natural elk CWD isolates and CJDElkPrPshare the same elk PrP sequence.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data demonstrate that highly zoonotic cervid prion strains are not only possible but also can be stably maintained in cervids and that CWD zoonosis is prion strain-dependent.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: R01NS052319, R01NS088604, R01NS109532</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O’Rourke for providing the sCJD samples and the CWD samples, respectively.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Adaptation of chronic wasting disease (CWD) prion strains in hosts with different PRNP genotypes</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Camilo Duque Velasqueza,c, Elizabeth Triscotta,c, Chiye Kima,c, Diana Morenoa,c, Judd Aikenb,c, and Debbie McKenziea,c</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Biological Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; bDepartment of Agriculture, Food & Nutritional Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; cCentre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2M8, Canada</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The contagious nature of CWD epizootics and the PrPCamino acid variation of cervids (and susceptible sympatric species) guarantee the expansion of prion conformational diversity and selective landscapes where new strains can arise. CWD strains can have novel transmission properties including altered host range that may increase zoonotic risk as circulating strains diversify and evolve. We are characterizing the host adaptability of characterized CWD strains as well as CWD isolates from different cervid species in various enzootic regions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Characterized CWD strains as well as a number of isolates from hunter-harvested deer were bioassayed in our rodent panel (transgenic mice expressing cervid alleles G96, S96 and H95-PrPC, elk PrPC, bovine PrPC, and both hamsters and non-transgenic laboratory mice). Strain characteristics were compared using computer based scoring of brain pathology (e.g. PrPCWDbrain distribution), western blot and protein misfolding cyclic amplification (PMCA).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Transmission of various isolates resulted in the selection of strain mixtures in hosts expressing similar PrPC, particularly for polymorphic white-tailed deer and for Norwegian reindeer. As of the second passage, transmission of P153 moose prions from Norway has not resulted in emergence of strains with properties similar to any North American CWD strains in our taxonomic collection (Wisc-1, CWD2, H95+and 116AG).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data indicates polymorphic white-tailed deer can favor infection with more than one strain. Similar to transmission studies of Colorado CWD isolates from cervids expressing a single PrPCprimary structure, the isolate from Norway reindeer (V214) represents a strain mixture, suggesting intrinsic strain diversity in the Nordfjella epizootic. The diversity of CWD strains with distinct transmission characteristics represents a threat to wildlife, sympatric domestic animals and public health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: Genome Canada and Genome Alberta (Alberta Prion Research Institute and Alberta Agriculture & Forestry); NSERC Grant number: #LSARP 10205; NSERC RGPIN-2017-05539</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We would like to thank Margo Pybus (Alberta Environment and Parks) Trent Bollinger (University of Saskatchewan) for providing us with tissue samples from hunter-harvested deer and Sylvie Benestad for providing moose and reindeer samples.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Application of PMCA to understand CWD prion strains, species barrier and zoonotic potential</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sandra Pritzkowa, Damian Gorskia, Frank Ramireza, Fei Wanga, Glenn C. Tellingb, Justin J. Greenleec, Sylvie L. Benestadd, and Claudio Sotoa aDepartment of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA; bDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA; cVirus and Prion Research Unit, United States Department of Agriculture, Ames, Iowa, USA; dNorwegian Veterinary Institute, OIE Reference Laboratory for CWD, Ås, Norway</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) is a prion disease affecting various species of cervids that continues to spread uncontrollably across North America and has recently been detected in Scandinavia (Norway, Sweden and Finland). The mechanisms responsible for the natural transmission of CWD are largely unknown. Furthermore, the risk of CWD transmission to other species, including humans, is also unknown and remains a dangerous enigma. In this study, we investigated the potential of CWD prions to infect several other animal species (sheep, cattle, pig, hamster, and mouse) including humans, by examining their capacity to convert the normal prion protein of distinct species in a PMCA reaction. Moreover, we also investigated whether the in vivo passage of CWD through intermediate species alters their capacity for zoonotic transmission, which may represent a major hazard to human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: For these studies, we used brain material from CWD-infected white-tailed deer (Odocoileus virginianus), elk (Cervus canadensis), and mule deer (Odocoileus hemionus) as species native to North America. We also used CWD-infected Moose (Alces alces), reindeer (Rangifer tarandus) and red deer (Cervus elaphus) as Norwegian cervids. We also used brains from cattle, sheep and pigs experimentally infected by CWD. To study interspecies-transmission and zoonotic potential, samples were tested via PMCA for the conversion of PrPCinto PrPScusing different combinations of inoculum and host species. Based on these analyses we estimated the spillover and zoonotic potential for different CWD isolates. We define and quantify spillover and zoonotic potential indices as the efficiency by which CWD prions sustain prion generation in vitro at the expense of normal prion proteins from various mammals and human, respectively.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show that prions from some cervid species, especially those found in Northern Europe, have a higher potential to transmit disease characteristics to other animals. Conversely, CWD-infected cervids originated in North America appear to have a greater potential to generate human PrPSc. We also found that in vivo transmission of CWD to cattle, but not to sheep or pigs substantially increases the ability of these prions to convert human PrPCby PMCA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our findings support the existence of different CWD prion strains with distinct spillover and zoonotic potentials. We also conclude that transmission of CWD to other animal species may increase the risk for CWD transmission to humans. Our studies may provide a tool to predict the array of animal species that a given CWD prion could affect and may contribute to understanding the risk of CWD for human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: National Institute of Health Grant number: P01 AI077774</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Generation of human chronic wasting disease in transgenic mice</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Zerui Wanga, Kefeng Qinb, Manuel V. Camachoa, Ignazio Cali a,c, Jue Yuana, Pingping Shena, Tricia Gillilanda, Syed Zahid Ali Shaha, Maria Gerasimenkoa, Michelle Tanga, Sarada Rajamanickama, Anika Yadatia, Lawrence B. Schonbergerd, Justin Greenleee, Qingzhong Konga,c, James A. Mastriannib, and Wen-Quan Zoua,c</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA; bDepartment of Neurology and Center for Comprehensive Care and Research on Memory Disorders, the University of Chicago Pritzker School of Medicine, Chicago, USA; cNational Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; dDivision of High-Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA, USA; eVirus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, Ames, IA, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) results from the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC) in the brains of deer and elk. It has been spreading rapidly throughout many regions of North America, exported inadvertently to South Korea, and more recently identified in Europe. Mad cow disease has caused variant Creutzfeldt-Jakob disease (vCJD) in humans and is currently the only known zoonotic prion disease. Whether CWD is transmissible to humans remains uncertain. The aims of our study were not only to confirm whether CWD prion isolates can convert human brain PrPCinto PrPScin vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPScis infectious.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Eight CWD prion isolates from 7 elks and 1 deer were used as the seeds while normal human brain homogenates containing either PrP-129 MM (n = 2) or PrP-129 VV (n = 1) were used as the substrates for sPMCA assay. A normal elk brain tissue sample was used as a negative control seed. Two lines of humanized transgenic (Tg) mice expressing either human PrP-129VV or −129 MM polymorphism were included for transmission studies to determine the infectivity of PMCA-amplified PrPSc. Wester blotting and immunohistochemistry and hematoxylin & eosin staining were used for determining PrPScand neuropathological changes of inoculated animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: We report here the generation of the first CWD-derived infectious human PrPScusing elk CWD PrPScto initiate conversion of human PrPCfrom normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPScwas derived from the human brain PrPCsubstrate. Two lines of humanized transgenic mice expressing human PrPCwith either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPScpatterns and neuropathological changes in the brain.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSchas the potential to overcome the species barrier and directly convert human PrPCinto infectious PrPScthat can produce bona fide prion disease when inoculated into humanized transgenic mice.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: CJD Foundation and NIH</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Mortality surveillance of persons potentially exposed to chronic wasting disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">R.A. Maddoxa, R.F. Klosb, L.R. Willb, S.N. Gibbons-Burgenerb, A. Mvilongoa, J.Y. Abramsa, B.S. Applebyc, L.B. Schonbergera, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bWisconsin Department of Health Services (WDHS), Division of Public Health, Madison, USA; cNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: It is unknown whether chronic wasting disease (CWD), a prion disease of cervids, can infect people, but consumption of meat from infected animals would be the most likely route of transmission. Wisconsin Department of Health Services, Division of Public Health (WDHS) personnel maintain a database consisting of information collected from hunters who reported eating, or an intention to eat, venison from CWD-positive cervids. These data, collected since 2003, allow for the evaluation of causes of mortality in individuals potentially exposed to CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: The WDHS database contains the name, date of birth, when available, year of CWD-positive deer harvest, and city and state of residence for each potentially exposed individual. The database also includes information on how the deer was processed (self-processed or by a commercial operator) and when applicable, names of others with whom the venison was shared. Duplicate entries (i.e., those who consumed venison from CWD-positive deer in multiple hunt years) are determined by first name, last name, and date of birth. All names in the database are cross-checked with reported cases of human prion disease in Wisconsin and cases in the National Prion Disease Pathology Surveillance Center (NPDPSC) diagnostic testing database. Persons with date of birth available are also cross-checked with prion disease decedents identified through restricted-use national multiple cause-of-death data via a data use agreement with the National Center for Health Statistics (NCHS).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: The database currently consists of 1561 records for hunt years 2003–2017 and 87 additional records for 2018–2019. Of these, 657 records have accompanying date of birth; 15 entries were removed as duplicates leaving 642 unique individuals. Of these individuals, 278 of 426 (66%) who ate venison from a CWD-positive deer and provided processing information reported self-processing. No matches were found among any persons in the database cross-checked with WDHS human prion disease surveillance data, NPDPSC data (February 2022 update), and NCHS data through 2020.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Because of the linkage of person and CWD-positive animal in the WDHS database, reviewing the cause of mortality in potentially exposed persons is possible. The number of individuals cross-checked so far is likely only a small percentage of those potentially exposed to CWD in Wisconsin, and many more years of vital status tracking are needed given an expected long incubation period should transmission to humans occur. Nevertheless, the findings of this ongoing review are thus far reassuring.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion disease incidence, United States, 2003–2020</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">R.A. Maddoxa, M.K. Persona, K. Kotobellib, A. Mvilongoa, B.S. Applebyb, L.B. Schonbergera, T.A. Hammetta, J.Y. Abramsa, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Mortality data, in conjunction with neuropathological and genetic testing results, are used to estimate prion disease incidence in the United States.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Prion disease decedents for 2003–2020 were identified from restricted-use U.S. national multiple cause-of-death data, via a data use agreement with the National Center for Health Statistics, and from the National Prion Disease Pathology Surveillance Center (NPDPSC) database. NPDPSC decedents with neuropathological or genetic test results positive for prion disease for whom no likely match was found in the NCHS multiple cause-of-death data were added as cases for incidence calculations, while those with negative neuropathology results but with cause-of-death data indicating prion disease were removed. Unmatched cases in the NPDPSC database lacking neuropathological testing but with a positive real-time quaking-induced conversion (RT-QuIC) test result were additionally assessed. Age-specific and age-adjusted average annual incidence rates were calculated from the combined data; the year 2000 as the standard population and the direct method were used for age-adjustment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: A total of 7,921 decedents were identified as having prion disease during 2003–2020 for an age-adjusted average annual incidence of 1.2 per million population. The age-adjusted incidence between males and females (1.3 and 1.1 per million, respectively) differed significantly (p < 0.0001). The age-specific average annual incidence among those <55 and ≥55 years of age was 0.2 and 4.8 per million, respectively; incidence among those ≥65 was 6.1 per million. Eighteen cases were <30 years of age for an age-specific incidence of 8.0 per billion; only 6 of these very young cases were sporadic (3 sporadic CJD, 3 sporadic fatal insomnia), with the rest being familial (9), variant (2), or iatrogenic (1). The age-adjusted annual incidence for the most recent year of data, 2020, was 1.3 per million. However, assessment of RT-QuIC positive cases lacking neuropathology in the NPDPSC database suggested that approximately 20% more cases may have occurred in that year; the addition of a subset of these cases that had date of death information available (n = 44) increased the 2020 rate to 1.4 per million.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Mortality data supplemented with the results of neuropathological, CSF RT-QuIC, and genetic testing can be used to estimate prion disease incidence. However, the identification in the NPDPSC database of RT-QuIC-positive cases lacking date of death information suggests that this strategy may exclude a number of probable prion disease cases. Prion disease cases <30 years of age, especially those lacking a pathogenic mutation, continue to be very rare.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: National Institutes of Health (NIH)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: USDA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: AP20VSSPRS00C143</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ATYPRION project: assessing the zoonotic potential of interspecies transmission of CWD isolates to livestock (preliminary results).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Enric Vidala,b, Juan Carlos Espinosac, Samanta Gilera,b, Montserrat Ordóñeza,b, Guillermo Canteroa,b, Vincent Béringued, Justin J. Greenleee, and Juan Maria Torresc</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aUnitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; bIRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; cCentro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid, Spain; dMolecular Virology and Immunology, French National Research Institute for Agriculture, Food and Environment (INRAE), Université Paris-Saclay, Jouy-en-Josas, France; eVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Since variant Creutzfeldt-Jackob disease was linked to the consumption of bovine spongiform encephalopathy prions, the study of the pathobiological features of animal prions, particularly their zoonotic potential, is of great concern to the scientific community and public health authorities. Furthermore, interspecies transmission of prions has been demonstrated as a putative evolutionary mechanism for prions, that can lead to the emergence of new features including the ability to infect humans. For instance, small ruminants’ atypical scrapie prions, when propagated in a bovine or porcine host, can shift to a classical BSE phenotype thus posing a potential risk in case of human exposure. So far, no hard evidence of zoonotic transmission of cervids’ chronic wasting disease (CWD) to humans has been published, however experimental transmission to bovine, ovine and caprine hosts has been achieved. Our goal is to investigate if, once passaged through these domestic species, CWD prions might become infectious to humans.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Different CWD isolates experimentally adapted to cattle, sheep and goat (Hamir et al, 2005, 2006, 2007, Greenlee et al 2012) have been intracerebrally inoculated to transgenic mouse models expressing the human cellular prion protein either homozygous for methionine or valine at codon 129 (Tg340-Met129 and Tg362-Val129). Additionally, inocula obtained from experimental transmission of elk CWD to ovinized (Tg501) and bovinized (BoTg110) transgenic mice, as well as white-tailed deer CWD to BoTg110 mice, are currently being bioassayed in both human PrPCtransgenic models.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results and conclusions: No evidence of transmission has been found on first passage for bovine adapted elk and mule deer CWD to none of the humanized models. The remaining bioassays are ongoing without showing clinical signs yet, as well as second passages for the negative 1stpassages.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: La Marató de TV3 foundation. Grant number: ATYPRION (201,821–30-31-32)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion Conference 2018 Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P172 Peripheral Neuropathy in Patients with Prion Disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Wang H(1), Cohen M(1), Appleby BS(1,2)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P180 Clinico-pathological analysis of human prion diseases in a brain bank series</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background and objective:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">See also poster P103</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WA16 Monitoring Potential CWD Transmission to Humans</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Belay ED</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Source Prion Conference 2018 Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://prionconference.blogspot.com/2018/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://prionconference.blogspot.com/2018/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion 2017 Conference Abstracts </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This is a progress report of a project which started in 2009. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In four animals wasting was observed, two of those had confirmed diabetes. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">At present, a total of 10 animals are sacrificed and read-outs are ongoing. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><a href="https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf</a><br style="outline: currentcolor;" /><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SATURDAY, FEBRUARY 23, 2019 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, NOVEMBER 04, 2014 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;">2004</span></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;"><br style="outline: currentcolor;" /></span></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;">Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD </span><br style="outline: currentcolor;" /></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;"><br style="outline: currentcolor;" /></span></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium; outline: currentcolor;">????: CBCnews</strong><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;"><br style="outline: currentcolor;" /></span></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;"><br style="outline: currentcolor;" /></span></span></div><div style="font-size: 13.3333px; outline: currentcolor;"><span style="background-color: whitesmoke; outline: currentcolor;"><span face="Arial, Helvetica, sans-serif" style="font-size: medium; font-weight: 700; outline: currentcolor;"><a href="https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html</a></span></span></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission Studies</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip.... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://jvi.asm.org/content/83/18/9608.full" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://jvi.asm.org/content/83/18/9608.full</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prions in Skeletal Muscles of Deer with Chronic Wasting Disease </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://science.sciencemag.org/content/311/5764/1117..long" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://science.sciencemag.org/content/311/5764/1117..long</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: TSS </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: CWD aka MAD DEER/ELK TO HUMANS ???</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: September 30, 2002 at 7:06 am PST</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: "Belay, Ermias"</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sent: Monday, September 30, 2002 9:22 AM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dear Sir/Madam,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ermias Belay, M.D. Centers for Disease Control and Prevention</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">-----Original Message-----</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: Sent: Sunday, September 29, 2002 10:15 AM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thursday, April 03, 2008</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip... full text ; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">sporadic = 54,983 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">spontaneous = 325,650 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ *** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: Steve Dealler </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: BSE-L@ References: </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dear Terry,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Steve Dealler </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Table 9 presents the results of an analysis of these data.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full report ;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE Inquiry Steve Dealler</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Management In Confidence</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE: Private Submission of Bovine Brain Dealler</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full text;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">MONDAY, FEBRUARY 25, 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***thus questioning the origin of human sporadic cases. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***thus questioning the origin of human sporadic cases*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2015 CONFERENCE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2016 TOKYO</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Saturday, April 23, 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Taylor & Francis</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WEDNESDAY, MARCH 16, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SHEEP BY-PRODUCTS AND WHAT ABOUT Scrapie TSE PrP and Potential Zoonosis? </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SO, WHO'S UP FOR SOME MORE TSE PRION POKER, WHO'S ALL IN $$$ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SO, ATYPICAL SCRAPIE ROUGHLY HAS 50 50 CHANCE ATYPICAL SCRAPIE IS CONTAGIOUS, AS NON-CONTAGIOUS, TAKE YOUR PICK, BUT I SAID IT LONG AGO WHEN USDA OIE ET AL MADE ATYPICAL SCRAPIE A LEGAL TRADING COMMODITY, I SAID YOUR PUTTING THE CART BEFORE THE HORSE, AND THAT'S EXACTLY WHAT THEY DID, and it's called in Texas, TEXAS TSE PRION HOLDEM POKER, WHO'S ALL IN $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686</a></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Paper</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Cellular prion protein distribution in the vomeronasal organ, parotid, and scent glands of white-tailed deer and mule deer</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Anthony Ness, Aradhana Jacob, Kelsey Saboraki, Alicia Otero, Danielle Gushue, Diana Martinez Moreno, Melanie de Peña, Xinli Tang, Judd Aiken, Susan Lingle & Debbie McKenzie ORCID Icon show less</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Pages 40-57 | Received 03 Feb 2022, Accepted 13 May 2022, Published online: 29 May 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Download citation</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://doi.org/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1080/19336896.2022.2079888</a></div></div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><div style="outline: currentcolor;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PIGS</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Title: Prion infectivity detected in swine challenged with chronic wasting disease via the intracerebral or oral route</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item MOORE, S - Orise Fellow item Kunkle, Robert item SMITH, JODI - Iowa State University item WEST-GREENLEE, M - Iowa State University item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 4/4/2016 Publication Date: N/A Citation: N/A</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of North American cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. In the US, feeding of ruminant by-products to ruminants is prohibited, but feeding of ruminant materials to swine, mink, and poultry still occurs. In addition, scavenging of CWD-affected cervid carcasses by feral pigs presents a potential risk for CWD exposure. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following oral or intracranial experimental challenge. At 8 weeks of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). At death a complete necropsy examination was performed, including testing of tissues for misfolded prion protein (PrPcwd) by western blotting (WB), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry (IHC). None of the pigs developed clinical signs consistent with prion disease. Four >6 month intracranially challenged pigs (survival times 45-73 mpc) were positive by ELISA, two were also positive by WB, and one was positive by IHC. One >6 month orally challenged pig (64 mpc) was positive by ELISA. To further investigate the potential for infectivity, brain tissue from selected pigs was bioassayed in mice expressing porcine PRNP. Tissue from the two WB-positive >6 month intracranially challenged pigs produced positive bioassay results, albeit with low attack rates and variable incubation periods. Interestingly, bioassay of material from the longest surviving >6 month orally challenged pig (72 mpc), which was negative for PrPcwd by all other tests, produced a positive bioassay result. Bioassay of material from additional animals is currently underway. This study demonstrates that pigs can serve as potential hosts for CWD, although with low attack rates and scant PrPcwd accumulation. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Detection of infectivity in orally challenged pigs using mouse bioassay raises the possibility that naturally exposed pigs act as a reservoir of CWD infectivity, even though affected pigs do not develop overt clinical signs or readily detectable PrPcwd.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see full report;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2017 Annual Report</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Objectives</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Objective 1: Investigate the mechanisms of protein misfolding in prion disease, including the genetic determinants of misfolding of the prion protein and the environmental influences on protein misfolding as it relates to prion diseases. Subobjective 1.A: Investigate the differences in the unfolded state of wild-type and disease associated prion proteins to better understand the mechanism of misfolding in genetic prion disease. Subobjective 1.B: Investigate the influence of metal ions on the misfolding of the prion protein in vitro to determine if environmental exposure to metal ions may alter disease progression. Objective 2: Investigate the pathobiology of prion strains in natural hosts, including the influence of prion source genotype on interspecies transmission and the pathobiology of atypical transmissible spongiform encephalopathies (TSEs). Subobjective 2.A: Investigate the pathobiology of atypical TSEs. Subobjective 2.B: Investigate the influence of prion source genotype on interspecies transmission. Objective 3: Investigate sampling methodologies for antemortem detection of prion disease, including the utility of blood sampling as a means to assess prion disease status of affected animals and the utility of environmental sampling for monitoring herd prion disease status. Subobjective 3.A: Investigate the utility of blood sampling as a means to assess prion disease status of affected animals. Subobjective 3.B: Investigate the utility of environmental sampling for monitoring herd prion disease status.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Approach</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The studies will focus on three animal transmissible spongiform encephalopathy (TSE) agents found in the United States: bovine spongiform encephalopathy (BSE); scrapie of sheep and goats; and chronic wasting disease (CWD) of deer, elk, and moose. The research will address sites of protein folding and misfolding as it relates to prion disease, accumulation of misfolded protein in the host, routes of infection, and ante mortem diagnostics with an emphasis on controlled conditions and natural routes of infection. Techniques used will include spectroscopic monitoring of protein folding/misfolding, clinical exams, histopathology, immunohistochemistry, and biochemical analysis of proteins. The enhanced knowledge gained from this work will help understand the underlying mechanisms of prion disease and mitigate the potential for unrecognized epidemic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Progress Report</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All 8 project plan milestones for FY17 were fully met. Research efforts directed toward meeting objective 1 of our project plan center around the production of recombinant prion protein from either bacteria or mammalian tissue culture systems and collection of thermodynamic data on the folding of the recombinant prion protein produced. Both bacterial and mammalian expression systems have been established. Thermodynamic data addressing the denatured state of wild-type and a disease associated variant of bovine prion protein has been collected and a manuscript is in preparation. In research pertaining to objective 2, all studies have been initiated and animals are under observation for the development of clinical signs. The animal studies for this objective are long term and will continue until onset of clinical signs. In vitro studies planned in parallel to the animals studies have similarly been initiated and are ongoing. Objective 3 of the project plan focuses on the detection of disease associated prion protein in body fluids and feces collected from a time course study of chronic wasting disease inoculated animals. At this time samples are being collected as planned and methods for analysis are under development.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Accomplishments</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1. Showed that swine are potential hosts for the scrapie agent. A naturally occurring prion disease has not been recognized in swine, but the agent of bovine spongiform encephalopathy does transmit to swine by experimental routes. Swine are thought to have a robust species barrier when exposed to the naturally occurring prion diseases of other species, but the susceptibility of swine to the agent of sheep scrapie has not been thoroughly tested. ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. Developed a method for amplification and discrimination of the 3 forms of BSE in cattle. The prion protein (PrP) is a protein that is the causative agent of transmissible spongiform encephalopathies (TSEs). The disease process involves conversion of the normal cellular PrP to a pathogenic misfolded conformation. This conversion process can be recreated in the lab using a misfolding amplification process known as real-time quaking induced conversion (RT-QuIC). RT-QuIC allows the detection of minute amounts of the abnormal infectious form of the prion protein by inducing misfolding in a supplied substrate. Although RT-QuIC has been successfully used to detect pathogenic PrP with substrates from a variety of host species, prior to this work bovine prion protein had not been proven for its practical uses for RT-QuIC. We demonstrated that prions from transmissible mink encephalopathy (TME) and BSE-infected cattle can be detected with using bovine prion proteins with RT-QuIC, and developed an RT-QuIC based approach to discriminate different forms of BSE. This rapid and robust method, both to detect and discriminate BSE types, is of importance as the economic implications for different types of BSE vary greatly.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Review Publications</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">cwd scrapie pigs oral routes </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CONFIDENTIAL</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">LINE TO TAKE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div></div></div></div><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Wednesday, July 28, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">France issues moratorium on prion research after fatal brain disease strikes two lab workers</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Volume 26, Number 8—August 2020</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/08/sporadic-creutzfeldt-jakob-disease.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/08/sporadic-creutzfeldt-jakob-disease.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Saturday, January 23, 2021</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Improved surveillance of surgical instruments reprocessing following the variant Creutzfeldt-Jakob disease crisis in England: findings from a 3-year survey</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://itseprion.blogspot.com/2021/01/improved-surveillance-of-surgical.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://itseprion.blogspot.com/2021/01/improved-surveillance-of-surgical.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2019/09/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2019/09/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SUNDAY, JULY 19, 2020 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Joseph J. Zubak Orthopaedic surgeon passed away Monday, July 6, 2020, Creutzfeldt-Jakob Disease (CJD)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2020/07/joseph-j-zubak-orthopaedic-surgeon.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Friday, January 29, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Scientists identify locations of early prion protein deposition in retina, what if?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://itseprion.blogspot.com/2021/01/scientists-identify-locations-of-early.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://itseprion.blogspot.com/2021/01/scientists-identify-locations-of-early.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">FRIDAY, SEPTEMBER 06, 2019 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Disinfection of Multi-Use Ocular Equipment for Ophthalmological Procedures: A Review of Clinical Effectiveness, Cost-Effectiveness, and Guidelines</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2019/09/disinfection-of-multi-use-ocular.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2019/09/disinfection-of-multi-use-ocular.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, OCTOBER 26, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sporadic Creutzfeldt-Jakob Disease in a Very Young Person Singeltary Reply 2021</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2021/10/sporadic-creutzfeldt-jakob-disease-in.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/10/sporadic-creutzfeldt-jakob-disease-in.html</a></div></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">Thursday, April 6, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1. </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html" rel="nofollow" style="color: #338fe9; outline: 0px;" target="_blank">https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html</a></div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; outline: currentcolor;">rather a large outbreak of a NEW PRION DISEASE IN CAMELS, and they have not a clue whether it's zoonotic or not...</div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="color: #26282a; outline: currentcolor;">Monday, November 14, 2022 </div><div dir="ltr" style="color: #26282a; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="color: #26282a; outline: currentcolor;">Prion Diseases in Dromedary Camels (CPD) 2022 Review </div><div dir="ltr" style="color: #26282a; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="color: #26282a; outline: currentcolor;"><a href="https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html</a></div></div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: arial; outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">WEDNESDAY, MARCH 29, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">The use of animal by-products in a circular bioeconomy: Time for a TSE road map 3? </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">SUNDAY, MARCH 19, 2023 </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;">Abandoned factory ‘undoubtedly’ contains dormant Mad Cow Disease that could threaten humans, Thruxted Mill, Queniborough CJD<br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><a href="https://bseinquiry.blogspot.com/2023/03/abandoned-factory-undoubtedly-contains.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bseinquiry.blogspot.com/2023/03/abandoned-factory-undoubtedly-contains.html</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; outline: currentcolor;"><br style="outline: currentcolor; text-align: justify;" /></div></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;"><div dir="ltr" style="font-size: 13.3333px; letter-spacing: inherit; outline: currentcolor; text-align: justify;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;"><span style="letter-spacing: inherit; outline: currentcolor;">Notice of Request To Renew an Approved Information Collection: Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission</span><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Greetings FSIS, USDA, et al,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thank you kindly for allowing the public to comment on ;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(a) whether the proposed collection of information is necessary for the proper performance of FSIS’ functions, including whether the information will have practical utility;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(b) the accuracy of FSIS’ estimate of the burden of the proposed collection of information, including the validity of the method and assumptions used;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(c) ways to enhance the quality, utility, and clarity of the information to be collected; and</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(d) ways to minimize the burden of the collection of information, including through the use of appropriate automated, electronic, mechanical, or other technological collection techniques, or other forms of information technology.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I will be commenting mostly on a, b, and c, because d, is wanting to minimize the burden of collection, and i do not think that is possible if ''These statutes mandate that FSIS protect the public by verifying that meat, poultry, and egg products are safe, wholesome, and properly labeled and packaged.'', is truly the intent of these statutes, and i would kindly like to explain why, and why it is so critical that these Specified Risk Materials SRM TSE Prion Statues are so important for public health, and WHY there is an urgent need to enhance them, considering the risk factors of Chronic Wasting Disease CWD TSE Prion in Cervid.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">THIS collection of SRM materials information should be done all the time, year after year, and ending it EVER would be foolish, imo, not scientific, and will lead to future risk to public health, if you consider just how bad USDA/FSIS/APHIS/FDA failed so badly with the FDA PART 589 TSE PRION FEED BAN, the SRM REMOVAL, THE BSE SURVEILLANCE AND TESTING PROGRAMS, THEY FAILED ALL OF THEM TERRIBLY IMO, AND BY CONTINUING TO INSIST ON TESTING 25K CATTLE FOR BSE IS A DISASTER WATING TO HAPPEND IMO!</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SPECIFIED RISK MATERS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Specified Risk Materials SRMs, are the most high risk infectious materials, organs, of a cow that is infected with Bovine Spongiform Encephalopathy, Transmissible Spongiform Encephalopathy, BSE TSE Prion. the atypical BSE strains are, like atypical L-type BSE are more infectious that the typical C-type BSE. Also, Science of the BSE TSE has evolved to show that there are more infectious tissues and organs than previously thought. I wish to kindly post all this evidence, as to show you why this information collection of SRMs are so vital to public safety, and why they should be enhanced for cattle, cervid, sheep, and goats, oh, and not to forget the new livestock prion disease in camel, the Camel Prion Disease CPD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ONE other thing, you must remember, SCIENCE AND TRANSMISSION STUDIES have now shown that CWD and Scrapie can transmit to PIGS by Oral route. This should be included in any enhancement of the SRM or FDA PART 589 TSE PRION FEED ban.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NOT to forget Zoonosis of all of the above, i will post the latest science to date at the bottom of the attached files.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thank You, terry</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary further comments in attachment;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf Monday, December 5, 2022 Notice of Request To Renew an Approved Information Collection: Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://specifiedriskmaterial.blogspot.com/2022/12/notice-of-request-to-renew-approved.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://specifiedriskmaterial.blogspot.com/2022/12/notice-of-request-to-renew-approved.html</a></div></div><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="letter-spacing: inherit; outline: currentcolor;">SEE MAD COW FEED VIOLATIONS AFER MAD COW FEED VIOLATIONS ;</span><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2016/03/docket-no-fda-2003-d-0432-formerly-03d.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2016/03/docket-no-fda-2003-d-0432-formerly-03d.html</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">PUBLIC SUBMISSION</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Use of Electronic Identification Eartags as Official Identification in Cattle and Bison APHIS-2021-0020-0001 Singeltary Submission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Comment from Singeltary Sr., Terry</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted by the Animal and Plant Health Inspection Service on Mar 21, 2023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.regulations.gov/comment/APHIS-2021-0020-0999" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0020-0999</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SEE ADDITIONAL COMMENTS IN ATTACHMENT;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div></div></div></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">Sunday, January 10, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission June 17, 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Greetings APHIS et al, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.regulations.gov/comment/APHIS-2018-0087-0002" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/APHIS-2018-0087-0002</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Comment from Singeltary Sr., Terry</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PDF]Freas, William TSS SUBMISSION File Format: PDF/Adobe Acrobat -</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20170301223601/https://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20170301223601/https://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf</a></div></div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor; text-align: justify;">Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission</div><div style="outline: currentcolor; text-align: justify;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor; text-align: justify;"><a href="https://www.regulations.gov/comment/APHIS-2021-0004-0002" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0004-0002</a></div><div style="outline: currentcolor; text-align: justify;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor; text-align: justify;"><a href="https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf</a></div><div style="outline: currentcolor; text-align: justify;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor; text-align: justify;">Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification</div><div style="outline: currentcolor; text-align: justify;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor; text-align: justify;"><a href="https://www.regulations.gov/document/APHIS-2018-0011-0003" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.regulations.gov/document/APHIS-2018-0011-0003</a></div><div style="outline: currentcolor; text-align: justify;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor; text-align: justify;"><a href="https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf</a></div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div dir="ltr" style="font-family: arial; outline: currentcolor;">Terry S. Singeltary Sr., Bacliff, Texas USA 77518 flounder9@verizon.net</div></div></div></div></div></div></div></div><div style="outline: currentcolor;"><br /></div></div></div></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-34527136840164960372023-04-24T11:35:00.001-05:002023-04-24T11:35:12.698-05:00Prion Disease on the Rise in the U.S., Now the question is, why?<p><span style="font-family: Helvetica Neue, Helvetica, Arial, sans-serif;">Prion Disease on the Rise in the U.S., Now the question is, why?</span></p><p><span style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">Prion Disease on the Rise in the U.S. — Now the question is, why?</span></p><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">by John Gever, Contributing Writer, MedPage Today April 5, 2022 </div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">SEATTLE -- The incidence of Creutzfeldt-Jakob disease (CJD), the spongiform brain malady, rose by more than half in the U.S. from 2000 to 2014, according to a new study reported here, although the reasons for the increase remain unclear.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">People were hospitalized for CJD at a rate of 1.6 per million persons in 2000 (95% CI 1.2-2.0), reported Alison Seitz, MD, of NewYork-Presbyterian Hospital in New York City, during a recorded platform presentation at the American Academy of Neurology annual meeting opens in a new tab or window.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">By 2014, she said, the rate had reached 2.7 per million (95% CI 2.2-3.2), for an average annual increase of 4.6%.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Importantly, her analysis did not adjust for the U.S. population's changing age distribution, which of course became older on average during the study period. (Seitz told MedPage Today that further analyses, including age adjustment, are still in process.) Because "sporadic" CJD, accounting for 80% to 95% of all cases, is most common in older people, some increase in crude incidence rates would be expected in an aging population.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">But growth in the older demographic has been considerably less than 4.6% annually -- more like 2% to 3%, according to a 2021 report opens in a new tab or window from the federal Administration for Community Living. That suggests something else is going on besides an increasingly top-heavy age pyramid.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Seitz noted that one possibility is simply increased attention to the presence of CJD. As it happens, 1993 was the year before "mad cow disease" exploded into the world's headlines. CJD cases linked to beef cattle opens in a new tab or window infected with bovine spongiform encephalopathy were detected in the U.K. in 1994, sparking a global panic. A few cases also occurred in the U.S., although against the background of naturally occurring CJD, they hardly registered in CDC.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Yet those same data also suggested a substantial increase in overall CJD fatalities starting in about 2000. The CDC gets its data primarily from death certificates. Seitz and colleagues decided to draw on a different data source, the National Inpatient (NIS), which might be more accurate considering all the known issues with death certificates. For one thing, the NIS has more complete information about the patients registered, including a full accounting of ICD diagnostic codes recorded for each individual. For the current study, the researchers searched for the codes 046.1, 046.11, and 046.19 to capture CJD cases.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Another nice feature of the NIS is that, while it doesn't cover all U.S. hospitalizations -- "it is a sample," Seitz observed, with about 8 million patient encounters per year -- the summary data are weighted to match overall population characteristics.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Medical News from Around the Web BBC Activity snacking may help with type 1 diabetes - study opens in a new tab or window FORBES Is Tinnitus Such As Ear-Ringing Linked To Covid-19 Vaccines? Understanding the end of the COVID public health emergency opens in a new tab or window Thus, the 1,837 CJD hospitalizations identified in the 2000-2014 NIS data extrapolate to 8,778 overall in the U.S. during the period, Seitz said.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">The overall upward trend over time wasn't steady. A spike in 2008 was bracketed by years of sharp decreases, for example. Moreover, these year-to-year changes weren't exactly matched in the CDC's mortality data, although CJD is uniformly fatal and usually within a year of diagnosis.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">Seitz also cited the possibility that hospital registrars have gotten freer with the codes for CJD, "appropriately or not," as another potential explanation for the increased incidence in NIS data.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">One potentially relevant factor Seitz didn't mention is chronic wasting (CWD), the prion-caused pathology affecting deer, elk, and related animals. The CDC says no confirmed cases of cervid-to-human transmission are known. Nevertheless, the condition has been spreading in wild deer and elk over the past two decades, now spanning "at least 27 states" in the Midwest, South, and East Coast, according to the agency's most recent statement.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">In 2021, more than 6.3 million deer are estimated to have been killed and presumably handled by hunters, so contact with infected animals seems likely, and likely to have grown over the period covered by the new study. Seitz told MedPage Today that, if CWD was a factor, one might expect that incidence rates would be higher in states where CWD is endemic. "We haven't considered doing a geographic analysis, but now you're making me think maybe we should try," she wrote in an email.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;">author['full_name'] John Gever was Managing Editor from 2014 to 2021; he is now a regular contributor.<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><a href="https://www.medpagetoday.com/meetingcoverage/aan/98043" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.medpagetoday.com/meetingcoverage/aan/98043</a><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="font-size: 16px; outline: none !important;">''The risk of CJD increases with age; the 2016–2020 average annual rate in the United States was about 5 cases per million in persons 55 years of age or older.''</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Creutzfeldt-Jakob disease deaths and age-adjusted death rate, United States, 1979–2020*</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Occurrence and Transmission</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Classic CJD has been recognized since the early 1920s. The majority of cases of CJD (about 85%) are believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide, including the United States, at a rate of roughly 1 to 2 cases per 1 million population per year. The risk of CJD increases with age; the 2016–2020 average annual rate in the United States was about 5 cases per million in persons 55 years of age or older.</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">A smaller proportion of patients (5–15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia. Death records are a good index of the incidence of CJD because the disease is always fatal, and the median duration of illness is about 4–5 months.</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;"><a href="https://www.cdc.gov/prions/cjd/occurrence-transmission.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.cdc.gov/prions/cjd/occurrence-transmission.html</a></div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">''The risk of CJD increases with age; the 2016–2020 average annual rate in the United States was about 5 cases per million in persons 55 years of age or older.''</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">*Deaths obtained from multiple cause-of-death data include all forms of human prion disease and are based on ICD-9 and ICD-10 codes and available computerized literal death certificate data. Some modifications have been made to these data based on relevant information obtained from other surveillance mechanisms. Rates are adjusted to the US standard 2000 projected population.</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Case counts include persons with probable CJD based on clinical tests such as a positive real-time quaking-induced conversion (RT-QuIC) provided a prion disease diagnosis is listed on the death certificate. When positive, the RT-QuIC test has been shown to be strongly indicative of prion disease (Rhoads, et al.) and, unless there is an alternative diagnosis, listing prion disease on the death certificate is strongly encouraged. Annual numbers of persons with positive RT-QuIC clinical tests, but lacking brain tissue analyses for disease confirmation, can be found on the National Prion Disease Pathology Surveillance Center website (NPDPSC Tables).</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Creutzfeldt-Jakob Disease Deaths and Age-Adjusted Death Rate, United States, 1979–2020* Year Deaths (approximate) Age-adjusted Death Rate</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1979 179 0.850</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1980 172 0.787</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1981 214 0.988</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1982 201 0.901</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1983 183 0.825</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1984 221 0.985</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1985 235 1.033</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1986 247 1.073</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1987 268 1.129</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1988 243 1.020</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1989 242 1.011</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1990 210 0.859</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1991 238 0.982</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1992 255 1.018</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1993 256 1.012</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1994 278 1.079</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1995 265 1.023</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1996 261 0.984</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1997 305 1.152</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1998 281 1.038</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">1999 272 0.990</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2000 238 0.853</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2001 259 0.917</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2002 260 0.904</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2003 284 0.967</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2004 279 0.921</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2005 296 0.972</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2006 290 0.925</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2007 330 1.019</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2008 352 1.086</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2009 353 1.067</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2010 396 1.149</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2011 409 1.161</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2012 380 1.054</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2013 478 1.300</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2014 441 1.168</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2015 481 1.227</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2016 492 1.225</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2017 510 1.241</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2018 479 1.141</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2019 561 1.319</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">2020 538 1.235</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Last Reviewed: November 14, 2022</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Source: Centers for Disease Control and Prevention , National Center for Emerging and Zoonotic Infectious Diseases (NCEZID) , Division of High-Consequence Pathogens and Pathology (DHCPP)</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-size: 16px; outline: none !important;"><a href="https://www.cdc.gov/prions/cjd/occurrence-transmission.html" rel="nofollow" style="color: #338fe9; outline: none !important;" target="_blank">https://www.cdc.gov/prions/cjd/occurrence-transmission.html</a></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">October 16, 2020 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Human Prion Disease Surveillance in Washington State, 2006-2017 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Liliana Sánchez-González, MD, MPH1,2; Ryan A. Maddox, PhD3; Larissa C. Lewis, RN1; et alJanis E. Blevins, MSM-HC4,5; Elizabeth J. Harker, MPH3,6; Brian S. Appleby, MD4; Marissa K. Person, MSPH3; Lawrence B. Schonberger, MD3; Ermias D. Belay, MD3; Chas DeBolt, RN, MPH1; Kathryn H. Lofy, MD1 Author Affiliations Article Information JAMA Netw Open. 2020;3(10):e2020690. doi:10.1001/jamanetworkopen.2020.20690 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Key Points Question What are the results of human prion disease surveillance in Washington state?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Findings In this cross-sectional study using state surveillance data from 2006 to 2017, 143 human prion disease cases were detected, with an average annual age-adjusted incidence consistent with national reports. The majority of cases (94%) were sporadic, and no cases met criteria for a variant Creutzfeldt-Jakob disease diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Meaning These findings indicate that prion disease surveillance in Washington state is beneficial for monitoring epidemiological trends, facilitating accurate diagnoses, and detecting variant Creutzfeldt-Jakob disease or other emerging human prion diseases should they occur.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract Importance Human prion disease surveillance is critical to detect possible cases of variant Creutzfeldt-Jakob disease and other acquired forms of prion disease in the United States. Results are presented here that describe 12 years of surveillance in Washington, the only US state that has reported the presence of classic bovine spongiform encephalopathy, an animal prion disease that has been shown to transmit to humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Objective To describe the current prion disease surveillance system in Washington and the epidemiological and clinical results of surveillance from 2006 through 2017.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Design, Setting, and Participants This cross-sectional study reports findings from the human prion disease surveillance system in place in Washington state from January 1, 2006, through December 31, 2017. Participants included Washington residents with a clinical suspicion of human prion disease or suggestive test results from the National Prion Disease Pathology Surveillance Center or with prion disease listed as a cause of death on the death certificate. Data for this report were analyzed from June 1, 2016, to July 1, 2020.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Exposure Human prion disease diagnosis.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Main Outcomes and Measures The main outcome was incidence of human prion disease cases, including identification of variant Creutzfeldt-Jakob disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results A total of 143 human prion disease cases were detected during the study period, none of which met criteria for a variant Creutzfeldt-Jakob disease diagnosis. Among 137 definite or probable cases, 123 (89.8%) occurred in persons aged 55 years or older, with a median age at death of 66 years (range, 38-84 years). Most patients were White (124 [92.5%] among 134 with reported race), and slightly over half were male (70 [51.1%]). The average annual age-adjusted prion disease incidence was 1.5 per million population per year, slightly higher than the national rate of 1.2 per million. A total of 99 cases (69.2%) were confirmed by neuropathology. Sporadic prion disease was the most common diagnosis, in 134 cases (93.7%), followed by familial prion disease in 8 cases (5.6%). One iatrogenic prion disease case (0.7%) was also reported.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions and Relevance The findings of this cross-sectional study suggest that demographic characteristics of patients with prion disease in Washington are consistent with national findings. The slightly higher incidence rate may be due to the state’s enhanced surveillance activities, including close collaboration with key partners and educational efforts targeted toward health care providers. Results indicate that surveillance will continue to be beneficial for monitoring epidemiological trends, facilitating accurate diagnoses, and detecting variant Creutzfeldt-Jakob disease or other emerging human prion disease cases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In this cross-sectional study of Washington state’s HPD surveillance system, findings suggest that the demographic characteristics of patients with prion disease between 2006 and 2017 were consistent with national findings. Despite a statewide prion disease surveillance program being in place since 2004, neither vCJD nor another new prion disease has been detected in the state. Given the long incubation periods associated with prion diseases, ongoing vigilance and collaboration with surveillance partners continue to be necessary.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://jamanetwork.com/journals/jamanetworkopen/fullarticle/2771849" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://jamanetwork.com/journals/jamanetworkopen/fullarticle/2771849</a></div></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion Disease, Human Year sCJD Familial CJD Iatrogenic CJD VPSPr GSS Syndrome Total (Definate or Probable)<br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2008 17 0 0 0 0 17</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2009 7 2 0 0 0 9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2010 7 1 0 0 0 8</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2011 9 0 0 0 0 9</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2012 14 1 0 1 0 16</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2013 13 0 1 0 1 15</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2014 11 1 0 0 0 12</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2015 11 1 0 0 0 12</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2016 17 1 0 0 0 18</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 10 0 0 0 0 10</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2018 15 0 0 0 0 15</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">sCJD: Spontaneous CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">GSS: Gerstmann-Straussler-Scheinker disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">VPSPr: Variably protease-sensitive prionopath</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Note: RT-QuIC testing became standard in 2015 and integrated in the case definition in 2018.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doh.wa.gov/sites/default/files/legacy/Documents/5100/420-004-CDAnnualReportIncidenceRates.pdf?uid=64469c43f0ee2" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doh.wa.gov/sites/default/files/legacy/Documents/5100/420-004-CDAnnualReportIncidenceRates.pdf?uid=64469c43f0ee2</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doh.wa.gov/public-health-healthcare-providers/notifiable-conditions/prion-disease" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doh.wa.gov/public-health-healthcare-providers/notifiable-conditions/prion-disease</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined¹ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Updated quarterly. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Last updated on: January 11th, 2023 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Year Total Neuropath Referrals² Prion Disease Sporadic Genetic Iatrogenic vCJD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 1999 & earlier 383 232 202 27 3 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2000 145 102 90 12 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2001 209 118 110 8 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2002 241 144 124 18 2 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2003 259 160 137 21 2 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2004 315 180 163 16 0 1³ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2005 330 179 157 21 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2006 365 179 159 17 1 2⁴ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2007 374 210 191 19 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2008 384 221 205 16 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2009 397 231 210 20 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2010 402 246 218 28 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2011 392 238 214 24 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2012 413 244 221 23 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2013 416 258 223 34 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2014 355 208 185 21 1 1⁵ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2015 401 263 243 20 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2016 395 277 248 29 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2017 375 266 247 19 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2018 308 221 202 18 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2019 433 280 259 21 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2020 366 252 227 24 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2021 343 248 223 22 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2022 307 199 165 13 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> TOTAL 83086 51567 46238 4919 14 4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Year CSF Only and RT-QuIC Positive10 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2015 241 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2016 360 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2017 406 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2018 431 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2019 538 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2020 494 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2021 516 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2022 492 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TOTAL 3478 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1Listed based on the year of death or, if not available, on the year of referral; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2Cases with suspected prion disease for which brain tissue was submitted; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3Disease acquired in the United Kingdom; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">4Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">5Disease possibly acquired in a Middle Eastern or Eastern European country; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">6Includes 25 cases in which the diagnosis is pending (1 from 2020, 2 from 2021 and 21 from 2022), and 20 inconclusive cases; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">7Includes 24 (3 from 2021 and 21 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">8The sporadic cases include 4504 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">9Total does not include 301 Familial cases diagnosed by blood test only. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">10Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">For a downloadable PDF version of our quarterly table, please click the link below: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined ">https://case.edu/medicine/pathology/divisions/national-prion-disease-pathology-surveillance-center/surveillance/tables-cases-examined </a><br /></div><div style="outline: none !important;"><br /></div><div style="outline: none !important;">NPDPSC Table of Cases Examined </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">© 2023 Case Western Reserve University 10900 Euclid Ave. Cleveland, Ohio 44106 216.368.2000 Legal Notice | Privacy Policy PATHOLOGY Campus Location: Wolstein Research Building 5129 2103 Cornell Road Cleveland, OH 44106 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Mailing Address: 10900 Euclid Ave. Cleveland, OH 44106-7288 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Phone: 216.368.3611 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Email: pathology@case.edu </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Tables of Cases Examined | Pathology | School of Medicine | Case Western Reserve University case.edu </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">National Prion Disease Pathology Surveillance Center Cases Examined1 (September 20, 2022) Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 1999 & earlier 383 232 202 27 3 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2000 145 102 90 12 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2001 209 118 110 8 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2002 241 144 124 18 2 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2003 259 160 137 21 2 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2004 315 180 163 16 0 13 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2005 328 179 157 21 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2006 365 179 159 17 1 24 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2007 374 210 191 19 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2008 384 221 205 16 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2009 397 231 210 20 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2010 401 246 218 28 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2011 392 238 214 24 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2012 413 244 221 23 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2013 416 258 223 34 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2014 355 208 185 21 1 15 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2015 401 263 243 20 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2016 395 277 248 29 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2017 375 266 247 19 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2018 308 221 202 18 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2019 434 281 259 22 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2020 365 252 227 24 1 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2021 343 248 223 22 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2022 213 124 98 9 0 0 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> TOTAL 82116 50827 45568 4889 14 4 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Year CSF Only & RT-QuIC Positive10 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2015 140 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2016 183 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2017 227 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2018 266 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2019 311 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2020 310 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2021 341 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2022 262 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> TOTAL 2040 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 1 Listed based on the year of death or, if not available, on year of referral; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 3 Disease acquired in the United Kingdom; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 5 Disease possibly acquired in a Middle Eastern or Eastern European country; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 6 Includes 28 cases in which the diagnosis is pending (1 from 2020, 3 from 2021 and 24 from 2022), and 20 inconclusive cases; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 7 Includes 20 (3 from 2021 and 17 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 8 The sporadic cases include 4437 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> 9 Total does not include 300 Familial cases diagnosed by blood only. 10 Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="font-size: 16px; outline: none !important;">WEDNESDAY, JANUARY 25, 2023 </div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html</a> </div><div style="font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="font-size: 16px; outline: none !important;"> <a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/</a></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;"><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><div dir="ltr" style="outline: none !important;">FRIDAY, APRIL 07, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2023/04/case-report-two-clusters-of-creutzfeldt.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; outline: none !important;"><div style="outline: none !important;">Tuesday APRIL 05, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SUNDAY, MAY 08, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, MAY 24, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, MAY 10, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> FRIDAY, DECEMBER 02, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">SUNDAY, DECEMBER 11, 2022 S</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">EAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007 Singeltary Submission </div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><a href="http://seac992007.blogspot.com/2022/12/seac-spongiform-encephalopathy-advisory.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://seac992007.blogspot.com/2022/12/seac-spongiform-encephalopathy-advisory.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> Friday, DECEMBER 24, 2021 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html</a> </div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USA CJD TSE Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined May 1, 2018</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, JULY 22, 2017</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Why the U.S. Needs to Continue Prion Disease Surveillance, instead of reducing funding to zero</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;">National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2019/03/national-prion-disease-pathology.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">USA CJD TSE Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined May 1, 2018</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2018/07/usa-cjd-tse-tables-of-cases-examined.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, JULY 22, 2017</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Why the U.S. Needs to Continue Prion Disease Surveillance, instead of reducing funding to zero</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://prionunitusaupdate.blogspot.com/2017/07/why-us-needs-to-continue-prion-disease.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div 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outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;">To the Editor: </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;">In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.. </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary, Sr Bacliff, Tex </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;">1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. </div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div style="color: #26282a; font-family: arial; font-size: 16px; outline: none !important;"><a href="http://jama.jamanetwork.com/article.aspx?articleid=1031186" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://jama.jamanetwork.com/article.aspx?articleid=1031186</a> </div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">August 10, 2009</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Greetings,</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">please see history, and the ever evolving TSE science to date ;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Saturday, June 13, 2009</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009</div><div dir="ltr" style="outline: none !important;"><br /></div><div dir="ltr" style="outline: none !important;"><div class="ydp17bdb2bbyiv3543621098" style="outline: none !important;"><div class="ydp17bdb2bbyiv3543621098apple-rich-link" data-url="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" style="display: inline-block; outline: none !important;"><a class="ydp17bdb2bbyiv3543621098lp-rich-link" dir="ltr" href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="border-radius: 10px; color: #338fe9; display: block; font-family: -apple-system, Helvetica, Arial, sans-serif; outline: none 0px !important; width: 300px;" target="_blank"><table border="0" cellpadding="0" cellspacing="0" class="ydp17bdb2bbyiv3543621098lp-rich-link-emailBaseTable" style="background-color: #e9e9eb; border-collapse: collapse; outline: none !important; table-layout: fixed; width: 300px;"><tbody style="outline: none !important;"><tr style="outline: none !important;"><td align="center" style="outline: none !important;"><div class="ydp17bdb2bbimg-preview-wrapper" style="outline: none !important;"><img alt="image.png" class="ydp17bdb2bbyiv3543621098lp-rich-link-mediaImage ydp17bdb2bbpreview" src="https://apis.mail.aol.com/ws/v3/mailboxes/@.id==VjN-ce6z-DxIu_S2g8qZB3zEhBzmZgrr3THL4IwkE1wVplQCkvSg4OstfRBZyImsL89EhqbiNPt5J57JQHOERly2uw/messages/@.id==ALYWtP8I8NlKZBosLwSzIEjwI0k/content/parts/@.id==1.3/thumbnail?appid=AolMailNorrin&downloadWhenThumbnailFails=true&pid=1.3" style="max-width: 320px; outline: none !important; width: 300px;" /><span class="ydp17bdb2bbimg-dl-btn" style="background-attachment: initial; background-clip: initial; background-image: initial; background-origin: initial; background-position: initial; background-repeat: initial; background-size: initial; border-radius: 2px; border: 1px solid rgb(130, 140, 147); outline: none !important;"><button class="ydp17bdb2bbc27KHO0_n ydp17bdb2bbb_0 ydp17bdb2bbM_0 ydp17bdb2bbi_0 ydp17bdb2bbI_T ydp17bdb2bby_Z2hYGcu ydp17bdb2bbA_6EqO ydp17bdb2bbc1AVi73_6FsP ydp17bdb2bbr_P ydp17bdb2bbC_q ydp17bdb2bbcvhIH6_T ydp17bdb2bbP_eo6" data-test-id="icon-btn-download" style="font-family: YahooSans, "Helvetica Neue", "Segoe UI", Helvetica, Arial, "Lucida Grande", sans-serif; outline: none !important;" tabindex="-1" title="Download"><span class="ydp17bdb2bbD_F ydp17bdb2bbab_C ydp17bdb2bbgl_C ydp17bdb2bbW_6D6F" style="outline: none !important; width: 20px;"></span></button></span></div></td></tr><tr style="outline: none !important;"><td style="outline: none !important;"><table bgcolor="#E9E9EB" cellpadding="0" cellspacing="0" class="ydp17bdb2bbyiv3543621098lp-rich-link-captionBar" style="outline: none !important; table-layout: fixed; width: 300px;"><tbody style="outline: none !important;"><tr style="outline: none !important;"><td class="ydp17bdb2bbyiv3543621098lp-rich-link-captionBar-textStackItem" style="outline: none !important; padding: 8px 0px;"><div class="ydp17bdb2bbyiv3543621098lp-rich-link-captionBar-textStack" style="margin: 0px 16px; max-width: 100%; outline: none !important;"><div class="ydp17bdb2bbyiv3543621098lp-rich-link-captionBar-textStack-topCaption-leading" style="font-size: 12px; outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank"><span style="color: black; outline: none !important;">Human Prion Diseases in the United States</span></a></div><div class="ydp17bdb2bbyiv3543621098lp-rich-link-captionBar-textStack-bottomCaption-leading" style="font-size: 11px; outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank"><span style="outline: none !important;">journals.plos.org</span></a></div></div></td></tr></tbody></table></td></tr></tbody></table></a></div></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="font-family: arial; font-size: 16px; outline: none !important;">Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009<br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">August 10, 2009</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Greetings,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">please see history, and the ever evolving TSE science to date ;</div></div><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><a href="https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/04ce2b24-613d-46e6-9802-4131e2bfa6fd</a><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><a href="https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><a href="https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well</a></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">ZOONOSIS, ZOONOTIC, TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PrP UPDATE 2023</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><p style="background-color: #fff3db; color: #29303b; font-family: Georgia, "Times New Roman", sans-serif; line-height: 1.5em; margin: 0px 0px 0.6em; outline: none !important; padding: 0px;"><span style="background-color: white; color: #1d2228; font-family: arial; font-size: 16px; outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</span></p><div style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;">Samia Hannaoui1 · Irina Zemlyankina1 · Sheng Chun Chang1 · Maria Immaculata Arifin1 · Vincent Béringue2 · Debbie McKenzie3 · Hermann M. Schatzl1 · Sabine Gilch1</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Received: 24 May 2022 / Revised: 5 August 2022 / Accepted: 7 August 2022 / Published online: 22 August 2022 © The Author(s) 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model. Inoculation of these mice with deer CWD isolates resulted in atypical clinical manifestation with prion seeding activity and efficient transmissible infectivity in the brain and, remarkably, in feces, but without classical neuropathological or Western blot appearances of prion diseases. Intriguingly, the protease-resistant PrP in the brain resembled that found in a familial human prion disease and was transmissible upon second passage. Our results suggest that CWD might infect humans, although the transmission barrier is likely higher compared to zoonotic transmission of cattle prions. Notably, our data suggest a different clinical presentation, prion signature, and tissue tropism, which causes challenges for detection by current diagnostic assays. Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission. These findings have strong implications for public health and CWD management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerst-mann-Sträussler-Scheinker syndrome (GSS) is defined by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profile and the N-terminal cleavage site.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent pas-sage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable. Here, humanized mice inoculated with CWD deer isolates had an atypical onset of the disease with myoclonus (93.75%), before presenting typical clinical signs, generating prions that presented with either atypical biochemical signature (#321 and #3063), shed in feces (#327), or were undetectable by the classical detection methods. The fact that we could not establish a strong correlation between disease manifestation in 3tg650 mice inoculated with Wisc-1- or 116AG-CWD and the presence of abnormal PrP (Western blot, IHC or RT-QuIC) might be explained by the presence of heterogeneous prions in the brains of infected mice with different seeding properties in vitro. Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43]. We could not establish a correlation between the presence of PrPSc deposits in mouse #328 and the absence of resist-ant PrP in Western blot even though prions present in the brain of this animal seeded the conversion of rPrP sub-strate very well (Fig. 1). Mouse #321 was not available for IHC; therefore, we cannot draw a conclusion about abnormal PrP deposits in the brain of this animal, but we can conclude that there is a correlation between level of prion seeding activity (Fig. 1) and the detection of atypical PrPres fragments (Fig. 2) present in the brain of this animal. We can only speculate that transmission of Wisc-1 to tg650 mice generated prions with characteristics that render them less prone to be unveiled with conventional methods. However, 77.7% of these mice showed prion seeding activity despite the absence of PK-resistant PrPSc for most of them, suggesting that protease-sensitive prions could be involved in these transmissions like it was shown in human prion diseases [54]. Further analyses such as conformation-dependent immunoassay [54] and in vivo sub-passages will be required to resolve these questions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The finding that infectious PrPSc was shed in fecal material of CWD-infected humanized mice and induced clinical disease, different tropism, and typical three banding pattern-PrPres in bank voles that is transmissible upon second passage is highly concerning for public health. The fact that this biochemical signature in bank voles resembles that of the Wisc-1 original deer isolate and is different from that of bvWisc-1, in the migration profile and the glyco-form-ratio, is valid evidence that these results are not a product of contamination in our study. If CWD in humans is found to be contagious and transmissible among humans, as it is in cervids [57], the spread of the dis-ease within humans might become endemic. In contrast to bank voles inoculated with fecal homogenates from mouse #327, so far, we could not detect a PK-resistant PrPSc fragment in the brain homogenates of fecal homogenate-inoculated tg650 mice. The presence of PrPres in these mice will allow us to determine if the molecular signature of hCWD prions from the brain (mouse #321 and #3063) vs feces are the same. Previously, Beringue et al. found that extraneural prions, compared to neural prions, helped more to over-come the species barrier to foreign prions, in addition, different strain types emerged from such serial transmission [11]. Our data also suggest that prions found in the periphery may hold higher zoonotic potential than prions found in neural tissues. In fact, upon second passage, 50% of the tg650 mice inoculated with fecal homogenates from mouse #327 had succumbed with terminal disease compared to only 20% of brain/spinal cord homogenates inoculated-tg650 mice suggesting that hCWD prions found in feces transmit disease more efficiently. Our results also suggest that epidemiological studies [25] may have missed sub-clinical and atypical infections that are/might be transmissible, undetected by the gold standard tests, i.e., Western blot, ELISA, and IHC.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ideally, an oral inoculation of mice with CWD prions would have mimicked best the natural route of exposure to acquired prion diseases like CWD. However, in our case, an oral inoculation would have been impossible considering the lifespan of the rodent models. It took us an extended amount of time, over two years and a half, to have a better idea of the extent of CWD transmission in the tg650 model, even with intracerebral inoculation, usually the fastest way to induce prion disease in a rodent model. Taking this into consideration, our study is the strongest proof-of-principle that CWD might be transmissible to humans. The overall risk for zoonotic transmission of CWD is likely lower than that for BSE; however, rather than predicting the absolute zoonotic risk for CWD, our study indicates the possibility of atypical features in humans. Furthermore, our findings provide striking insights into how CWD might manifest in humans and the impact it may have on human health. We have used Wisc-1/CWD1, one of the most common CWD strains, notably white-tailed deer prions, which have been shown to be more prone to generate human prions in vitro [47]. This implies a high risk of exposure to this strain, e.g., through consumption or handling of infected carcasses, in contrast to rarer CWD strains, and therefore, an actual risk for human health. Fecal shedding of infectious prions, if it occurs in humans, is particularly concerning because of potential human-to-human transmission and adaptation of hCWD. Overall, our findings suggest that CWD surveil-lance in humans should encompass a wider spectrum of tissues/organs tested and include new criteria in the diagnosis of potential patients.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1007/s00401-022-02482-9" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1007/s00401-022-02482-9</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://link.springer.com/epdf/10.1007/s00401-022-02482-9?sharing_token=Vrc9lVnCx6nATJCPtcFbwPe4RwlQNchNByi7wbcMAY4BjPjkpxdzH-iFThODxSPKAK1QgQ-_2ZGPNoYIZyD3IDH8BQbGxEd1NHRwsyD-plz3_gnMpuGB-13C4DkxsBjdAJb3TEqBq0mBLdO-wl3E0flYPQbhh654WM_ijkAKt_U=&fbclid=IwAR0urg8a9eojZqpU6GePUgt5fQxsWqxsxwTzF_E_MktS3jf11wDxZP9Jy10" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://link.springer.com/epdf/10.1007/s00401-022-02482-9?sharing_token=Vrc9lVnCx6nATJCPtcFbwPe4RwlQNchNByi7wbcMAY4BjPjkpxdzH-iFThODxSPKAK1QgQ-_2ZGPNoYIZyD3IDH8BQbGxEd1NHRwsyD-plz3_gnMpuGB-13C4DkxsBjdAJb3TEqBq0mBLdO-wl3E0flYPQbhh654WM_ijkAKt_U=&fbclid=IwAR0urg8a9eojZqpU6GePUgt5fQxsWqxsxwTzF_E_MktS3jf11wDxZP9Jy10</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">SEE A FEW HIGHLIGHTS;</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><span style="background-color: #fcfcfc; color: #333333; font-family: Georgia, Palatino, serif; font-size: 18px; outline: none !important;">''Here, we provide evidence for a zoonotic potential of CWD prions, and its probable signature using mice expressing human prion protein (PrP) as an infection model.''</span><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">''Furthermore, the presence of infectious prions in feces is concerning because if this occurs in humans, it is a source for human-to-human transmission.'' ''These findings have strong implications for public health and CWD management.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''We demonstrate that this transgenic line was susceptible to infection with CWD prions and displayed a distinct leading clinical sign, an atypical PrPSc signature and unusual fecal shedding of infectious prions.'' </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Importantly, these prions generated by the human PrP transgenic mice were transmissible upon passage.'' </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our results are the first evidence of a zoonotic risk of CWD when using one of the most common CWD strains, Wisc-1/CWD1 for ''infection. ''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Our findings strongly suggest that CWD should be regarded as an actual public health risk. Here, we use humanized mice to show that CWD prions can cross the species barrier to humans, and remarkably, infectious prions can be excreted in feces.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43].''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''This implies a high risk of exposure to this strain, e.g., through consumption or handling of infected carcasses, in contrast to rarer CWD strains, and therefore, an actual risk for human health.'' </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Fecal shedding of infectious prions, if it occurs in humans, is particularly concerning because of potential human-to-human transmission and adaptation of hCWD.'' </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Overall, our findings suggest that CWD surveillance in humans should encompass a wider spectrum of tissues/organs tested and include new criteria in the diagnosis of potential patients.</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">***> PLEASE NOTE;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">''Our results indicate that if CWD crosses the species-barrier to humans, it is unlikely to resemble the most common forms of human prion diseases with respect to clinical signs, tissue tropism and PrPSc signature. For instance, PrPSc in variable protease-sensitive prionopathy (VPSPr), a sporadic form of human prion disease, and in the genetic form Gerstmann-Sträussler-Scheinker syndrome (GSS) is defned by an atypical PK-resistant PrPSc fragment that is non-glycosylated and truncated at both C- and N-termini, with a molecular weight between 6 and 8 kDa [24, 44–46]. These biochemical features are unique and distinctive from PrPSc (PrP27-30) found in most other human or animal prion disease. The atypical PrPSc signature detected in brain homogenate of tg650 mice #321 (1st passage) and #3063 (2nd passage), and the 7–8 kDa fragment (Figs. 2, 4) are very similar to that of GSS, both in terms of migration profle and the N-terminal cleavage site.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''CWD in humans might remain subclinical but with PrPSc deposits in the brain with an unusual morphology that does not resemble the patterns usually seen in different prion diseases (e.g., mouse #328; Fig. 3), clinical with untraceable abnormal PrP (e.g., mouse #327) but still transmissible and uncovered upon subsequent passage (e.g., mouse #3063; Fig. 4), or prions have other reservoirs than the usual ones, hence the presence of infectivity in feces (e.g., mouse #327) suggesting a potential for human-to-human transmission and a real iatrogenic risk that might be unrecognizable. Here, humanized mice inoculated with CWD deer isolates had an atypical onset of the disease with myoclonus (93.75%), before presenting typical clinical signs, generating prions that presented with either atypical biochemical signature (#321 and #3063), shed in feces (#327), or were undetectable by the classical detection methods. The fact that we could not establish a strong correlation between disease manifestation in tg650 mice inoculated with Wisc-1- or 116AG-CWD and the presence of abnormal PrP (Western blot, IHC or RTQuIC) might be explained by the presence of heterogeneous prions in the brains of infected mice with diferent seeding properties in vitro. Indeed, such heterogeneity and distinct seeding activities and infectivity of abnormal PrP fragments was observed in VPSPr cases [20, 43].''</div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">VPSPr, GSS, and CWD zoonosis, concerns there from, where did i hear this concern before?</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">wow, just wow, i don't know where to start with Canada and CJD and strange neurological conditions mounting. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">seems the New Brunswick cases just sill not go away, and CJD cases in Canada keep rising.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report Update January 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(PLEASE NOTE, ''The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians.'' HAS BEEN USED 'ad nauseam' YEAR, AFTER YEAR, AFTER YEAR, OF RISKING CJD CASES.)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(PLEASE NOTE, OCTOBER OF 2022 THERE WERE 65 CJD CASES REPORTED, TWO MONTHS LATER, DECEMBER 2022, CJD CASES JUMPED TO 154 CASES REPORTED...tss)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(PLEASE NOTE, STILL, CANADA DOES NOT REPORT VPSPr CJD TSE Prion Cases to the public, see links below for this explanation and discussion. tss)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">THIS problem must be addressed immediately imo.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">QUOTE;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">end</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://vpspr.blogspot.com/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</div></div></div></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;">SNIP...SEE FULL TEXT;</div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">SUNDAY, APRIL 9, 2023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/04/transmission-of-cervid-prions-to.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/04/transmission-of-cervid-prions-to.html</a><br style="outline: none !important;" /></div><div style="outline: none !important;"><br /></div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;">PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada. Grant number: 1R01NS121016-01; 201,600,023</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Eric Cassmanna, Xu Qib, Qingzhong Kongb, and Justin Greenleea</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aNational Animal Disease Center, Agricultural Research Service, US Department of Agriculture, Ames, IA, USA bDepartments of Pathology, Neurology, National Center for Regenerative Medicine, and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer host.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Pooled brain material (GG96) from a CWD positive herd was used to oronasally inoculate two white-tailed deer with wild-type prion protein genotype and intracranially inoculate a raccoon. Brain homogenates (10% w/v) from the raccoon and the two white-tailed deer were used to intracranially inoculate separate groups of transgenic mice that express human prion protein with methionine (M) at codon 129 (Tg40h). Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Humanized transgenic mice inoculated with the raccoon passaged CWD agent from white-tailed deer exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPScwas detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPScalso was detected in brain tissue by western blot and immunohistochemistry. No PrPScwas detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from white-tailed deer did not have detectable PrPScusing conventional immunoassay techniques.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: The host range of the CWD agent from white-tailed deer was expanded in our experimental model after one passage through raccoons.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We thank Quazetta Brown, Lexi Frese, Rylie Frese, Kevin Hassall, Leisa Mandell, and Trudy Tatum for providing excellent technical support to this project.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stable and highly zoonotic cervid prion strain is possible</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, and Qingzhong Kong Department of Pathology, Case Western Reserve University, Cleveland, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in some areas. Multiple in vitro conversion experiments and in vivo animal studies suggest that the CWD-to-human transmission barrier is not unbreakable. A major public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: We inoculated a few sCJD brain samples into cervidized transgenic mice, which were intended as negative controls for bioassays of brain tissues from sCJD cases who had hunted or consumed vension from CWD-endemic states. Some of these mice became infected and their brain tissues were further examined by serial passages in humanized or cervidized mice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a ‘cervidized’ CJD strain that we termed CJDElkPrP. We observed 100% transmission of CJDElkPrPin transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrPtwo more times in the Tg12 mice. We found that such second and third passage CJDElkPrPprions also led to 100% infection in the Tg40h mice. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice, despite that natural elk CWD isolates and CJDElkPrPshare the same elk PrP sequence.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data demonstrate that highly zoonotic cervid prion strains are not only possible but also can be stably maintained in cervids and that CWD zoonosis is prion strain-dependent.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: NIH</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: R01NS052319, R01NS088604, R01NS109532</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O’Rourke for providing the sCJD samples and the CWD samples, respectively.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Adaptation of chronic wasting disease (CWD) prion strains in hosts with different PRNP genotypes</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Camilo Duque Velasqueza,c, Elizabeth Triscotta,c, Chiye Kima,c, Diana Morenoa,c, Judd Aikenb,c, and Debbie McKenziea,c</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aDepartment of Biological Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; bDepartment of Agriculture, Food & Nutritional Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; cCentre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2M8, Canada</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: The contagious nature of CWD epizootics and the PrPCamino acid variation of cervids (and susceptible sympatric species) guarantee the expansion of prion conformational diversity and selective landscapes where new strains can arise. CWD strains can have novel transmission properties including altered host range that may increase zoonotic risk as circulating strains diversify and evolve. We are characterizing the host adaptability of characterized CWD strains as well as CWD isolates from different cervid species in various enzootic regions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Characterized CWD strains as well as a number of isolates from hunter-harvested deer were bioassayed in our rodent panel (transgenic mice expressing cervid alleles G96, S96 and H95-PrPC, elk PrPC, bovine PrPC, and both hamsters and non-transgenic laboratory mice). Strain characteristics were compared using computer based scoring of brain pathology (e.g. PrPCWDbrain distribution), western blot and protein misfolding cyclic amplification (PMCA).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Transmission of various isolates resulted in the selection of strain mixtures in hosts expressing similar PrPC, particularly for polymorphic white-tailed deer and for Norwegian reindeer. As of the second passage, transmission of P153 moose prions from Norway has not resulted in emergence of strains with properties similar to any North American CWD strains in our taxonomic collection (Wisc-1, CWD2, H95+and 116AG).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our data indicates polymorphic white-tailed deer can favor infection with more than one strain. Similar to transmission studies of Colorado CWD isolates from cervids expressing a single PrPCprimary structure, the isolate from Norway reindeer (V214) represents a strain mixture, suggesting intrinsic strain diversity in the Nordfjella epizootic. The diversity of CWD strains with distinct transmission characteristics represents a threat to wildlife, sympatric domestic animals and public health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: Genome Canada and Genome Alberta (Alberta Prion Research Institute and Alberta Agriculture & Forestry); NSERC Grant number: #LSARP 10205; NSERC RGPIN-2017-05539</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We would like to thank Margo Pybus (Alberta Environment and Parks) Trent Bollinger (University of Saskatchewan) for providing us with tissue samples from hunter-harvested deer and Sylvie Benestad for providing moose and reindeer samples.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Application of PMCA to understand CWD prion strains, species barrier and zoonotic potential</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sandra Pritzkowa, Damian Gorskia, Frank Ramireza, Fei Wanga, Glenn C. Tellingb, Justin J. Greenleec, Sylvie L. Benestadd, and Claudio Sotoa aDepartment of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA; bDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA; cVirus and Prion Research Unit, United States Department of Agriculture, Ames, Iowa, USA; dNorwegian Veterinary Institute, OIE Reference Laboratory for CWD, Ås, Norway</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD) is a prion disease affecting various species of cervids that continues to spread uncontrollably across North America and has recently been detected in Scandinavia (Norway, Sweden and Finland). The mechanisms responsible for the natural transmission of CWD are largely unknown. Furthermore, the risk of CWD transmission to other species, including humans, is also unknown and remains a dangerous enigma. In this study, we investigated the potential of CWD prions to infect several other animal species (sheep, cattle, pig, hamster, and mouse) including humans, by examining their capacity to convert the normal prion protein of distinct species in a PMCA reaction. Moreover, we also investigated whether the in vivo passage of CWD through intermediate species alters their capacity for zoonotic transmission, which may represent a major hazard to human health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: For these studies, we used brain material from CWD-infected white-tailed deer (Odocoileus virginianus), elk (Cervus canadensis), and mule deer (Odocoileus hemionus) as species native to North America. We also used CWD-infected Moose (Alces alces), reindeer (Rangifer tarandus) and red deer (Cervus elaphus) as Norwegian cervids. We also used brains from cattle, sheep and pigs experimentally infected by CWD. To study interspecies-transmission and zoonotic potential, samples were tested via PMCA for the conversion of PrPCinto PrPScusing different combinations of inoculum and host species. Based on these analyses we estimated the spillover and zoonotic potential for different CWD isolates. We define and quantify spillover and zoonotic potential indices as the efficiency by which CWD prions sustain prion generation in vitro at the expense of normal prion proteins from various mammals and human, respectively.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show that prions from some cervid species, especially those found in Northern Europe, have a higher potential to transmit disease characteristics to other animals. Conversely, CWD-infected cervids originated in North America appear to have a greater potential to generate human PrPSc. We also found that in vivo transmission of CWD to cattle, but not to sheep or pigs substantially increases the ability of these prions to convert human PrPCby PMCA.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our findings support the existence of different CWD prion strains with distinct spillover and zoonotic potentials. We also conclude that transmission of CWD to other animal species may increase the risk for CWD transmission to humans. Our studies may provide a tool to predict the array of animal species that a given CWD prion could affect and may contribute to understanding the risk of CWD for human health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: National Institute of Health Grant number: P01 AI077774</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Generation of human chronic wasting disease in transgenic mice</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Zerui Wanga, Kefeng Qinb, Manuel V. Camachoa, Ignazio Cali a,c, Jue Yuana, Pingping Shena, Tricia Gillilanda, Syed Zahid Ali Shaha, Maria Gerasimenkoa, Michelle Tanga, Sarada Rajamanickama, Anika Yadatia, Lawrence B. Schonbergerd, Justin Greenleee, Qingzhong Konga,c, James A. Mastriannib, and Wen-Quan Zoua,c</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA; bDepartment of Neurology and Center for Comprehensive Care and Research on Memory Disorders, the University of Chicago Pritzker School of Medicine, Chicago, USA; cNational Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; dDivision of High-Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA, USA; eVirus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, Ames, IA, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD) results from the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC) in the brains of deer and elk. It has been spreading rapidly throughout many regions of North America, exported inadvertently to South Korea, and more recently identified in Europe. Mad cow disease has caused variant Creutzfeldt-Jakob disease (vCJD) in humans and is currently the only known zoonotic prion disease. Whether CWD is transmissible to humans remains uncertain. The aims of our study were not only to confirm whether CWD prion isolates can convert human brain PrPCinto PrPScin vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPScis infectious.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Eight CWD prion isolates from 7 elks and 1 deer were used as the seeds while normal human brain homogenates containing either PrP-129 MM (n = 2) or PrP-129 VV (n = 1) were used as the substrates for sPMCA assay. A normal elk brain tissue sample was used as a negative control seed. Two lines of humanized transgenic (Tg) mice expressing either human PrP-129VV or −129 MM polymorphism were included for transmission studies to determine the infectivity of PMCA-amplified PrPSc. Wester blotting and immunohistochemistry and hematoxylin & eosin staining were used for determining PrPScand neuropathological changes of inoculated animals.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: We report here the generation of the first CWD-derived infectious human PrPScusing elk CWD PrPScto initiate conversion of human PrPCfrom normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPScwas derived from the human brain PrPCsubstrate. Two lines of humanized transgenic mice expressing human PrPCwith either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPScpatterns and neuropathological changes in the brain.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSchas the potential to overcome the species barrier and directly convert human PrPCinto infectious PrPScthat can produce bona fide prion disease when inoculated into humanized transgenic mice.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: CJD Foundation and NIH</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Mortality surveillance of persons potentially exposed to chronic wasting disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">R.A. Maddoxa, R.F. Klosb, L.R. Willb, S.N. Gibbons-Burgenerb, A. Mvilongoa, J.Y. Abramsa, B.S. Applebyc, L.B. Schonbergera, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bWisconsin Department of Health Services (WDHS), Division of Public Health, Madison, USA; cNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: It is unknown whether chronic wasting disease (CWD), a prion disease of cervids, can infect people, but consumption of meat from infected animals would be the most likely route of transmission. Wisconsin Department of Health Services, Division of Public Health (WDHS) personnel maintain a database consisting of information collected from hunters who reported eating, or an intention to eat, venison from CWD-positive cervids. These data, collected since 2003, allow for the evaluation of causes of mortality in individuals potentially exposed to CWD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: The WDHS database contains the name, date of birth, when available, year of CWD-positive deer harvest, and city and state of residence for each potentially exposed individual. The database also includes information on how the deer was processed (self-processed or by a commercial operator) and when applicable, names of others with whom the venison was shared. Duplicate entries (i.e., those who consumed venison from CWD-positive deer in multiple hunt years) are determined by first name, last name, and date of birth. All names in the database are cross-checked with reported cases of human prion disease in Wisconsin and cases in the National Prion Disease Pathology Surveillance Center (NPDPSC) diagnostic testing database. Persons with date of birth available are also cross-checked with prion disease decedents identified through restricted-use national multiple cause-of-death data via a data use agreement with the National Center for Health Statistics (NCHS).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: The database currently consists of 1561 records for hunt years 2003–2017 and 87 additional records for 2018–2019. Of these, 657 records have accompanying date of birth; 15 entries were removed as duplicates leaving 642 unique individuals. Of these individuals, 278 of 426 (66%) who ate venison from a CWD-positive deer and provided processing information reported self-processing. No matches were found among any persons in the database cross-checked with WDHS human prion disease surveillance data, NPDPSC data (February 2022 update), and NCHS data through 2020.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Because of the linkage of person and CWD-positive animal in the WDHS database, reviewing the cause of mortality in potentially exposed persons is possible. The number of individuals cross-checked so far is likely only a small percentage of those potentially exposed to CWD in Wisconsin, and many more years of vital status tracking are needed given an expected long incubation period should transmission to humans occur. Nevertheless, the findings of this ongoing review are thus far reassuring.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion disease incidence, United States, 2003–2020</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">R.A. Maddoxa, M.K. Persona, K. Kotobellib, A. Mvilongoa, B.S. Applebyb, L.B. Schonbergera, T.A. Hammetta, J.Y. Abramsa, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Mortality data, in conjunction with neuropathological and genetic testing results, are used to estimate prion disease incidence in the United States.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Prion disease decedents for 2003–2020 were identified from restricted-use U.S. national multiple cause-of-death data, via a data use agreement with the National Center for Health Statistics, and from the National Prion Disease Pathology Surveillance Center (NPDPSC) database. NPDPSC decedents with neuropathological or genetic test results positive for prion disease for whom no likely match was found in the NCHS multiple cause-of-death data were added as cases for incidence calculations, while those with negative neuropathology results but with cause-of-death data indicating prion disease were removed. Unmatched cases in the NPDPSC database lacking neuropathological testing but with a positive real-time quaking-induced conversion (RT-QuIC) test result were additionally assessed. Age-specific and age-adjusted average annual incidence rates were calculated from the combined data; the year 2000 as the standard population and the direct method were used for age-adjustment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: A total of 7,921 decedents were identified as having prion disease during 2003–2020 for an age-adjusted average annual incidence of 1.2 per million population. The age-adjusted incidence between males and females (1.3 and 1.1 per million, respectively) differed significantly (p < 0.0001). The age-specific average annual incidence among those <55 and ≥55 years of age was 0.2 and 4.8 per million, respectively; incidence among those ≥65 was 6.1 per million. Eighteen cases were <30 years of age for an age-specific incidence of 8.0 per billion; only 6 of these very young cases were sporadic (3 sporadic CJD, 3 sporadic fatal insomnia), with the rest being familial (9), variant (2), or iatrogenic (1). The age-adjusted annual incidence for the most recent year of data, 2020, was 1.3 per million. However, assessment of RT-QuIC positive cases lacking neuropathology in the NPDPSC database suggested that approximately 20% more cases may have occurred in that year; the addition of a subset of these cases that had date of death information available (n = 44) increased the 2020 rate to 1.4 per million.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: Mortality data supplemented with the results of neuropathological, CSF RT-QuIC, and genetic testing can be used to estimate prion disease incidence. However, the identification in the NPDPSC database of RT-QuIC-positive cases lacking date of death information suggests that this strategy may exclude a number of probable prion disease cases. Prion disease cases <30 years of age, especially those lacking a pathogenic mutation, continue to be very rare.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: National Institutes of Health (NIH)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: USDA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Grant number: AP20VSSPRS00C143</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ATYPRION project: assessing the zoonotic potential of interspecies transmission of CWD isolates to livestock (preliminary results).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Enric Vidala,b, Juan Carlos Espinosac, Samanta Gilera,b, Montserrat Ordóñeza,b, Guillermo Canteroa,b, Vincent Béringued, Justin J. Greenleee, and Juan Maria Torresc</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">aUnitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; bIRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; cCentro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid, Spain; dMolecular Virology and Immunology, French National Research Institute for Agriculture, Food and Environment (INRAE), Université Paris-Saclay, Jouy-en-Josas, France; eVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims: Since variant Creutzfeldt-Jackob disease was linked to the consumption of bovine spongiform encephalopathy prions, the study of the pathobiological features of animal prions, particularly their zoonotic potential, is of great concern to the scientific community and public health authorities. Furthermore, interspecies transmission of prions has been demonstrated as a putative evolutionary mechanism for prions, that can lead to the emergence of new features including the ability to infect humans. For instance, small ruminants’ atypical scrapie prions, when propagated in a bovine or porcine host, can shift to a classical BSE phenotype thus posing a potential risk in case of human exposure. So far, no hard evidence of zoonotic transmission of cervids’ chronic wasting disease (CWD) to humans has been published, however experimental transmission to bovine, ovine and caprine hosts has been achieved. Our goal is to investigate if, once passaged through these domestic species, CWD prions might become infectious to humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Material and Methods: Different CWD isolates experimentally adapted to cattle, sheep and goat (Hamir et al, 2005, 2006, 2007, Greenlee et al 2012) have been intracerebrally inoculated to transgenic mouse models expressing the human cellular prion protein either homozygous for methionine or valine at codon 129 (Tg340-Met129 and Tg362-Val129). Additionally, inocula obtained from experimental transmission of elk CWD to ovinized (Tg501) and bovinized (BoTg110) transgenic mice, as well as white-tailed deer CWD to BoTg110 mice, are currently being bioassayed in both human PrPCtransgenic models.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results and conclusions: No evidence of transmission has been found on first passage for bovine adapted elk and mule deer CWD to none of the humanized models. The remaining bioassays are ongoing without showing clinical signs yet, as well as second passages for the negative 1stpassages.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Funded by: La Marató de TV3 foundation. Grant number: ATYPRION (201,821–30-31-32)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion Conference 2018 Abstracts</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P172 Peripheral Neuropathy in Patients with Prion Disease</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Wang H(1), Cohen M(1), Appleby BS(1,2)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P180 Clinico-pathological analysis of human prion diseases in a brain bank series</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Background and objective:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Aims:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Methods:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Results:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">See also poster P103</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WA16 Monitoring Potential CWD Transmission to Humans</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Belay ED</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=====</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Source Prion Conference 2018 Abstracts</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://prionconference.blogspot.com/2018/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://prionconference.blogspot.com/2018/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion 2017 Conference Abstracts </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">This is a progress report of a project which started in 2009. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In four animals wasting was observed, two of those had confirmed diabetes. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">At present, a total of 10 animals are sacrificed and read-outs are ongoing. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><a href="https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf</a><br style="outline: none !important;" /><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SATURDAY, FEBRUARY 23, 2019 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">TUESDAY, NOVEMBER 04, 2014 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission Studies</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip.... </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://jvi.asm.org/content/83/18/9608.full" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://jvi.asm.org/content/83/18/9608.full</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prions in Skeletal Muscles of Deer with Chronic Wasting Disease </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://science.sciencemag.org/content/311/5764/1117..long" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://science.sciencemag.org/content/311/5764/1117..long</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: TSS </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: CWD aka MAD DEER/ELK TO HUMANS ???</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: September 30, 2002 at 7:06 am PST</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: "Belay, Ermias"</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sent: Monday, September 30, 2002 9:22 AM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Sir/Madam,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Ermias Belay, M.D. Centers for Disease Control and Prevention</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">-----Original Message-----</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: Sent: Sunday, September 29, 2002 10:15 AM</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Thursday, April 03, 2008</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip... full text ; </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">sporadic = 54,983 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">spontaneous = 325,650 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ *** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">From: Steve Dealler </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">To: BSE-L@ References: </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Dear Terry,</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Steve Dealler </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Table 9 presents the results of an analysis of these data.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full report ;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE Inquiry Steve Dealler</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Management In Confidence</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">BSE: Private Submission of Bovine Brain Dealler</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...see full text;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">MONDAY, FEBRUARY 25, 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***thus questioning the origin of human sporadic cases. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***thus questioning the origin of human sporadic cases*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">=============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">============== </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2015 CONFERENCE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">PRION 2016 TOKYO</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Saturday, April 23, 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Taylor & Francis</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">WEDNESDAY, MARCH 16, 2022 </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SHEEP BY-PRODUCTS AND WHAT ABOUT Scrapie TSE PrP and Potential Zoonosis? </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SO, WHO'S UP FOR SOME MORE TSE PRION POKER, WHO'S ALL IN $$$ </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">SO, ATYPICAL SCRAPIE ROUGHLY HAS 50 50 CHANCE ATYPICAL SCRAPIE IS CONTAGIOUS, AS NON-CONTAGIOUS, TAKE YOUR PICK, BUT I SAID IT LONG AGO WHEN USDA OIE ET AL MADE ATYPICAL SCRAPIE A LEGAL TRADING COMMODITY, I SAID YOUR PUTTING THE CART BEFORE THE HORSE, AND THAT'S EXACTLY WHAT THEY DID, and it's called in Texas, TEXAS TSE PRION HOLDEM POKER, WHO'S ALL IN $$$</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686</a></div></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Paper</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Cellular prion protein distribution in the vomeronasal organ, parotid, and scent glands of white-tailed deer and mule deer</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Anthony Ness, Aradhana Jacob, Kelsey Saboraki, Alicia Otero, Danielle Gushue, Diana Martinez Moreno, Melanie de Peña, Xinli Tang, Judd Aiken, Susan Lingle & Debbie McKenzie ORCID Icon show less</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Pages 40-57 | Received 03 Feb 2022, Accepted 13 May 2022, Published online: 29 May 2022</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Download citation</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://doi.org/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://doi.org/10.1080/19336896.2022.2079888</a></div></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><div style="outline: none !important;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">PIGS</div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><div style="outline: none !important;"><div style="outline: none !important;">Title: Prion infectivity detected in swine challenged with chronic wasting disease via the intracerebral or oral route</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author item MOORE, S - Orise Fellow item Kunkle, Robert item SMITH, JODI - Iowa State University item WEST-GREENLEE, M - Iowa State University item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 4/4/2016 Publication Date: N/A Citation: N/A</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Interpretive Summary:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of North American cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. In the US, feeding of ruminant by-products to ruminants is prohibited, but feeding of ruminant materials to swine, mink, and poultry still occurs. In addition, scavenging of CWD-affected cervid carcasses by feral pigs presents a potential risk for CWD exposure. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following oral or intracranial experimental challenge. At 8 weeks of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). At death a complete necropsy examination was performed, including testing of tissues for misfolded prion protein (PrPcwd) by western blotting (WB), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry (IHC). None of the pigs developed clinical signs consistent with prion disease. Four >6 month intracranially challenged pigs (survival times 45-73 mpc) were positive by ELISA, two were also positive by WB, and one was positive by IHC. One >6 month orally challenged pig (64 mpc) was positive by ELISA. To further investigate the potential for infectivity, brain tissue from selected pigs was bioassayed in mice expressing porcine PRNP. Tissue from the two WB-positive >6 month intracranially challenged pigs produced positive bioassay results, albeit with low attack rates and variable incubation periods. Interestingly, bioassay of material from the longest surviving >6 month orally challenged pig (72 mpc), which was negative for PrPcwd by all other tests, produced a positive bioassay result. Bioassay of material from additional animals is currently underway. This study demonstrates that pigs can serve as potential hosts for CWD, although with low attack rates and scant PrPcwd accumulation. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Detection of infectivity in orally challenged pigs using mouse bioassay raises the possibility that naturally exposed pigs act as a reservoir of CWD infectivity, even though affected pigs do not develop overt clinical signs or readily detectable PrPcwd.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Interpretive Summary:</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">''These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.''</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">see full report;</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2017 Annual Report</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Objectives</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Objective 1: Investigate the mechanisms of protein misfolding in prion disease, including the genetic determinants of misfolding of the prion protein and the environmental influences on protein misfolding as it relates to prion diseases. Subobjective 1.A: Investigate the differences in the unfolded state of wild-type and disease associated prion proteins to better understand the mechanism of misfolding in genetic prion disease. Subobjective 1.B: Investigate the influence of metal ions on the misfolding of the prion protein in vitro to determine if environmental exposure to metal ions may alter disease progression. Objective 2: Investigate the pathobiology of prion strains in natural hosts, including the influence of prion source genotype on interspecies transmission and the pathobiology of atypical transmissible spongiform encephalopathies (TSEs). Subobjective 2.A: Investigate the pathobiology of atypical TSEs. Subobjective 2.B: Investigate the influence of prion source genotype on interspecies transmission. Objective 3: Investigate sampling methodologies for antemortem detection of prion disease, including the utility of blood sampling as a means to assess prion disease status of affected animals and the utility of environmental sampling for monitoring herd prion disease status. Subobjective 3.A: Investigate the utility of blood sampling as a means to assess prion disease status of affected animals. Subobjective 3.B: Investigate the utility of environmental sampling for monitoring herd prion disease status.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Approach</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">The studies will focus on three animal transmissible spongiform encephalopathy (TSE) agents found in the United States: bovine spongiform encephalopathy (BSE); scrapie of sheep and goats; and chronic wasting disease (CWD) of deer, elk, and moose. The research will address sites of protein folding and misfolding as it relates to prion disease, accumulation of misfolded protein in the host, routes of infection, and ante mortem diagnostics with an emphasis on controlled conditions and natural routes of infection. Techniques used will include spectroscopic monitoring of protein folding/misfolding, clinical exams, histopathology, immunohistochemistry, and biochemical analysis of proteins. The enhanced knowledge gained from this work will help understand the underlying mechanisms of prion disease and mitigate the potential for unrecognized epidemic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Progress Report</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">All 8 project plan milestones for FY17 were fully met. Research efforts directed toward meeting objective 1 of our project plan center around the production of recombinant prion protein from either bacteria or mammalian tissue culture systems and collection of thermodynamic data on the folding of the recombinant prion protein produced. Both bacterial and mammalian expression systems have been established. Thermodynamic data addressing the denatured state of wild-type and a disease associated variant of bovine prion protein has been collected and a manuscript is in preparation. In research pertaining to objective 2, all studies have been initiated and animals are under observation for the development of clinical signs. The animal studies for this objective are long term and will continue until onset of clinical signs. In vitro studies planned in parallel to the animals studies have similarly been initiated and are ongoing. Objective 3 of the project plan focuses on the detection of disease associated prion protein in body fluids and feces collected from a time course study of chronic wasting disease inoculated animals. At this time samples are being collected as planned and methods for analysis are under development.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Accomplishments</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">1. Showed that swine are potential hosts for the scrapie agent. A naturally occurring prion disease has not been recognized in swine, but the agent of bovine spongiform encephalopathy does transmit to swine by experimental routes. Swine are thought to have a robust species barrier when exposed to the naturally occurring prion diseases of other species, but the susceptibility of swine to the agent of sheep scrapie has not been thoroughly tested. ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. Developed a method for amplification and discrimination of the 3 forms of BSE in cattle. The prion protein (PrP) is a protein that is the causative agent of transmissible spongiform encephalopathies (TSEs). The disease process involves conversion of the normal cellular PrP to a pathogenic misfolded conformation. This conversion process can be recreated in the lab using a misfolding amplification process known as real-time quaking induced conversion (RT-QuIC). RT-QuIC allows the detection of minute amounts of the abnormal infectious form of the prion protein by inducing misfolding in a supplied substrate. Although RT-QuIC has been successfully used to detect pathogenic PrP with substrates from a variety of host species, prior to this work bovine prion protein had not been proven for its practical uses for RT-QuIC. We demonstrated that prions from transmissible mink encephalopathy (TME) and BSE-infected cattle can be detected with using bovine prion proteins with RT-QuIC, and developed an RT-QuIC based approach to discriminate different forms of BSE. This rapid and robust method, both to detect and discriminate BSE types, is of importance as the economic implications for different types of BSE vary greatly.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Review Publications</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">snip...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div></div><div style="outline: none !important;"><div style="outline: none !important;">cwd scrapie pigs oral routes </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">CONFIDENTIAL</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">LINE TO TAKE</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: none !important;"><br style="outline: none !important;" /></div><div style="outline: none !important;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div></div></div></div><br style="outline: none !important;" /></div><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">Thursday, April 6, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1. </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html" rel="nofollow" style="color: #338fe9; outline: none 0px !important;" target="_blank">https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html</a></div></div><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">rather a large outbreak of a NEW PRION DISEASE IN CAMELS, and they have not a clue whether it's zoonotic or not...</div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="color: #26282a; outline: none !important;">Monday, November 14, 2022 </div><div dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;">Prion Diseases in Dromedary Camels (CPD) 2022 Review </div><div dir="ltr" style="color: #26282a; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="color: #26282a; outline: none !important;"><a href="https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html</a></div></div></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div data-setdir="false" dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><div style="outline: none !important;"><div dir="ltr" style="outline: none !important;">WEDNESDAY, MARCH 29, 2023 </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;">The use of animal by-products in a circular bioeconomy: Time for a TSE road map 3? </div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html" rel="nofollow" style="color: #196ad4; outline: none !important;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/the-use-of-animal-by-products-in.html</a><br style="outline: none !important;" /></div><div dir="ltr" style="outline: none !important;"><br style="outline: none !important;" /></div></div></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;"><br style="outline: none !important;" /></div><div dir="ltr" style="font-family: arial; font-size: 16px; outline: none !important;">Terry S. Singeltary Sr., Bacliff, Texas USA 77518 flounder9@verizon.net</div></div></div><div data-setdir="false" dir="ltr" style="outline: none !important;"><br /></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-58403391598070621052023-04-07T13:10:00.001-05:002023-04-07T17:33:48.402-05:00Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan<p><span style="background-color: white; font-family: arial; font-size: 16px;">Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan</span></p><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Front Neurol. 2023 Mar 20;14:1134225. doi: 10.3389/fneur.2023.1134225.eCollection 2023.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Case report: Two clusters of Creutzfeldt-Jakob disease cases within 1 year in West Michigan</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ling Ling Rong 1, Nicholas J Lannen 1, Evan C Tank 1, Jessica L Feistel 1, Christopher J Therasse 2, Anvita Potluri 1, Muhib Khan 1, Jiangyong Min 1</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Affiliations</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1Department of Neurosciences, Corewell Health West, Michigan State University, Grand Rapids, MI, United States.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2Department of Radiology, Corewell Health West, Michigan State University, Grand Rapids, MI, United States.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PMID: 37021286 PMCID: PMC10067729 DOI: 10.3389/fneur.2023.1134225</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and uniformly fatal neurodegenerative disease. The reported incidence of CJD is 1 to 2 per million people worldwide annually, with fewer than 1,000 cases in the United States per year. In this study, we report a unique case series on temporo-spatial clusters of CJD cases in West Michigan.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods: A total of five CJD cases consisting of two temporal clusters were seen from July 2021 to June 2022 at Corewell Health West hospitals. All patients had brain MRI, EEG, and CSF tests. Four patients underwent autopsies.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: All patients' MRIs showed characteristic CJD patterns. Four patients had positive CJD panels in CSF. One patient had typical CJD EEG findings. Four patients were confirmed as sporadic CJD by autopsy. All patients died within 3 months after CJD was suspected.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion: All patients lived within a 90-mile radius of Grand Rapids, MI, and two lived in the same county. West Michigan has a population of 1.6 million people, and the four counties where five patients lived have a combined population of 395,104, indicating CJD's new case rate of 3.1 and 12.5 per million people, respectively. Corewell Health is one of the three major healthcare systems in West Michigan. The actual incidence of CJD in West Michigan is likely even higher. This dense temporal and spatial cluster of CJD cases poses a serious public health challenge and warrants urgent investigation.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Keywords: Creutzfeldt-Jacob disease; West Michigan; cluster; prion; rapidly progressive dementia; real-time quaking-induced conversion.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Copyright © 2023 Rong, Lannen, Tank, Feistel, Therasse, Potluri, Khan and Min.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conflict of interest statement</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Introduction</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Creutzfeldt–Jakob disease (CJD) is a transmissible, rapidly progressive neurological disease, caused by misfolded prion protein in the brain (1). The reported prevalence of CJD is 1 to 2 per million people worldwide annually and less than 1,000 cases in the United States per year (2–5)1 CJD subtypes include sporadic, genetic, iatrogenic, and variant CJD with 85–90% of cases being sporadic (2, 6, 7). sCJD can further be divided into subtypes including MM/(MV)1, MM2, VV1, VV2, and MV2 based on disease-related prion protein features and prion protein genotype in the host at the methionine (M) and valine (V) polymorphic codon 129 (8–11)1. The clinical diagnostic criteria for probable sCJD are rapidly progressive dementia plus at least two of the following: myoclonus, visual or cerebellar signs, pyramidal/extrapyramidal signs, and akinetic mutism. Vertigo, headache, and neuropsychiatric symptoms can also present. Patients gradually lose mobility, speech, and progress into a comatose state (2, 6, 7, 12, 13). Despite extensive research since its initial description 100 years ago, CJD remains an incurable disease with a survival of 4–12 months from symptom onset in the vast majority of patients (2, 6, 7).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Over the past few decades, there have been increased reports on sCJD. Some studied regional or national geographical distribution or temporal occurrence, but their cases occurred during periods of 9 to 15 years (14–18). Few case series focused on cases with similar clinical presentation without patients' geographic information (19, 20), or on cases over 5 years in the same region (21).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our case series includes two temporal clusters of CJD cases in one region. Within 1 year from July 2021 to June 2022, we observed five CJD cases at Corewell Health West Butterworth (BW) and Blodgett (BL) Hospitals in Grand Rapids, Michigan (MI). These two hospitals are 3 miles apart. All five cases had supportive brain magnetic resonance imaging (MRI), four of them had supportive cerebrospinal fluid (CSF) findings, and one case had a characteristic electroencephalogram (EEG) pattern. All patients died within 3 months after CJD was suspected. We report this dense temporo-spatial cluster of CJD cases to call for an urgent investigation by public health officials.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Case series</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Patient 1: A 67-year-old white woman who worked as a clinic manager was admitted on 14 July 2021 to BW due to rapid neurological decline. Her initial symptom was severe insomnia starting in mid-January 2021. By February 2021, her symptoms progressed to vertigo, diplopia, and imbalance. By May 2021, she was not able to function at work due to cognitive impairment. Her family noticed intermittent “childlike” behavior. On admission, she was fully alert, awake, and oriented with normal cranial nerves. Montreal Cognitive Assessment (MoCA) testing revealed profound deficits with a corrected score of 17/30.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Patient 2: A 78-year-old white man who was a semi-retired funeral home director was admitted on 31 July 2021 to BW for rapidly progressive cognitive decline along with dysfunctional gait, abnormal speech, and intermittent body jerking. In early May 2021, he started to have intermittent hand weakness, paresthesia, and forgetfulness. Due to unsteadiness, he began using a cane in June 2021 but quickly progressed to a walker. Outpatient electromyography and nerve conduction velocity (EMG/NCV) studies were unrevealing. MRI brain reported a 3-mm subacute infarct in left caudate head and ventriculomegaly. In the same month, he developed expressive aphasia progressing to a paucity of speech. Over 2 weeks, he had a catastrophic decline with excessive daytime somnolence. He was unable to perform activities of daily living and developed alternating urinary incontinence and retention. On admission, he was somnolent but easily startled by auditory stimuli. He followed limited, one-step commands and moved all extremities but was oriented to self only.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Patient 3: A 77-year-old white man who was a semi-retired attorney was transferred to BL for continuous video EEG monitoring on 03 May 2022 from an outside hospital (OSH), which was 75 miles east of Grand Rapids. In mid-March 2022, he complained of “brain fog” after he started medication for his newly diagnosed hypertension. The dyscognia persisted despite discontinuation of the anti-hypertensive. He developed visual disturbance and reported seeing his own fingers abnormally elongated, and his legs were fat and bowed. He was able to provide a full history and had an intact neurological examination on admission at the OSH 6 days before transfer to our facility. Brain MRI at OSH reported subtle cortical restricted diffusion involving both posterior temporoparietal and occipital regions. Levetiracetam was initiated after a 1-h EEG captured intermittent delta slowing over the left frontal region without rhythmicity. CSF at OSH was unremarkable except pending the CJD panel. Upon transfer to BL, he was awake and alert but with limited orientation, verbal output, and impaired abstraction. His motor and sensory examinations were intact.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Patient 4: A 78-year-old white woman and homemaker presented to the local emergency department on 03 June 2022 for cognitive decline over several months, accelerating over a few weeks before presentation. Her brain MRI reported multiple infarcts in different territories concerning global hypoxic ischemia; the on-call tele-stroke physician requested transfer to BW for further evaluation as the MRI pattern suggested CJD rather than hypoxic ischemia. The patient's initial symptom was intermittent forgetfulness, which started in August 2021 and had worsened since late December 2021. In March 2022, she developed “pressure in head” and complained “I do not feel my brain work.” The patient was diagnosed with anxiety and treated with anxiolytics without benefit. In late April 2022, gait abnormality and word-finding difficulties arose, and in May 2022, she developed auditory hallucinations. By June 2022, jerks in her upper extremities were noticed. Upon admission, she was oriented to person only and had impaired attention, reasoning, and verbal expression but had preserved motor strength and sensation.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Patient 5: A 64-year-old white woman who worked as a nurse was admitted to BL on 17 June 2022 for rapidly progressive cognitive decline. Her initial symptoms were “brain fog,” dizziness, and fatigue, starting in January 2022. By late April 2022, she endorsed imbalance, diplopia, and multiple falls along with visual hallucinations, paranoia, and memory dysfunctions. On admission, she was awake, alert, oriented to self and could only recognize her close friends. Her cranial nerves were intact. She had mild proximal weakness in both bilateral upper and lower extremities. The vibratory sensation of bilateral lower extremities was impaired in a length-dependent distribution. She required stabilization on standing (Figure 1).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Laboratory testing and results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All five patients had initial or repeated 1.5 or 3 Tesla brain MRI with and without contrast (w/wo) at BW or BL, respectively, revealing restricted diffusion and corresponding hyperintense T2 FLAIR signal involving bilateral caudate nuclei and putamina in a symmetric pattern (patient 1); asymmetric diffusion restriction signals in the cerebral cortex of cingulum, left temporoparietal lobes, and caudate nucleus (patient 2); prominent multifocal diffusion restriction involving the bi-hemispheric cerebral cortex, more posteriorly and on the left compared to the right (patient 3); symmetric cortical diffusion restriction involving paramedian, lateral parietal cortices, temporal cortices, and to a lesser extent in the frontal lobe with involvement of left greater than the right (patient 4); restricted diffusion in the bilateral caudate nuclei (left > right) and the left mesial temporal lobe, including the amygdala, the hippocampus, and the forniceal column (patient 5) (Figure 2).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EEG was performed on all patients. The EEG of patient 3 showed periodic sharp wave complexes (PSWC), a characteristic CJD pattern. Other patients' EEGs were unremarkable (patient 5), had non-specific rare generalized periodic discharges with triphasic morphology (patients 1 and 4); moderate bitemporal slowing with a right predominance (patient 1); generalized rhythmic delta activity (patient 2) (Figure 3). Blood, CSF basic tests, and Mayo Clinic autoimmune encephalopathy panel in serum (ENS2) were negative. The autoimmune encephalopathy panel and paraneoplastic panel in CSF were all negative. All patients' CSF CJD panels from the National Prion Disease Pathology Surveillance Center (NPDPSC) reported >98% likelihood of prion disease except patient 4 whose CJD likelihood and 14-3-3 proteins were inconclusive, RT-QuIC was negative, and T-tau level was high (19937 pg/ml) (Table 1).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Treatment and final diagnosis</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All patients (except patient 4) received empirical treatment: five doses of 1,000 mg methylprednisolone intravenously daily, followed by five rounds of plasma exchange (patient 1); one dose of 400 mg/kg intravenous immunoglobulin (IVIg) (patient 2); five doses of 400 mg/kg IVIg alone daily (patient 3); or with five doses of IV methylprednisolone daily (patient 5). Patients did not have any benefits from the aforementioned treatment. Patients died in October 2021, August 2021, May 2022, July 2022, and July 2022, respectively. Four patients underwent autopsy and genetic analysis (the family of patient 2 declined autopsy). No gene mutation was detected in any patient. Except for codon 129 polymorphism, no other polymorphism was found in any patient. The final diagnosis was as follows: patients 1 and 5 were sCJD-VV2, and patients 3 and 4 were sCJD-MM1 (Table 1).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All patients in this case series had a rapid cognitive decline. Two patients also had visual disturbances (particularly patient 3 who presented with impaired visual perception at an early stage), and the illness progressed rapidly, possibly representing the Heidenhain variant of CJD (12, 22). On admission, the patients were 67, 78, 77, 78, and 64 years of age, and the durations from the time of symptoms onset to death were 8, 4, 3, 11, and 6 months, respectively. This is mostly consistent with sporadic CJD, which has reported a range of 55–75 years of peak onset age and a median survival duration of 4–12 months (2, 5, 7, 23). The durations from the time when positive CJD in CSF was reported (in patients 1, 2, 3, and 5) or from the time when brain MRI showed typical CJD patterns (patient 4) to death were 72, 10, 2, 26, and 43 days, respectively. All were shorter than the reported typical 4 to 6 months from diagnosis to death (5, 23).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Brain MRI, EEG, and advanced CSF studies are the most utilized diagnostic tests for CJD (7, 24–26). Brain MRI with diffusion-weighted imaging (DWI) has a sensitivity of 67–91% (27, 28) and a specificity of 97% for diagnosing sCJD (27). With increased awareness of sCJD, its diagnostic criteria, and improvement in MRI accessibility and scan quality, the sensitivity of MRI for CJD could reach 99% (28). Our five patients' brain MRI revealed typical sCJD patterns, that is hyperintensities in the cortical gray matter (cortical ribboning sign) and the deep nuclei (basal ganglia and thalamus). The cortical ribboning sign was proposed to be the biomarker in the prodromal phase of sCJD diagnosis (29, 30). The MRI of patient 1 demonstrated a symmetric pattern of bilateral DWI and T2 FLAIR correlated signal in caudate nuclei and putamina. MRIs of patients 2, 4, and 5 revealed asymmetric, cortically based DWI changes in the cingulate, the caudate nuclei, and the left temporoparietal cortex, and the MRI of patient 3 showed multifocal cortically based DWI pattern, more on the left. Park et al. found that being greater than 60 years of age and diffusion restriction in the caudate nucleus and putamen were independent prognostic factors of shorter survival duration in patients with sCJD (27) with median overall survival of 1.7 months compared to 14.2 months in the intermediate risk group. Radiographically, our five patients belong to the high-risk group.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">RT-QuIC is a breakthrough technology for diagnosing CJD with specificity reaching 99–100% (31–33). Its sensitivity can increase from 77 to 96% after modified techniques (31). Patients' (1, 2, 3, and 5) CSF CJD panel reported a likelihood of CJD of more than 98%, positive RT-QuIC, high T-tau protein, and positive 14-3-3 protein (more than 71,000 Au/ml in patients 3 and 5, and no titer reported in patient 1 and 2). The CSF of patient 4 was inconclusive for CJD likelihood and 14-3-3 proteins. Her RT-QuIC was negative, but T-tau protein was 19,937 pg/ml. As per NPDPSC test report, the sensitivity of RT-QuIC is lower when specimens are discolored by blood. Shir et al. reported that elevated CSF 14-3-3 and T-tau proteins as well as clinical symptoms such as myoclonus and visual or cerebellar abnormalities are associated with shorter disease duration (7), which held true for patient 3.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EEG has a lower diagnostic value when compared to brain MRI and CJD panel in CSF. The reported sensitivity of EEG-specific abnormalities to diagnose probable sCJD ranged from 38.2 to 68.75% (34, 35). However, the characteristic EEG finding in CJD, periodic sharp wave complexes (PSWCs), has 86% specificity (36) and 95% positive predictive value (37). Our case series confirmed low sensitivity and high specificity of EEG for diagnosing CJD. Of the five patients, four patients showed EEG abnormality (80%) with 20% specific (patient 3 showed characteristic periodic sharp wave complexes PSWC at 1 Hz, bi-hemispheric with left predominance) and 60% non-specific abnormalities (patients 1, 2, and 5). Mundlamurri et al. reported that in the early stage of sCJD, patients' EEGs can be normal or non-specifically abnormal (35). In very early phases (1.67 months after onset and before the emergence of generalized PSWC) of sCJD, the predominant findings of EEG can be (1) lateralized periodic discharges (LPDs), (2) central sagittal sporadic epileptiform discharges (CSSEDs), and (3) focal epileptiform discharges (38). It is suggested that the early presence of the PSWC pattern has a prognostic value because these patients have significantly lower average survival time (39). The EEG of patient 3 captured PSWC on day 48 after the illness onset. He died 19 days after the EEG was done and 2 days after positive CJD in CSF was reported.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Brain biopsy or autopsy remains the gold standard for final diagnosis. Four patients underwent autopsy and genetic analysis. All four patients had sporadic CJD, of the two most common subtypes, sCJD-VV2 in Patients 1 and 5, and sCJD-MM1 in patients 3 and 4. Different subtypes have different clinical and neuropathological features, as well as survival times and test results (8, 40, 41). The sensitivity of RT-QuIC for detecting MM1 and VV2 is high (96.3%) but can be negative for MM2 and VV1 subtypes (33, 42). Younes et al. reported that MM1, MV1, and VV2 are related to short duration/fast progression, while MV2, VV1, and MM2 are associated with long duration/slow progression (43). Our case series revealed that patients 3 and 4 were sCJD-MM1 but with an 8-month difference in survival length; patients 1 and 5 were diagnosed with sCJD-VV2 with similar survival durations, 8 vs. 6 months. Patient 2 had a short survival duration; unfortunately, his family declined an autopsy.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Geographical clusters of sCJD have been reported, but most clusters contained cases distributed over many years (14, 17, 18, 44–48), and few were temporo-spatial clusters. A French cluster of three cases of CJD occurring in 1998 reported that two of the patients lived in the same village. Molecular and phenotypic analyses showed both patients were homozygous for methionine at the polymorphic codon 129 but one patient was MM1 while another had mixed features of MM1 and MM2 both clinically and histo-pathologically (48). RT-QuIC was not yet invented. A Japanese cluster of three CJD cases occurred between 1988 and 1989 near Fukuoka city; no hospitalization time was mentioned in the report, nor were CSF studies or codon 129 polymorphism analyses done on these patients (49). A cluster of four cases in Burlington, Ontario, Canada, between April 1989 and April 1990 with two additional cases on further inquiry, and a cluster of seven cases in Nassau County, New York, between mid-June 1999 and mid-June 2000 (50, 51) were reported without genetic studies. Some clustering was found later to be an aggregation of genetic CJD cases (52, 53).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our five cases in two clusters were seen within 1 year in Grand Rapids, Michigan. Cluster one included patients 1 and 2, seen within 1 month from July to August of 2021; cluster two included patients 3, 4, and 5, observed within 1 month between May and June of 2022. All patients lived within a 90-mile radius of Grand Rapids. No interpersonal connections were identified among them. All patients were white with differing professions (Table 1). None of them had a family history of Creutzfeldt–Jakob disease, or personal history of corneal transplants, craniotomy, administration of human growth hormone derived from pools of pituitary glands, or surgical procedure at the same facility. However, families of patients 1, 2, and 4 reported consuming venison. More intriguingly, families and relatives of these three patients reported additional (at least four) possible or probable CJD cases occurring between 2007 and 2022 in their friends or communities (unpublished data). One of the patients was a 63-year-old white woman and mayor, who lived 35 miles from patient 2, and died of CJD in March 2022. Thus, such a wave of dense temporo-spatial clustering of CJD in West Michigan is very unusual and alarming.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our case series does not support that CJD incidence has no geographical differences (4, 54). West Michigan has 1.6 million people, and the combined population of four counties where five patients lived is 395,104 in 2022, which makes the CJD new case rate 3.1 and 12.5 per million people in West Michigan and combined four counties, respectively, which is higher than reported 1 to 2 per million people worldwide and 350–710 cases in the United States annually (2–5)1. Adding the cases reported by our three patients' families, the new case occurrence would be even higher. Michigan disease surveillance system (MDSS) reported 19 CJD cases by 31 December 2022 and only 12 cases in 2018, and this reflects a 58% increase2 We do not have enough evidence to conclude that our two clusters are purely due to heightened awareness, more sensitive tests, and better ascertainment, nor could we be certain that they just simultaneously occurred (55). Our study has several limitations, including an observational study, a limited time period, not using the conventionally used solar year period, and a relatively small population and area in West Michigan. As such, this case series highlights only a possible trend. More research and evidence are certainly required to reach a conclusion. We have planned additional retrospective studies, which we expect will surmount these shortcomings. Epidemiological surveillance, research, development of new diagnostic technologies, and public health endeavors are critical (4, 56).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusion</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">For five sCJD cases in two dense clusters within 1 year in Grand Rapids, MI is more than expected. Extensive screening in West Michigan may eventually arrive at a reliable incidence rate of CJD in this region. These two clusters along with additional cases reported by our patients' families warrant urgent investigation. Further research including epidemiological study regarding possible transmission events, common environmental factors that trigger CJD occurrence as well as continuous surveillance, and further improving diagnostic techniques are critical and necessary.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Data availability statement The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ethics statement Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author contributions LR: writing the original draft, selecting MRI images, and finalizing the manuscript. NL, JF, AP, MK, and JM: reviewing and editing. ET: selecting EEG pictures, draft reviewing, and editing. CT: selecting MRI images. All authors contributed to the article and approved the submitted version.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conflict of interest</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Publisher's note</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Footnotes</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://pubmed.ncbi.nlm.nih.gov/37021286/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://pubmed.ncbi.nlm.nih.gov/37021286/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.frontiersin.org/articles/10.3389/fneur.2023.1134225/full" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.frontiersin.org/articles/10.3389/fneur.2023.1134225/full</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="-webkit-text-size-adjust: auto; white-space: pre-wrap;">''However, families of patients 1, 2, and 4 reported consuming venison. More intriguingly, families and relatives of these three patients reported additional (at least four) possible or probable CJD cases occurring between 2007 and 2022 in their friends or communities (unpublished data).''</span></div><div style="outline: currentcolor;"><span style="-webkit-text-size-adjust: auto; white-space: pre-wrap;"><br /></span></div><div style="outline: currentcolor;"><span style="-webkit-text-size-adjust: auto; caret-color: rgb(15, 20, 25); color: #0f1419; font-family: -apple-system, BlinkMacSystemFont, "Segoe UI", Roboto, Helvetica, Arial, sans-serif; font-size: 15px; white-space: pre-wrap;">“We do not have enough evidence to conclude that our two clusters are purely due to heightened awareness, more sensitive tests, and better ascertainment, nor could we be certain that they just simultaneously occurred (55).”</span></div><div style="outline: currentcolor;"><span style="-webkit-text-size-adjust: auto; caret-color: rgb(15, 20, 25); color: #0f1419; font-family: -apple-system, BlinkMacSystemFont, "Segoe UI", Roboto, Helvetica, Arial, sans-serif; font-size: 15px; white-space: pre-wrap;"><br /></span></div><div style="outline: currentcolor;"><br /></div><div dir="ltr" style="outline: currentcolor;">Extremely disturbing to say the least. </div></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">WAS CJD Questionnaires asking real questions for potential routes and sources, were they given out and filled out by Family et al? This is Extremely important, and should happen with all victims of any human TSE Prion, due to the iatrogenic threat i.e. friendly fire, or pass it forward TSE Prion. See history of USA CJD Questionnaire, and why it's so important to be asking all the questions;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">FRIDAY, JANUARY 15, 2021</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CJD TSE Prion Questionnaire USA, UK, and the history there from, have you filled out this questionnaire?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">if not, why not?</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CJD TSE Prion Questionnaire USA, UK, Singeltary</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CJD FOUNDATION Questionnaire</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdfoundation.org/files/pdf/Patient%20Questionnaire%202016.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">UK CJD Questionnaire</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506075100/http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NOT to open up old wounds, but here is my 23 years of endeavors to get the USA to have a CJD Questionnaire for every family of a person whom died of cjd tse prion in the USA in every State, pertaining to real questions of all the potential routes of CJD in that questionnaire. seems i have failed terribly. there was great debate, much anguish, and i did take it personally, when others took credit for what i had been trying to get done. but this was long ago, and today the CJD Foundation seems to be working hard to change there old ways, and seem to be looking to find the routes of sporadic cjd as well. this is just that history, like it or not...kind regards, terry</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">THE MAKING OF THE USA CJD QUESTIONNAIRE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://beta.regulations.gov/document/FDA-2012-D-0307-0035" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://beta.regulations.gov/document/FDA-2012-D-0307-0035</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2012/03/cjd-foundation-cwru-gambetti-familial.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdquestionnaire.blogspot.com/2009/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdquestionnaire.blogspot.com/2009/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://cjdquestionnaire.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://cjdquestionnaire.blogspot.com/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full history of the USA CJD Questionnaire here;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdquestionnaire.blogspot.com/2021/01/cjd-tse-prion-questionnaire-usa-uk-and.html</a> </div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">zoonotic transmission of CWD to Humans has not been _documented_, yet, does not mean it has not already happened. science has shown by transmission studies that CWD transmission to humans is very likely, if it has not happened already. so, what would iatrogenic transmission to humans from CWD look like? Consumption, CWD exposure to humans, and friendly fire there from, what if? </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">let's look at the science shall we.</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">1st, what about CWD TSE Prion in the area of Grand Rapids;</div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">CHRONIC WASTING DISEASE OF CERVID is in Michigan, and it has been documented in this area around Grand Rapids.</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;">''All patients lived within a 90-mile radius of Grand Rapids, MI, and two lived in the same county''</span><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;"><br style="outline: currentcolor;" /></span></div><div dir="ltr" style="outline: currentcolor;"><span style="outline: currentcolor;"><div style="outline: currentcolor;">Chronic wasting disease found in deer at West Michigan farm</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Published: Jan. 14, 2020, 2:32 p.m.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">By Justin P. Hicks | jhicks3@mlive.com</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">NEWAYGO COUNTY, MI -- Three white-tailed deer have tested positive for chronic wasting disease at a deer farm in Newaygo County.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Michigan Department of Agriculture and Rural Development reported Tuesday, Jan. 14 that samples from three 4-year-old deer tested positive for CWD during routine testing under the state’s surveillance program for farmed deer.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Officials said the disease still hasn’t been detected in free-ranging deer in Newaygo County. An investigation will be conducted to rule out exposure of any other farmed deer.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“Chronic wasting disease is a serious disease affecting both farmed and free-ranging deer,” State Veterinarian Nora Wineland said in a prepared statement. “MDARD and the Michigan Department of Natural Resources work together, in partnership with the state’s deer farmers, to ensure the protection of all of Michigan’s deer.”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD is a fatal neurological disease that affects white-tailed deer, mule deer, elk and moose. It can be transmitted directly from animal to animal or indirectly through the environment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Since 2008, the disease has been detected in four other privately-owned deer facilities from Kent, Mecosta and Montcalm counties. Last year, 83 deer in Montcalm County were confirmed to have CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There have been no reported cases of chronic wasting disease infection in humans. As a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend that infected animals not be consumed by humans or domestic animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.mlive.com/news/grand-rapids/2020/01/chronic-wasting-disease-found-in-deer-at-west-michigan-farm.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.mlive.com/news/grand-rapids/2020/01/chronic-wasting-disease-found-in-deer-at-west-michigan-farm.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;">MICHIGAN 25 Michigan deer test positive for CWD in 2021 by: Matt Jaworowski</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Posted: Apr 21, 2022 / 01:01 PM EDT</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Updated: Apr 21, 2022 / 01:01 PM EDT</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SHARE GRAND RAPIDS, Mich. (WOOD) — The latest data from the Michigan Department of Natural Resources shows that 25 deer tested positive for chronic wasting disease in 2021.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The DNR said a little over 7,200 deer were tested last year: a low number for pre-pandemic standards but triple the number of tests run in 2020. In that year, the agency recorded 20 deer with CWD. But the department warns comparing year-to-year data loses a lot of context due to its testing strategy.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“Some years we’ve intensified surveillance in known hot spots, and other years (including 2021) we have looked elsewhere trying to find the disease, specifically avoiding those hot spots,” Chad Stewart, a DNR Deer, Elk and Moose Management Specialist, told News 8. “With that, it’s not really possible to compare the numbers between years at face value.”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Of the 25 CWD-positive deer, three cases of chronic wasting disease were detected in Isabella County, the first cases were found there.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Michigan’s first confirmed case of CWD was found in 2015 and has since been found in Clinton, Dickinson, Eaton, Gratiot, Ingham, Jackson, Kent and Montcalm counties, with Montcalm County, northeast Kent County and southern Jackson County showing the highest concentration of cases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD testing peaked in 2018 and 2019, with a combined 50,000 deer tested and 127 positive tests. Stewart warned that hunters shouldn’t dismiss CWD as a threat despite the lower numbers.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“The distribution of our samples greatly affects the number of positives we expect to find. Intensive collection of samples in known CWD locations like Montcalm and Kent counties would certainly lead to a high number of positives being detected,” Stewart said in a news release. “Our goal this year was to begin to understand what CWD looks like in areas that are historically under-sampled, and we made a lot of strides on that front.”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stewart also warned that, as of now, there’s no easy way to eradicate the disease completely.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">“Once it becomes established, it is unlikely that we can reverse course on the disease. Prevention and early detection remain our best options for CWD management,” Stewart said.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.wlns.com/news/michigan/25-michigan-deer-test-positive-for-cwd-in-2021/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.wlns.com/news/michigan/25-michigan-deer-test-positive-for-cwd-in-2021/</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">SUNDAY, JANUARY 22, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Michigan Chronic Wasting Disease CWD TSE Prion Totals Since 2015 To Present 242 Confirmed Cases </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2023/01/michigan-chronic-wasting-disease-cwd.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/01/michigan-chronic-wasting-disease-cwd.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, FEBRUARY 28, 2023 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Michigan MDARD Captive CWD Positives depopulated and quarantined </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2023/02/michigan-mdard-captive-cwd-positives.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/02/michigan-mdard-captive-cwd-positives.html</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">MICHIGAN CWD Map</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://www.michigan.gov/dnr/managing-resources/wildlife/cwd/hunters/cwd-testing-results-for-deer-harvested-in-2021" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.michigan.gov/dnr/managing-resources/wildlife/cwd/hunters/cwd-testing-results-for-deer-harvested-in-2021</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">THE exposure rate of Cervid CWD exposure via consumption, then going on to have surgical, medical, dental, tissue donations, blood donations, there from, then tracing back CJD TSE Prion victims there from, would be impossible to traceback with todays surveillance traceback efforts, especially when the USDA et al can't even traceback BSE cattle to date (i will spare you those details, but will be in a url link at the bottom.).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></span></div>2nd iatrogenic CJD<br style="outline: currentcolor;" /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">iatrogenic CJD TSE PrP</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">least we forget...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery *** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Friendly fire, pass it forward, they call it iatrogenic cjd, or what i call 'tse prion poker', are you all in $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">all iatrogenic cjd is, is sporadic cjd, before the iatrogenic event is discovered, traced back, proven, documented, put into the academic domain, and then finally the public domain, this very seldom happens, thus problem solved, it's all sporadic cjd...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Friday, March 11, 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prevalence of Surgical Procedures at Symptomatic Onset of Prion Disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Letter Surgery</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">March 9, 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://itseprion.blogspot.com/2022/03/prevalence-of-surgical-procedures-at.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://itseprion.blogspot.com/2022/03/prevalence-of-surgical-procedures-at.html</a><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://itseprion.blogspot.com/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://itseprion.blogspot.com/</a></div></div></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;">3rd CWD Zoonosis Zoonotic Science To Date</div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px; outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">TUESDAY, MAY 11, 2021 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusion</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://thescipub.com/pdf/ajidsp.2021.43.48.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://thescipub.com/pdf/ajidsp.2021.43.48.pdf</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2021/05/a-unique-presentation-of-creutzfeldt.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/05/a-unique-presentation-of-creutzfeldt.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">ABOUT that deer antler spray and CWD TSE PRION...</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease.</div><div style="outline: currentcolor;">just saying...</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Sender: "Patricia Cantos"</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">To: "Terry S Singeltary Sr. (E-mail)"</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Subject: Your submission to the Inquiry</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Date: Fri, 3 Jul 1998 10:10:05 +0100</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">3 July 1998</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Mr Terry S Singeltary Sr.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">E-Mail: Flounder at wt.net</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Ref: E2979</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Dear Mr Singeltary,</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">http://www.bse.org.uk.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it?</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">In the meantime, thank you for you comments. Please do not hesitate to contact me on...</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">snip...end...tss</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year _previously_ and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. _both_ cases confirmed. ...kind regards, terry</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">IPLEX, mad by standard process;</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">also;</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">what about potential mad cow candy bars ?</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">see their potential mad cow candy bar list too...</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">THESE are just a few of MANY of just this ONE COMPANY...TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">''So, in sum, dietary supplements sold in the United States often contain ruminant tissues from undisclosed sources. Personally, I am rather squeamish and I don't think I would be eating prostate or testicle or pituitary, but I am also a little bit wary of consuming products with those glands, not just out of personal repugnance but simply out of a health concern.'' </div></div></div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">DEPARTMENT OF HEALTH AND HUMAN SERVICES</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">Friday, January 19, 2001 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">15<span style="outline: currentcolor; white-space: pre-wrap;"> </span> Open Public Hearing </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">16<span style="outline: currentcolor; white-space: pre-wrap;"> </span>DR. FREAS: We are opening the open public hearing </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17<span style="outline: currentcolor; white-space: pre-wrap;"> </span>now. We have received one response to speak in this </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">18<span style="outline: currentcolor; white-space: pre-wrap;"> </span>afternoon's open public hearing. That is from Dr. Scott </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">19<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Norton. If Dr. Norton is here, would you please come </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">20<span style="outline: currentcolor; white-space: pre-wrap;"> </span>forward. You can either use the podium or the microphone, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">21<span style="outline: currentcolor; white-space: pre-wrap;"> </span>whichever is your choice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">22<span style="outline: currentcolor; white-space: pre-wrap;"> </span>DR. NORTON: I am Scott Norton and I am a </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">23<span style="outline: currentcolor; white-space: pre-wrap;"> </span>physician in the Washington D.C. area. I am here speaking </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">24<span style="outline: currentcolor; white-space: pre-wrap;"> </span>as a private citizen today. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">25<span style="outline: currentcolor; white-space: pre-wrap;"> </span>I first became concerned about the presence of </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span>231 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1<span style="outline: currentcolor; white-space: pre-wrap;"> </span>tissues from ruminant animals in dietary supplements about </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2<span style="outline: currentcolor; white-space: pre-wrap;"> </span>six months ago and expressed my concern in a letter that was </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">3<span style="outline: currentcolor; white-space: pre-wrap;"> </span>published in New England Journal of Medicine in July of Year </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">4<span style="outline: currentcolor; white-space: pre-wrap;"> </span>2000. </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">5<span style="outline: currentcolor; white-space: pre-wrap;"> </span> A couple of the products that I had looked at, and </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">6<span style="outline: currentcolor; white-space: pre-wrap;"> </span>examined their labels, that raised these concerns I brought </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">7<span style="outline: currentcolor; white-space: pre-wrap;"> </span>in right here. I will just read some of the organs that are </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">8<span style="outline: currentcolor; white-space: pre-wrap;"> </span>found in one that is called Male Power. Deer antler, </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">9<span style="outline: currentcolor; white-space: pre-wrap;"> </span>pancreas, orchic--despite what we just heard that the FDA </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">10<span style="outline: currentcolor; white-space: pre-wrap;"> </span>prefers the term "testicular tissue" to be written on the </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">11<span style="outline: currentcolor; white-space: pre-wrap;"> </span>labels, I have never seen a dietary supplement say </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">12<span style="outline: currentcolor; white-space: pre-wrap;"> </span>"testicle." They always say "orchis" or "orchic" which may </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">13<span style="outline: currentcolor; white-space: pre-wrap;"> </span>sound rather flowery to the etymologically impaired--thymus, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">14<span style="outline: currentcolor; white-space: pre-wrap;"> </span>adrenal, heart, lymph node, prostate, spleen and pituitary. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">15<span style="outline: currentcolor; white-space: pre-wrap;"> </span>There are actually seventeen organs in that particular </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">16<span style="outline: currentcolor; white-space: pre-wrap;"> </span>product. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17<span style="outline: currentcolor; white-space: pre-wrap;"> </span> There is another product that is called Brain </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">18<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Nutrition that tells us that it is vitamins and minerals </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">19<span style="outline: currentcolor; white-space: pre-wrap;"> </span>essential for important brain function. It does not mention </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">20<span style="outline: currentcolor; white-space: pre-wrap;"> </span>that there is any glandulars on at least the bold print. </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">21<span style="outline: currentcolor; white-space: pre-wrap;"> </span>But if you look at the small print on the back, we learn </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">22<span style="outline: currentcolor; white-space: pre-wrap;"> </span>that it has brain extract and pituitary extract, raw, in </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">23<span style="outline: currentcolor; white-space: pre-wrap;"> </span>there. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">24<span style="outline: currentcolor; white-space: pre-wrap;"> </span> We know that many of the organs that can be found </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">25<span style="outline: currentcolor; white-space: pre-wrap;"> </span>in the dietary supplements do fall in that list of organs </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span>232 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">1<span style="outline: currentcolor; white-space: pre-wrap;"> </span>that are suspect for contamination with TSEs, the labels, in </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">2<span style="outline: currentcolor; white-space: pre-wrap;"> </span>nearly all cases, identify neither the animal source nor the </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">3<span style="outline: currentcolor; white-space: pre-wrap;"> </span>geographic location from which the organs were derived. I </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">4<span style="outline: currentcolor; white-space: pre-wrap;"> </span>have seen one line that did specify from New Zealand cattle </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">5<span style="outline: currentcolor; white-space: pre-wrap;"> </span>but no other manufacturer will list either the species or </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">6<span style="outline: currentcolor; white-space: pre-wrap;"> </span>the geographic location. </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">7<span style="outline: currentcolor; white-space: pre-wrap;"> </span>The FDA's and the USDA's import alerts that we </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">8<span style="outline: currentcolor; white-space: pre-wrap;"> </span>just learned about prohibit the use of these organs in </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">9<span style="outline: currentcolor; white-space: pre-wrap;"> </span>foods, medicines and medical devices. But my reading of the </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">10<span style="outline: currentcolor; white-space: pre-wrap;"> </span>alert, 17-04, suggests that DSHEA does allow some loopholes </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">11<span style="outline: currentcolor; white-space: pre-wrap;"> </span>for these tissues to possible slip in. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">12<span style="outline: currentcolor; white-space: pre-wrap;"> </span>I will just read from 17-04 that we heard. On the </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">13<span style="outline: currentcolor; white-space: pre-wrap;"> </span>first page, it says that, "This alert does not establish any </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">14<span style="outline: currentcolor; white-space: pre-wrap;"> </span>obligations on regulated entities." I love seeing </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">15<span style="outline: currentcolor; white-space: pre-wrap;"> </span>legislation that starts out with that caveat. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">16<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Then it says, further, "The USDA regulations do </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17<span style="outline: currentcolor; white-space: pre-wrap;"> </span>not apply to bovine-derived materials intended for human </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">18<span style="outline: currentcolor; white-space: pre-wrap;"> </span>consumption as finished dietary supplements." We also learn </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">19<span style="outline: currentcolor; white-space: pre-wrap;"> </span>that the prohibition, or the import alert, is limited to </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">20<span style="outline: currentcolor; white-space: pre-wrap;"> </span>bulk lots of these tissues, completed tissues, from BSE- </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">21<span style="outline: currentcolor; white-space: pre-wrap;"> </span>derived countries. It does not mention if it is not a bulk </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">22<span style="outline: currentcolor; white-space: pre-wrap;"> </span>import or if it is raw materials rather than finished </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">23<span style="outline: currentcolor; white-space: pre-wrap;"> </span>materials. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">24<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Further, we know that it is strongly recommended </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">25<span style="outline: currentcolor; white-space: pre-wrap;"> </span>but not actually prohibited in the language here. So I have </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span>233 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">1<span style="outline: currentcolor; white-space: pre-wrap;"> </span>not taken the assurances from that import alert that Dr. </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">2<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Moore was trying to convey to us. </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">3<span style="outline: currentcolor; white-space: pre-wrap;"> </span>So, in sum, dietary supplements sold in the United </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">4<span style="outline: currentcolor; white-space: pre-wrap;"> </span>States often contain ruminant tissues from undisclosed </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">5<span style="outline: currentcolor; white-space: pre-wrap;"> </span>sources. Personally, I am rather squeamish and I don't </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">6<span style="outline: currentcolor; white-space: pre-wrap;"> </span>think I would be eating prostate or testicle or pituitary, </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">7<span style="outline: currentcolor; white-space: pre-wrap;"> </span>but I am also a little bit wary of consuming products with </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">8<span style="outline: currentcolor; white-space: pre-wrap;"> </span>those glands, not just out of personal repugnance but simply </div><div style="outline: currentcolor;"><span style="outline: currentcolor; white-space: pre-wrap;"> </span></div><div style="outline: currentcolor;">9<span style="outline: currentcolor; white-space: pre-wrap;"> </span>out of a health concern. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">10<span style="outline: currentcolor; white-space: pre-wrap;"> </span>So my question to the advisory committee is this; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">11<span style="outline: currentcolor; white-space: pre-wrap;"> </span>is my caution reasonable and, if it is, should we take </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">12<span style="outline: currentcolor; white-space: pre-wrap;"> </span>further efforts to inform, or even protect, the American </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">13<span style="outline: currentcolor; white-space: pre-wrap;"> </span>public from such exposure. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">14<span style="outline: currentcolor; white-space: pre-wrap;"> </span>I was curious about Dr. Moore's remarks. I sensed </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">15<span style="outline: currentcolor; white-space: pre-wrap;"> </span>two messages. One was the initial reassurance that FDA has </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">16<span style="outline: currentcolor; white-space: pre-wrap;"> </span>the regulatory authority but then I also learned that it is </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17<span style="outline: currentcolor; white-space: pre-wrap;"> </span>the manufacturer's responsibility to provide those </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">18<span style="outline: currentcolor; white-space: pre-wrap;"> </span>assurances, that the FDA doesn't actually inspect. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">19<span style="outline: currentcolor; white-space: pre-wrap;"> </span>I think that the FDA commissioners from Harvey </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">20<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Wylie to David Kessler would say that that track record has </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">21<span style="outline: currentcolor; white-space: pre-wrap;"> </span>proven itself. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">22<span style="outline: currentcolor; white-space: pre-wrap;"> </span>Thank you very much. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">23<span style="outline: currentcolor; white-space: pre-wrap;"> </span>[Applause.] </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">24<span style="outline: currentcolor; white-space: pre-wrap;"> </span>DR. BROWN: Thanks, Dr. Norton. </div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">25<span style="outline: currentcolor; white-space: pre-wrap;"> </span> Committee Discussion </div><div style="outline: currentcolor;"> </div><div dir="ltr" style="outline: currentcolor;">snip...</div></div><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">17 But I think that we could exhibit some quite</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">18 reasonable concern about blood donors who are taking dietary</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">19 supplements that contain a certain amount of unspecified-</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">20 origin brain, brain-related, brain and pituitary material.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">21 If they have done this for more than a sniff or something</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">22 like that, then, perhaps, they should be deferred as blood</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">23 donors.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">24 That is probably worse than spending six months in</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">25 the U.K.</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">1/19/01</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;">3681t2.rtf(845) page 501</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><a href="http://www.fda.gov/ohrms/dockets/ac/cber01.htm" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.fda.gov/ohrms/dockets/ac/cber01.htm</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://web.archive.org/web/20110616141110/http://www.fda.gov/ohrms/dockets/ac/cber01.htm" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20110616141110/http://www.fda.gov/ohrms/dockets/ac/cber01.htm</a><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">see actual paper;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://web.archive.org/web/20110616141110/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2.rtf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20110616141110/http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2.rtf</a><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><a href="http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm</a></div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><a href="http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf</a></div><div style="outline: currentcolor;"> </div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">-------- Original Message --------</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA'' </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: Thu, 01 May 2003 11:23:01 -0500 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: "Terry S. Singeltary Sr." </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: NelliganJ at gao.gov</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The General Accounting Office (GAO) today released the following reports and testimonies:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">REPORTS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1. Dietary Supplements: Review of Health-Related Call Records for Users of Metabolife 356. GAO-03-494, March 31. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.gao.gov/cgi-bin/getrpt?GAO-03-494" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.gao.gov/cgi-bin/getrpt?GAO-03-494</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.gao.gov/highlights/d03494high.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.gao.gov/highlights/d03494high.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">see updated url link;</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="http://web.archive.org/web/20050205084229/http://www.gao.gov/highlights/d03494high.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20050205084229/http://www.gao.gov/highlights/d03494high.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">GREETINGS GAO:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">i was suprised that i did not see any listing of bovine tissue in metabolife on it's label. have they ceased using these desiccated tissues???</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">i see that the lable on this product METABOLIFE 356, does not state that it has any tissues of desiccated bovine organs? i no the product use to, so i am curious if they have ceased the use of the tissues of cattle they _use_ to use (see below)???</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">METABOLIFE 356 BOVINE COMPLEX/GLANDULAR SYSTEM OVARIES, PROSTATE, SCROTUM AND ADRENAL USDA SOURCE CATTLE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">i tried warning them years ago of this potential threat of CJD/TSEs;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: Randy Smith To: "'flounder at wt.net'" Subject: Metabolife Date: Mon, 7 Dec 1998 14:21:35 -0800</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dear Sir,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We are looking at reformulation. I agree that slow virus diseases present a problem in some areas of the world.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our product uses healthy USDA inspected cattle for the glandular extract.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">If you have any links to more information on this subject I would like to examine them.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thank you for your interest and concern,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dr. Smith ============</div></div><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">snip...</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see full text ;</div><div style="outline: currentcolor;"> </div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html</a></div></div><br style="outline: currentcolor;" /></div></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada. Grant number: 1R01NS121016-01; 201,600,023</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Eric Cassmanna, Xu Qib, Qingzhong Kongb, and Justin Greenleea</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aNational Animal Disease Center, Agricultural Research Service, US Department of Agriculture, Ames, IA, USA bDepartments of Pathology, Neurology, National Center for Regenerative Medicine, and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer host.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Pooled brain material (GG96) from a CWD positive herd was used to oronasally inoculate two white-tailed deer with wild-type prion protein genotype and intracranially inoculate a raccoon. Brain homogenates (10% w/v) from the raccoon and the two white-tailed deer were used to intracranially inoculate separate groups of transgenic mice that express human prion protein with methionine (M) at codon 129 (Tg40h). Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Humanized transgenic mice inoculated with the raccoon passaged CWD agent from white-tailed deer exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPScwas detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPScalso was detected in brain tissue by western blot and immunohistochemistry. No PrPScwas detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from white-tailed deer did not have detectable PrPScusing conventional immunoassay techniques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: The host range of the CWD agent from white-tailed deer was expanded in our experimental model after one passage through raccoons.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We thank Quazetta Brown, Lexi Frese, Rylie Frese, Kevin Hassall, Leisa Mandell, and Trudy Tatum for providing excellent technical support to this project.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stable and highly zoonotic cervid prion strain is possible</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, and Qingzhong Kong Department of Pathology, Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in some areas. Multiple in vitro conversion experiments and in vivo animal studies suggest that the CWD-to-human transmission barrier is not unbreakable. A major public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: We inoculated a few sCJD brain samples into cervidized transgenic mice, which were intended as negative controls for bioassays of brain tissues from sCJD cases who had hunted or consumed vension from CWD-endemic states. Some of these mice became infected and their brain tissues were further examined by serial passages in humanized or cervidized mice.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a ‘cervidized’ CJD strain that we termed CJDElkPrP. We observed 100% transmission of CJDElkPrPin transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrPtwo more times in the Tg12 mice. We found that such second and third passage CJDElkPrPprions also led to 100% infection in the Tg40h mice. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice, despite that natural elk CWD isolates and CJDElkPrPshare the same elk PrP sequence.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data demonstrate that highly zoonotic cervid prion strains are not only possible but also can be stably maintained in cervids and that CWD zoonosis is prion strain-dependent.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: NIH</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: R01NS052319, R01NS088604, R01NS109532</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O’Rourke for providing the sCJD samples and the CWD samples, respectively.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Adaptation of chronic wasting disease (CWD) prion strains in hosts with different PRNP genotypes</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Camilo Duque Velasqueza,c, Elizabeth Triscotta,c, Chiye Kima,c, Diana Morenoa,c, Judd Aikenb,c, and Debbie McKenziea,c</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Biological Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; bDepartment of Agriculture, Food & Nutritional Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; cCentre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2M8, Canada</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: The contagious nature of CWD epizootics and the PrPCamino acid variation of cervids (and susceptible sympatric species) guarantee the expansion of prion conformational diversity and selective landscapes where new strains can arise. CWD strains can have novel transmission properties including altered host range that may increase zoonotic risk as circulating strains diversify and evolve. We are characterizing the host adaptability of characterized CWD strains as well as CWD isolates from different cervid species in various enzootic regions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Characterized CWD strains as well as a number of isolates from hunter-harvested deer were bioassayed in our rodent panel (transgenic mice expressing cervid alleles G96, S96 and H95-PrPC, elk PrPC, bovine PrPC, and both hamsters and non-transgenic laboratory mice). Strain characteristics were compared using computer based scoring of brain pathology (e.g. PrPCWDbrain distribution), western blot and protein misfolding cyclic amplification (PMCA).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Transmission of various isolates resulted in the selection of strain mixtures in hosts expressing similar PrPC, particularly for polymorphic white-tailed deer and for Norwegian reindeer. As of the second passage, transmission of P153 moose prions from Norway has not resulted in emergence of strains with properties similar to any North American CWD strains in our taxonomic collection (Wisc-1, CWD2, H95+and 116AG).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our data indicates polymorphic white-tailed deer can favor infection with more than one strain. Similar to transmission studies of Colorado CWD isolates from cervids expressing a single PrPCprimary structure, the isolate from Norway reindeer (V214) represents a strain mixture, suggesting intrinsic strain diversity in the Nordfjella epizootic. The diversity of CWD strains with distinct transmission characteristics represents a threat to wildlife, sympatric domestic animals and public health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: Genome Canada and Genome Alberta (Alberta Prion Research Institute and Alberta Agriculture & Forestry); NSERC Grant number: #LSARP 10205; NSERC RGPIN-2017-05539</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We would like to thank Margo Pybus (Alberta Environment and Parks) Trent Bollinger (University of Saskatchewan) for providing us with tissue samples from hunter-harvested deer and Sylvie Benestad for providing moose and reindeer samples.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Application of PMCA to understand CWD prion strains, species barrier and zoonotic potential</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sandra Pritzkowa, Damian Gorskia, Frank Ramireza, Fei Wanga, Glenn C. Tellingb, Justin J. Greenleec, Sylvie L. Benestadd, and Claudio Sotoa aDepartment of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA; bDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA; cVirus and Prion Research Unit, United States Department of Agriculture, Ames, Iowa, USA; dNorwegian Veterinary Institute, OIE Reference Laboratory for CWD, Ås, Norway</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) is a prion disease affecting various species of cervids that continues to spread uncontrollably across North America and has recently been detected in Scandinavia (Norway, Sweden and Finland). The mechanisms responsible for the natural transmission of CWD are largely unknown. Furthermore, the risk of CWD transmission to other species, including humans, is also unknown and remains a dangerous enigma. In this study, we investigated the potential of CWD prions to infect several other animal species (sheep, cattle, pig, hamster, and mouse) including humans, by examining their capacity to convert the normal prion protein of distinct species in a PMCA reaction. Moreover, we also investigated whether the in vivo passage of CWD through intermediate species alters their capacity for zoonotic transmission, which may represent a major hazard to human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: For these studies, we used brain material from CWD-infected white-tailed deer (Odocoileus virginianus), elk (Cervus canadensis), and mule deer (Odocoileus hemionus) as species native to North America. We also used CWD-infected Moose (Alces alces), reindeer (Rangifer tarandus) and red deer (Cervus elaphus) as Norwegian cervids. We also used brains from cattle, sheep and pigs experimentally infected by CWD. To study interspecies-transmission and zoonotic potential, samples were tested via PMCA for the conversion of PrPCinto PrPScusing different combinations of inoculum and host species. Based on these analyses we estimated the spillover and zoonotic potential for different CWD isolates. We define and quantify spillover and zoonotic potential indices as the efficiency by which CWD prions sustain prion generation in vitro at the expense of normal prion proteins from various mammals and human, respectively.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show that prions from some cervid species, especially those found in Northern Europe, have a higher potential to transmit disease characteristics to other animals. Conversely, CWD-infected cervids originated in North America appear to have a greater potential to generate human PrPSc. We also found that in vivo transmission of CWD to cattle, but not to sheep or pigs substantially increases the ability of these prions to convert human PrPCby PMCA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our findings support the existence of different CWD prion strains with distinct spillover and zoonotic potentials. We also conclude that transmission of CWD to other animal species may increase the risk for CWD transmission to humans. Our studies may provide a tool to predict the array of animal species that a given CWD prion could affect and may contribute to understanding the risk of CWD for human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: National Institute of Health Grant number: P01 AI077774</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Generation of human chronic wasting disease in transgenic mice</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Zerui Wanga, Kefeng Qinb, Manuel V. Camachoa, Ignazio Cali a,c, Jue Yuana, Pingping Shena, Tricia Gillilanda, Syed Zahid Ali Shaha, Maria Gerasimenkoa, Michelle Tanga, Sarada Rajamanickama, Anika Yadatia, Lawrence B. Schonbergerd, Justin Greenleee, Qingzhong Konga,c, James A. Mastriannib, and Wen-Quan Zoua,c</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA; bDepartment of Neurology and Center for Comprehensive Care and Research on Memory Disorders, the University of Chicago Pritzker School of Medicine, Chicago, USA; cNational Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; dDivision of High-Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA, USA; eVirus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, Ames, IA, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) results from the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC) in the brains of deer and elk. It has been spreading rapidly throughout many regions of North America, exported inadvertently to South Korea, and more recently identified in Europe. Mad cow disease has caused variant Creutzfeldt-Jakob disease (vCJD) in humans and is currently the only known zoonotic prion disease. Whether CWD is transmissible to humans remains uncertain. The aims of our study were not only to confirm whether CWD prion isolates can convert human brain PrPCinto PrPScin vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPScis infectious.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Eight CWD prion isolates from 7 elks and 1 deer were used as the seeds while normal human brain homogenates containing either PrP-129 MM (n = 2) or PrP-129 VV (n = 1) were used as the substrates for sPMCA assay. A normal elk brain tissue sample was used as a negative control seed. Two lines of humanized transgenic (Tg) mice expressing either human PrP-129VV or −129 MM polymorphism were included for transmission studies to determine the infectivity of PMCA-amplified PrPSc. Wester blotting and immunohistochemistry and hematoxylin & eosin staining were used for determining PrPScand neuropathological changes of inoculated animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: We report here the generation of the first CWD-derived infectious human PrPScusing elk CWD PrPScto initiate conversion of human PrPCfrom normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPScwas derived from the human brain PrPCsubstrate. Two lines of humanized transgenic mice expressing human PrPCwith either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPScpatterns and neuropathological changes in the brain.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSchas the potential to overcome the species barrier and directly convert human PrPCinto infectious PrPScthat can produce bona fide prion disease when inoculated into humanized transgenic mice.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: CJD Foundation and NIH</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Mortality surveillance of persons potentially exposed to chronic wasting disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">R.A. Maddoxa, R.F. Klosb, L.R. Willb, S.N. Gibbons-Burgenerb, A. Mvilongoa, J.Y. Abramsa, B.S. Applebyc, L.B. Schonbergera, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bWisconsin Department of Health Services (WDHS), Division of Public Health, Madison, USA; cNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: It is unknown whether chronic wasting disease (CWD), a prion disease of cervids, can infect people, but consumption of meat from infected animals would be the most likely route of transmission. Wisconsin Department of Health Services, Division of Public Health (WDHS) personnel maintain a database consisting of information collected from hunters who reported eating, or an intention to eat, venison from CWD-positive cervids. These data, collected since 2003, allow for the evaluation of causes of mortality in individuals potentially exposed to CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: The WDHS database contains the name, date of birth, when available, year of CWD-positive deer harvest, and city and state of residence for each potentially exposed individual. The database also includes information on how the deer was processed (self-processed or by a commercial operator) and when applicable, names of others with whom the venison was shared. Duplicate entries (i.e., those who consumed venison from CWD-positive deer in multiple hunt years) are determined by first name, last name, and date of birth. All names in the database are cross-checked with reported cases of human prion disease in Wisconsin and cases in the National Prion Disease Pathology Surveillance Center (NPDPSC) diagnostic testing database. Persons with date of birth available are also cross-checked with prion disease decedents identified through restricted-use national multiple cause-of-death data via a data use agreement with the National Center for Health Statistics (NCHS).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: The database currently consists of 1561 records for hunt years 2003–2017 and 87 additional records for 2018–2019. Of these, 657 records have accompanying date of birth; 15 entries were removed as duplicates leaving 642 unique individuals. Of these individuals, 278 of 426 (66%) who ate venison from a CWD-positive deer and provided processing information reported self-processing. No matches were found among any persons in the database cross-checked with WDHS human prion disease surveillance data, NPDPSC data (February 2022 update), and NCHS data through 2020.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Because of the linkage of person and CWD-positive animal in the WDHS database, reviewing the cause of mortality in potentially exposed persons is possible. The number of individuals cross-checked so far is likely only a small percentage of those potentially exposed to CWD in Wisconsin, and many more years of vital status tracking are needed given an expected long incubation period should transmission to humans occur. Nevertheless, the findings of this ongoing review are thus far reassuring.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion disease incidence, United States, 2003–2020</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">R.A. Maddoxa, M.K. Persona, K. Kotobellib, A. Mvilongoa, B.S. Applebyb, L.B. Schonbergera, T.A. Hammetta, J.Y. Abramsa, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Mortality data, in conjunction with neuropathological and genetic testing results, are used to estimate prion disease incidence in the United States.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Prion disease decedents for 2003–2020 were identified from restricted-use U.S. national multiple cause-of-death data, via a data use agreement with the National Center for Health Statistics, and from the National Prion Disease Pathology Surveillance Center (NPDPSC) database. NPDPSC decedents with neuropathological or genetic test results positive for prion disease for whom no likely match was found in the NCHS multiple cause-of-death data were added as cases for incidence calculations, while those with negative neuropathology results but with cause-of-death data indicating prion disease were removed. Unmatched cases in the NPDPSC database lacking neuropathological testing but with a positive real-time quaking-induced conversion (RT-QuIC) test result were additionally assessed. Age-specific and age-adjusted average annual incidence rates were calculated from the combined data; the year 2000 as the standard population and the direct method were used for age-adjustment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: A total of 7,921 decedents were identified as having prion disease during 2003–2020 for an age-adjusted average annual incidence of 1.2 per million population. The age-adjusted incidence between males and females (1.3 and 1.1 per million, respectively) differed significantly (p < 0.0001). The age-specific average annual incidence among those <55 and ≥55 years of age was 0.2 and 4.8 per million, respectively; incidence among those ≥65 was 6.1 per million. Eighteen cases were <30 years of age for an age-specific incidence of 8.0 per billion; only 6 of these very young cases were sporadic (3 sporadic CJD, 3 sporadic fatal insomnia), with the rest being familial (9), variant (2), or iatrogenic (1). The age-adjusted annual incidence for the most recent year of data, 2020, was 1.3 per million. However, assessment of RT-QuIC positive cases lacking neuropathology in the NPDPSC database suggested that approximately 20% more cases may have occurred in that year; the addition of a subset of these cases that had date of death information available (n = 44) increased the 2020 rate to 1.4 per million.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: Mortality data supplemented with the results of neuropathological, CSF RT-QuIC, and genetic testing can be used to estimate prion disease incidence. However, the identification in the NPDPSC database of RT-QuIC-positive cases lacking date of death information suggests that this strategy may exclude a number of probable prion disease cases. Prion disease cases <30 years of age, especially those lacking a pathogenic mutation, continue to be very rare.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: National Institutes of Health (NIH)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: USDA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Grant number: AP20VSSPRS00C143</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ATYPRION project: assessing the zoonotic potential of interspecies transmission of CWD isolates to livestock (preliminary results).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Enric Vidala,b, Juan Carlos Espinosac, Samanta Gilera,b, Montserrat Ordóñeza,b, Guillermo Canteroa,b, Vincent Béringued, Justin J. Greenleee, and Juan Maria Torresc</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">aUnitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; bIRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; cCentro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid, Spain; dMolecular Virology and Immunology, French National Research Institute for Agriculture, Food and Environment (INRAE), Université Paris-Saclay, Jouy-en-Josas, France; eVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims: Since variant Creutzfeldt-Jackob disease was linked to the consumption of bovine spongiform encephalopathy prions, the study of the pathobiological features of animal prions, particularly their zoonotic potential, is of great concern to the scientific community and public health authorities. Furthermore, interspecies transmission of prions has been demonstrated as a putative evolutionary mechanism for prions, that can lead to the emergence of new features including the ability to infect humans. For instance, small ruminants’ atypical scrapie prions, when propagated in a bovine or porcine host, can shift to a classical BSE phenotype thus posing a potential risk in case of human exposure. So far, no hard evidence of zoonotic transmission of cervids’ chronic wasting disease (CWD) to humans has been published, however experimental transmission to bovine, ovine and caprine hosts has been achieved. Our goal is to investigate if, once passaged through these domestic species, CWD prions might become infectious to humans.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Material and Methods: Different CWD isolates experimentally adapted to cattle, sheep and goat (Hamir et al, 2005, 2006, 2007, Greenlee et al 2012) have been intracerebrally inoculated to transgenic mouse models expressing the human cellular prion protein either homozygous for methionine or valine at codon 129 (Tg340-Met129 and Tg362-Val129). Additionally, inocula obtained from experimental transmission of elk CWD to ovinized (Tg501) and bovinized (BoTg110) transgenic mice, as well as white-tailed deer CWD to BoTg110 mice, are currently being bioassayed in both human PrPCtransgenic models.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results and conclusions: No evidence of transmission has been found on first passage for bovine adapted elk and mule deer CWD to none of the humanized models. The remaining bioassays are ongoing without showing clinical signs yet, as well as second passages for the negative 1stpassages.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Funded by: La Marató de TV3 foundation. Grant number: ATYPRION (201,821–30-31-32)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion Conference 2018 Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P172 Peripheral Neuropathy in Patients with Prion Disease</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Wang H(1), Cohen M(1), Appleby BS(1,2)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P180 Clinico-pathological analysis of human prion diseases in a brain bank series</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Background and objective:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Aims:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Methods:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Results:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">See also poster P103</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WA16 Monitoring Potential CWD Transmission to Humans</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Belay ED</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=====</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Source Prion Conference 2018 Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://prionconference.blogspot.com/2018/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://prionconference.blogspot.com/2018/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion 2017 Conference Abstracts </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">This is a progress report of a project which started in 2009. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In four animals wasting was observed, two of those had confirmed diabetes. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">At present, a total of 10 animals are sacrificed and read-outs are ongoing. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><a href="https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://cjdfoundation.org/files/pdf/CWD%20study%20oral%20transmission%20of%20CWD%20to%20primates.pdf</a><br style="outline: currentcolor;" /><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SATURDAY, FEBRUARY 23, 2019 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">TUESDAY, NOVEMBER 04, 2014 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission Studies</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip.... </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://jvi.asm.org/content/83/18/9608.full" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://jvi.asm.org/content/83/18/9608.full</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prions in Skeletal Muscles of Deer with Chronic Wasting Disease </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://science.sciencemag.org/content/311/5764/1117..long" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://science.sciencemag.org/content/311/5764/1117..long</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: TSS </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: CWD aka MAD DEER/ELK TO HUMANS ???</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: September 30, 2002 at 7:06 am PST</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: "Belay, Ermias"</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sent: Monday, September 30, 2002 9:22 AM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dear Sir/Madam,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Ermias Belay, M.D. Centers for Disease Control and Prevention</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">-----Original Message-----</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: Sent: Sunday, September 29, 2002 10:15 AM</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Thursday, April 03, 2008</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip... full text ; </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">sporadic = 54,983 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">spontaneous = 325,650 hits <a href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">> However, to date, no CWD infections have been reported in people. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ *** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">From: Steve Dealler </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">To: BSE-L@ References: </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Dear Terry,</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Steve Dealler </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Table 9 presents the results of an analysis of these data.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full report ;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE Inquiry Steve Dealler</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Management In Confidence</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">BSE: Private Submission of Bovine Brain Dealler</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...see full text;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">MONDAY, FEBRUARY 25, 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.nature.com/articles/srep11573</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***thus questioning the origin of human sporadic cases. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***thus questioning the origin of human sporadic cases*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">=============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">============== </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2015 CONFERENCE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">PRION 2016 TOKYO</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Saturday, April 23, 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Taylor & Francis</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">WEDNESDAY, MARCH 16, 2022 </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SHEEP BY-PRODUCTS AND WHAT ABOUT Scrapie TSE PrP and Potential Zoonosis? </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SO, WHO'S UP FOR SOME MORE TSE PRION POKER, WHO'S ALL IN $$$ </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">SO, ATYPICAL SCRAPIE ROUGHLY HAS 50 50 CHANCE ATYPICAL SCRAPIE IS CONTAGIOUS, AS NON-CONTAGIOUS, TAKE YOUR PICK, BUT I SAID IT LONG AGO WHEN USDA OIE ET AL MADE ATYPICAL SCRAPIE A LEGAL TRADING COMMODITY, I SAID YOUR PUTTING THE CART BEFORE THE HORSE, AND THAT'S EXACTLY WHAT THEY DID, and it's called in Texas, TEXAS TSE PRION HOLDEM POKER, WHO'S ALL IN $$$</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686</a></div></div></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Paper</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Cellular prion protein distribution in the vomeronasal organ, parotid, and scent glands of white-tailed deer and mule deer</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Anthony Ness, Aradhana Jacob, Kelsey Saboraki, Alicia Otero, Danielle Gushue, Diana Martinez Moreno, Melanie de Peña, Xinli Tang, Judd Aiken, Susan Lingle & Debbie McKenzie ORCID Icon show less</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Pages 40-57 | Received 03 Feb 2022, Accepted 13 May 2022, Published online: 29 May 2022</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Download citation</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://doi.org/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://doi.org/10.1080/19336896.2022.2079888</a></div></div></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><div style="outline: currentcolor;"><a href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2079888</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">PIGS</div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><div style="outline: currentcolor;"><div style="outline: currentcolor;">Title: Prion infectivity detected in swine challenged with chronic wasting disease via the intracerebral or oral route</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item MOORE, S - Orise Fellow item Kunkle, Robert item SMITH, JODI - Iowa State University item WEST-GREENLEE, M - Iowa State University item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 4/4/2016 Publication Date: N/A Citation: N/A</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of North American cervids. The potential for swine to serve as a host for the agent of chronic wasting disease is unknown. In the US, feeding of ruminant by-products to ruminants is prohibited, but feeding of ruminant materials to swine, mink, and poultry still occurs. In addition, scavenging of CWD-affected cervid carcasses by feral pigs presents a potential risk for CWD exposure. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following oral or intracranial experimental challenge. At 8 weeks of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 month challenge groups). The remaining pigs (>6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). At death a complete necropsy examination was performed, including testing of tissues for misfolded prion protein (PrPcwd) by western blotting (WB), enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry (IHC). None of the pigs developed clinical signs consistent with prion disease. Four >6 month intracranially challenged pigs (survival times 45-73 mpc) were positive by ELISA, two were also positive by WB, and one was positive by IHC. One >6 month orally challenged pig (64 mpc) was positive by ELISA. To further investigate the potential for infectivity, brain tissue from selected pigs was bioassayed in mice expressing porcine PRNP. Tissue from the two WB-positive >6 month intracranially challenged pigs produced positive bioassay results, albeit with low attack rates and variable incubation periods. Interestingly, bioassay of material from the longest surviving >6 month orally challenged pig (72 mpc), which was negative for PrPcwd by all other tests, produced a positive bioassay result. Bioassay of material from additional animals is currently underway. This study demonstrates that pigs can serve as potential hosts for CWD, although with low attack rates and scant PrPcwd accumulation. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Detection of infectivity in orally challenged pigs using mouse bioassay raises the possibility that naturally exposed pigs act as a reservoir of CWD infectivity, even though affected pigs do not develop overt clinical signs or readily detectable PrPcwd.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Interpretive Summary:</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">''These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.''</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">see full report;</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2017 Annual Report</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Objectives</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Objective 1: Investigate the mechanisms of protein misfolding in prion disease, including the genetic determinants of misfolding of the prion protein and the environmental influences on protein misfolding as it relates to prion diseases. Subobjective 1.A: Investigate the differences in the unfolded state of wild-type and disease associated prion proteins to better understand the mechanism of misfolding in genetic prion disease. Subobjective 1.B: Investigate the influence of metal ions on the misfolding of the prion protein in vitro to determine if environmental exposure to metal ions may alter disease progression. Objective 2: Investigate the pathobiology of prion strains in natural hosts, including the influence of prion source genotype on interspecies transmission and the pathobiology of atypical transmissible spongiform encephalopathies (TSEs). Subobjective 2.A: Investigate the pathobiology of atypical TSEs. Subobjective 2.B: Investigate the influence of prion source genotype on interspecies transmission. Objective 3: Investigate sampling methodologies for antemortem detection of prion disease, including the utility of blood sampling as a means to assess prion disease status of affected animals and the utility of environmental sampling for monitoring herd prion disease status. Subobjective 3.A: Investigate the utility of blood sampling as a means to assess prion disease status of affected animals. Subobjective 3.B: Investigate the utility of environmental sampling for monitoring herd prion disease status.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Approach</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">The studies will focus on three animal transmissible spongiform encephalopathy (TSE) agents found in the United States: bovine spongiform encephalopathy (BSE); scrapie of sheep and goats; and chronic wasting disease (CWD) of deer, elk, and moose. The research will address sites of protein folding and misfolding as it relates to prion disease, accumulation of misfolded protein in the host, routes of infection, and ante mortem diagnostics with an emphasis on controlled conditions and natural routes of infection. Techniques used will include spectroscopic monitoring of protein folding/misfolding, clinical exams, histopathology, immunohistochemistry, and biochemical analysis of proteins. The enhanced knowledge gained from this work will help understand the underlying mechanisms of prion disease and mitigate the potential for unrecognized epidemic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Progress Report</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">All 8 project plan milestones for FY17 were fully met. Research efforts directed toward meeting objective 1 of our project plan center around the production of recombinant prion protein from either bacteria or mammalian tissue culture systems and collection of thermodynamic data on the folding of the recombinant prion protein produced. Both bacterial and mammalian expression systems have been established. Thermodynamic data addressing the denatured state of wild-type and a disease associated variant of bovine prion protein has been collected and a manuscript is in preparation. In research pertaining to objective 2, all studies have been initiated and animals are under observation for the development of clinical signs. The animal studies for this objective are long term and will continue until onset of clinical signs. In vitro studies planned in parallel to the animals studies have similarly been initiated and are ongoing. Objective 3 of the project plan focuses on the detection of disease associated prion protein in body fluids and feces collected from a time course study of chronic wasting disease inoculated animals. At this time samples are being collected as planned and methods for analysis are under development.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Accomplishments</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">1. Showed that swine are potential hosts for the scrapie agent. A naturally occurring prion disease has not been recognized in swine, but the agent of bovine spongiform encephalopathy does transmit to swine by experimental routes. Swine are thought to have a robust species barrier when exposed to the naturally occurring prion diseases of other species, but the susceptibility of swine to the agent of sheep scrapie has not been thoroughly tested. ARS researchers at Ames, Iowa conducted this experiment to test the susceptibility of swine to U.S. scrapie isolates by intracranial and oral inoculation. Necropsies were done on a subset of animals at approximately 6 months post inoculation (PI): the time the pigs were expected to reach market weight. Remaining pigs were maintained and monitored for clinical signs of transmissible spongiform encephalopathies (TSE) until study termination at 80 months PI or when removed due to intercurrent disease. Brain samples were examined by multiple diagnostic approaches, and for a subset of pigs in each inoculation group, bioassay in mice expressing porcine prion protein. At 6 months PI, no evidence of scrapie infection was noted by any diagnostic method. However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">2. Determined that pigs naturally exposed to chronic wasting disease (CWD) may act as a reservoir of CWD infectivity. Chronic wasting disease is a naturally occurring, fatal, neurodegenerative disease of cervids. The potential for swine to serve as a host for the agent of CWD disease is unknown. The purpose of this study was to investigate the susceptibility of swine to the CWD agent following experimental oral or intracranial inoculation. Pigs were assigned to 1 of 3 groups: intracranially inoculated; orally inoculated; or non-inoculated. At market weight age, half of the pigs in each group were tested ('market weight' groups). The remaining pigs ('aged' groups) were allowed to incubate for up to 73 months post inoculation (MPI). Tissues collected at necropsy were examined for disease-associated prion protein (PrPSc) by multiple diagnostic methods. Brain samples from selected pigs were bioassayed in mice expressing porcine prion protein. Some pigs from each inoculated group were positive by one or more tests. Bioassay was positive in 4 out of 5 pigs assayed. Although only small amounts of PrPSc were detected using sensitive methods, this study demonstrates that pigs can serve as hosts for CWD. Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. Developed a method for amplification and discrimination of the 3 forms of BSE in cattle. The prion protein (PrP) is a protein that is the causative agent of transmissible spongiform encephalopathies (TSEs). The disease process involves conversion of the normal cellular PrP to a pathogenic misfolded conformation. This conversion process can be recreated in the lab using a misfolding amplification process known as real-time quaking induced conversion (RT-QuIC). RT-QuIC allows the detection of minute amounts of the abnormal infectious form of the prion protein by inducing misfolding in a supplied substrate. Although RT-QuIC has been successfully used to detect pathogenic PrP with substrates from a variety of host species, prior to this work bovine prion protein had not been proven for its practical uses for RT-QuIC. We demonstrated that prions from transmissible mink encephalopathy (TME) and BSE-infected cattle can be detected with using bovine prion proteins with RT-QuIC, and developed an RT-QuIC based approach to discriminate different forms of BSE. This rapid and robust method, both to detect and discriminate BSE types, is of importance as the economic implications for different types of BSE vary greatly.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Review Publications</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">snip...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div></div><div style="outline: currentcolor;"><div style="outline: currentcolor;">cwd scrapie pigs oral routes </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">CONFIDENTIAL</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">LINE TO TAKE</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- </div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"> "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">DO Hagger RM 1533 MT Ext 3201</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf</a></div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;">Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....</div><div style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><a href="http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf" rel="nofollow" style="color: #196ad4; outline: currentcolor;" target="_blank">http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf</a></div></div></div></div><br style="outline: currentcolor;" /></div><div style="outline: currentcolor;"><div dir="ltr" style="outline: currentcolor;">Thursday, April 6, 2023 </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;">WOAH OIE CHAPTER 11.4 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 11.4.1. </div><div dir="ltr" style="outline: currentcolor;"><br style="outline: currentcolor;" /></div><div dir="ltr" style="outline: currentcolor;"><a href="https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html" rel="nofollow" style="color: #338fe9; outline: 0px;" target="_blank">https://woahoie.blogspot.com/2023/04/woah-oie-chapter-114-bovine-spongiform.html</a></div></div><br style="outline: currentcolor;" /></div><div data-setdir="false" dir="ltr" style="outline: currentcolor;">Terry S. Singeltary Sr., Bacliff, Texas USA 77518 flounder9@verizon.net</div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-49667067403321616932023-02-08T21:32:00.002-06:002023-02-08T21:34:49.278-06:00Creutzfeldt–Jakob Disease Misdiagnosed as Multiple System Atrophy<div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Movement Disorders Clinical Practice</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Early View </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">CLINICO-PATHOLOGICAL CASE </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Creutzfeldt–Jakob Disease Misdiagnosed as Multiple System Atrophy </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Nicholas B. Martin BS, Shunsuke Koga MD, PhD, Brian S. Appleby MD, Hiroaki Sekiya MD, PhD, DennisW. Dickson MD First published: 06 January 2023 </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://doi.org/10.1002/mdc3.13654">https://doi.org/10.1002/mdc3.13654</a><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Relevant disclosures and conflict of interest are listed at the end of this article.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Abstract </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Background</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by various combinations of autonomic failure, parkinsonism, and cerebellar syndromes. Although consensus criteria have been widely used to diagnose MSA, accurate clinical diagnosis remains challenging. Other neurodegenerative disorders, such as Lewy body disease, can mimic MSA.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Objectives</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">We described clinical and neuropathologic findings of two patients with Creutzfeldt–Jakob disease (CJD) who had antemortem clinical diagnoses of MSA.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Methods</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">The brain bank for neurodegenerative disorders was queried for cases with a clinical diagnosis of MSA, but neuropathologic findings of CJD.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Results</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Case 1 was a 55-year-old man with a 6-month history of orthostatic hypotension, parkinsonism, cerebellar ataxia, bradyphrenia, and memory impairment. Case 2 was a 65-year-old man who had a 5-year history of cerebellar ataxia, parkinsonism, and cognitive impairment, as well as a 7-year history of dream enactment behavior. Neither case had characteristic α-synuclein immunoreactive neuronal or glial inclusions typical of MSA. Instead, they had spongiform encephalopathy with neuronal loss and gliosis with prion protein-immunoreactive kuru-like plaques. Genetic analyses in case 1 had wild-type PRNP, whereas case 2 revealed a 4-octapeptide repeat insertion in PRNP.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Conclusions</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Even when clinical features suggest MSA, CJD should also be considered if the progression is rapid or the disease course is atypical, such as the absence of autonomic dysfunction for an extended period.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mdc3.13654">https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mdc3.13654</a><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">***> Creutzfeldt–Jakob Disease Misdiagnosed as Multiple System Atrophy</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">To the Editor: </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.. </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Terry S. Singeltary, Sr Bacliff, Tex </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:<span dir="ltr">2322-2323</span>. </div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="http://jama.jamanetwork.com/article.aspx?articleid=1031186">http://jama.jamanetwork.com/article.aspx?articleid=1031186</a><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Elsevier Editorial System(tm) for The Lancet Infectious Diseases</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Manuscript Draft</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Manuscript Number:</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Article Type: Personal View</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Corresponding Author: Mr. Terry S. Singeltary,</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Corresponding Author's Institution: na</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">First Author: Terry S Singeltary, none</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Order of Authors: Terry S Singeltary, none; Terry S. Singeltary</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory August 2007</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">August 2007</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007. With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE (one strain TSE in cows), and the nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human.</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Terry S. Singeltary Sr., <span dir="ltr">Bacliff, Texas USA 77518</span> <span dir="ltr">flounder9@verizon.net</span></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><span dir="ltr"><br /></span></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><span dir="ltr"><a href="http://web.archive.org/web/20110507181935/http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf">http://web.archive.org/web/20110507181935/http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf</a><br /></span></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">WEDNESDAY, JANUARY 25, 2023</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html">https://creutzfeldt-jakob-disease.blogspot.com/2023/01/canada-creutzfeldt-jakob-disease.html</a><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">WEDNESDAY, FEBRUARY 8, 2023</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER SURVEILLANCE TABLES OF CASES EXAMINED January 11th, 2023</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><a href="https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html">https://prionunitusaupdate.blogspot.com/2023/02/national-prion-disease-pathology.html</a><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;">Terry</div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div><div style="-webkit-text-size-adjust: auto; font-family: UICTFontTextStyleTallBody; font-size: 17px;"><br /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-53989113921076557032023-01-28T10:58:00.003-06:002023-01-28T10:58:27.554-06:00Application of real‑time quaking‑induced conversion in Creutzfeldt–Jakob disease surveillance<p> <span style="background-color: white; font-family: arial; font-size: 16px;">Journal of Neurology</span></p><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;"><a fg_scanned="1" href="https://doi.org/10.1007/s00415-022-11549-2" style="color: #196ad4;">doi.org/10.1007/s00415-022-11549-2</a><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">ORIGINAL COMMUNICATION</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Application of real‑time quaking‑induced conversion in Creutzfeldt–Jakob disease surveillance</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Peter Hermann1 · Matthias Schmitz1,2 · Maria Cramm1 · Stefan Goebel1 · Timothy Bunck1 · Julia Schütte‑Schmidt1 · Walter Schulz‑Schaefer3 · Christine Stadelmann4 · Jakob Matschke5 · Markus Glatzel5 · Inga Zerr1,2</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Received: 25 November 2022 / Revised: 22 December 2022 / Accepted: 23 December 2022</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">© The Author(s) 2023</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Abstract</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Background Evaluation of the application of CSF real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance to investigate test accuracy, influencing factors, and associations with disease incidence.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Methods In a prospective surveillance study, CSF real-time quaking-induced conversion was performed in patients with clinical suspicion of prion disease (2014–2022). Clinically or histochemically characterized patients with sporadic Creutzfeldt– Jakob disease (n=888) and patients with fnal diagnosis of non-prion disease (n=371) were included for accuracy and association studies.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Results The overall test sensitivity for sporadic Creutzfeldt–Jakob disease was 90% and the specificity 99%. Lower sensitivity was associated with early disease stage (p=0.029) and longer survival (p<0.001). The frequency of false positives was significantly higher in patients with inflammatory CNS diseases (3.7%) than in other diagnoses (0.4%, p=0.027). The incidence increased from 1.7 per million person-years (2006–2017) to 2.0 after the test was added to diagnostic the criteria (2018–2021).</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Conclusion We validated high diagnostic accuracy of CSF real-time quaking-induced conversion but identified inflammatory brain disease as a potential source of (rare) false-positive results, indicating thorough consideration of this condition in the differential diagnosis of Creutzfeldt–Jakob disease. The surveillance improved after amendment of the diagnostic criteria, whereas the incidence showed no suggestive alterations during the COVID-19 pandemic.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Development of CJD incidence in Germany (2006– 2021)</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">The cumulative incidence of sCJD has increased from 1.7 per million person-years 2006–2017 to 2.0 per million person-years in 2018–2021, when diagnostic criteria including RT-QuIC were applied prospectively (Fig. 3). These data also include sCJD cases that were classified without RTQuIC analyses (based on 14-3-3, MRI, and EEG only) as well as autopsy results from cases without available clinical data. In some RT-QuIC-positive cases, no further or no sufficient clinical information was available to the CJD Surveillance group. These cases were indicated as “unclarified” throughout this manuscript. Including these patients resulted in a cumulative incidence of 2.1 per million person-years (2018–2021). We could not observe any suggestive alteration of sCJD incidence in the years of the COVID-19 pandemic 2020 (2.10) and 2021 (2.01) compared to the preceding year 2019 (2.08), the first year in which all cases were systematically classified according to the amended criteria.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">snip...</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Discussion</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">CSF RT-QuIC has become the gold standard in the laboratory-based diagnosis of sCJD [29]. It is currently applied as a solitary criterion within the biomarker-set of diagnostic protocols [12] and as a “confirmatory” test after CJD typical results from other biomarker analyses [30]. Here, we present comprehensive data from a well-established surveillance system including all patients with diagnosis of sCJD (n=888) and a highly specific cohort of CJD mimics (n=371).</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">We observed a sensitivity of 90% for sporadic sCJD, which is comparable to some reports from surveillance centers using 1st generation RT-QuIC [31, 32] and slightly below the sensitivity of a modified protocol called 2nd generation RT-QuIC (IQ), which was introduced in 2015 [33] and showed a sensitivity ranging from 92 to 96% [33–35]. Several factors that may influence the test sensitivity have been proposed in the past. We observed no significant differences between true and false-positive sCJD patients regarding age and sex, in contrast to a previous study that identified false-negative results to be associated with lower age and female sex [19]. We can only speculate on the reasons for this discrepancy, but it could possibly be associated with different test protocols or with the investigated sCJD cohorts. In that study, only autopsy confirmed cases were evaluated and we observed differences of the age and the sex distribution between probable and definite cases in our cohort (Table 2). We observed no difference of the overall test sensitivity between probable and definite patients in our cohort, though. However, we could replicate an association between longer disease duration and test negativity, and showed that false negative RT-QuIC is associated with early disease stage.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">We also validated high sensitivity for the most frequent MM/MV1, VV2, and MV2 sCJD subtypes [19, 34, 35], whereas sensitivity seems to be lower in the rare MM2 and VV1 subtypes [19, 34]. In some studies [34], sensitivity was slightly higher than in ours, possibly due to different substrates or test protocols. On the other hand, investigated case numbers of these subtypes were rather low in all studies (around 10 or less in each), which may not allow to draw final conclusions. An explanation for the low sensitivity may be that MM2C and VV1 subtypes show predominant cortical PrPSc pathology in early disease stages [6] and slower disease progression than most other sCJD subtypes, possibly resulting in less amount of PrPSc in the CSF. This would be in line with evaluations in genetic prion diseases that reported low sensitivity in entities with slow disease progression (GSS) or pathology restricted to defined structures (brainstem and thalamus in FFI, cerebellum in GSS) in early disease stages [36].</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Regarding the specificity of RT-QuIC, previous analyses showed an excellent accuracy of CSF RT-QuIC of about 99% or higher [12]. Our data from eight years of clinical application indicated a specificity of 99% but for the first time, we evaluated test-related and clinical data in a series of five false-positive cases. Four of them were diagnosed with immune-mediated encephalitis and the rate of false positives was significantly higher than in other diagnostic groups (p=0.027). In addition, single (one of the three) false-positive signal increases were also significantly more frequent among differential diagnoses with inflammatory pathophysiologic background (p=0.001), suggesting a potential causal relationship between encephalitis and false-positive RT-QuIC results. Of course, analytical and pre-analytical factors cannot be excluded. In the literature, only 10 cases of false-positive RT-QuIC or Endpoint-QuIC with clear diagnosis have been reported (see Table 5). They were diagnosed with vascular dementia [32], Alzheimer’s disease [25], mixed dementia [19], and tauopathies [35, 37], immune-mediated encephalitis [20, 35, 38], and amyloid associated vasculitis [39].</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">The possibility of a higher likelihood of false-positive RT-QuIC among encephalitis patients is an important issue because many inflammatory encephalopathies are highly treatable and may represent the most important clinical mimics of CJD and causes of rapidly progressive dementia [40]. On the other hand, all false-positive patients showed either clinical or CSF characteristics that pointed to other diagnoses than CJD, indicating that consideration of factors such as inflammatory signs in the CSF may improve the specificity of an RT-QuIC-based clinical diagnosis. Total CSF Tau protein may also give additional clues because of better specificity in the discrimination of CJD and acute encephalopathies than 14-3-3, but Tau was not available for the false-positive patients. On the other hand, total-tau may also be extremely elevated in encephalitis [41] due to ongoing severe neuronal damage. More important, none of the patients showed CJD-typical MRI. In our autopsy series, no patient received incorrect ante-mortem diagnosis of CJD based on RT-QuIC positivity. We identified only five false-positive results in eight years of RT-QuIC application in a sum of 4599 patients. However, clinical information was only available for 371 control patients, leading to the reported specificity of 99%.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Further investigations have to validate our findings about false-positive RT-QuIC and investigate potential mechanisms. So far, previous studies have not found association of RT-QuIC efficiency and neuronal damage markers such as total-tau and proteins 14-3-3 in sCJD patients [42]. Total PrP was also not associated with seeding efficiency in sCJD [42] but has not been investigated as a factor for false-positive RT-QuIC, yet. On the other hand, total PrP was not shown to be significantly altered in encephalitis compared to cerebral ischemia or control patients [43]. Another potential reason may be the influence of factors in the CSF that are directly linked to neuro-inflammation. Epileptic activity in encephalitis patients was also discussed as a cause for false-positive results [21]. Our data did not allow the evaluation of the presence of seizures in relation to lumbar puncture in control patients, but patients with primary diagnosis of seizures or status epilepticus caused by idiopathic epilepsy syndromes, or reversible conditions such as alcohol withdrawal showed a low frequency of positive test replicates (one in 30 patients). However, the mechanisms for false-positive results may be related to the CSF. RT-QuIC from other body tissues such as olfactory mucosa [44, 45] are an alternative clinical test and should be investigated in future studies on the specificity of RT-QuIC in neuro-inflammatory diseases. Clarification of the reasons for false-positive PrPSc RT-QuIC reactions may also be highly relevant for the application of other protein amplification assays such as α-Synuclein RT-QuIC. So far, only very few false-positive results have been reported and were associated with Wernicke’s encephalopathy, Alzheimer’s disease, and encephalitis [46, 47].</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Although our study provides comprehensive data on clinical experience with RT-QuIC, the study has naturally some limitations. First, our test protocol [24] uses chimeric hamster-sheep recombinant PrP as substrate, whereas most other centers are using hamster recombinant PrP [32, 33]. Some centers showed that IQ-CSF RT-QuIC may have a superior sensitivity for sCJD compared to previous protocols [30, 45, 48]. It remains unclear, whether IQ RT-QuIC underlays the same or similar confounders for PrP seeding as our protocol but international ring trials have shown that RT-QuIC results are highly concordant among different test centers and test protocols [25, 49]. Our protocol has been well established over years and we have achieved a high level of experience to perform the test in a reliable and reproducible way.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Regarding the sensitivity, further histochemical characterization of sCJD was only available in a limited number of cases (n=161), discouraging reliable conclusions on the sensitivity of rare (MM2, VV1) sCJD subtypes. Similarly, Codon 129 was analyzed in a rather small subset of patients (n=114). Lastly, our surveillance data includes a number of uncharacterized cases with suspected prion disease or positive CSF RT-QuIC. In these cases, further clinical data were not available and thus, we cannot exclude additional false-positive or false-negative test results in this group. Our cohort may be prone to an according selection bias. On the other hand, we assume that this bias might rather be less relevant than in retrospective case–control studies or in studies including only autopsy-confirmed cases.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">As a secondary outcome, we observed an increased overall sCJD incidence in Germany after inclusion of the test in the clinical diagnostic protocol. In this context, we assume that the increase from 1.7 (2009–2017) to 2.0 (2018–2021) person-years is a result of the improved clinical detection of early sCJD cases (based on positive RT-QuIC) as previously suggested or observed by our group [18] and others [13, 17, 19]. This effect was also described in the context of previous criteria modifications [50]. Another interesting observation was the apparent lack of an alteration of the overall sCJD incidence during the Covid-19 pandemic. Annual numbers of positive RT-QuIC results remained stable between 2019 and 2021 (Fig. 3). Although we cannot exclude influence of viral infections on genesis or course of prion diseases, our surveillance data do not suggest an immediate causal relationship between COVID-19-related factors and CJD on the population level.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Conclusion</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Chimeric PrP CSF RT-QuIC is an accurate diagnostic tool for the differential diagnosis of sCJD. If the test is interpreted in the context of a complete diagnostic work-up, it may provide an extremely high level of ante-mortem diagnostic certainty. RT-QuIC negativity combined with absence of CJD-typical results in 14-3-3 analysis and MRI indicates extremely low likelihood of sCJD. The routine application of RT-QuIC improves CJD surveillance and leads to a formal increase of the disease incidence. However, the sensitivity is influenced by disease stage and disease subtype. False positive results may occur and clinicians have to be aware of this possibility. In cases with ambiguous clinical presentation, we recommend consideration of other diagnostics, in particular MRI, and repetitive RT-QuIC analyses from the same sample to exclude the influence of analytical factors. Nonetheless, a consecutive lumbar puncture, at best after therapeutic intervention, may be necessary to detect false positivity based on pre-analytical or disease-related factors. In this context, RT-QuIC from other body tissues such as olfactory mucosa may be an alternative, if available. Inflammatory CNS disease, especially immune-mediated encephalitides, should always be considered as potential clinical and laboratory mimics of CJD. Although general comparability of different RT-QuIC protocols and substrates have been shown, our pilot findings need to be verified through studies using other body tissues and test protocols such as IQ.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Supplementary Information The online version contains supplementary material available at <a fg_scanned="1" href="https://doi.org/10.1007/s00415-022-11549-2" style="color: #196ad4;">doi.org/10.1007/s00415-022-11549-2</a> .</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Acknowledgements We would like to thank all collaborating health care institutions, hospitals, physicians, patients, and families that provided information for diagnostic classifications. In addition, we acknowledge the Center for Neuropathology and Prion Research at the Ludwig-Maximilian-University in Munich, Germany for performing neuropathological investigations in several cases of suspected prion disease.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Funding Open Access funding enabled and organized by Projekt DEAL. This study was funded by the Robert-Koch-Institute through funds from the Federal Ministry of Health; grant number 1369-341. Data availability All manuscript-related data is available and will be provided upon reasonable request.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Declarations</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Conflicts of interest The authors declare that they have no relevant financial or non-financial interests to disclose.</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;"><a href="https://link.springer.com/content/pdf/10.1007/s00415-022-11549-2.pdf" style="color: #196ad4;">link.springer.com/content/pdf/10.1007/s00415-022-11549-2.pdf</a><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div style="background-color: white; font-family: arial; font-size: 16px;">Keywords Creutzfeldt–Jakob disease · Prion · Diagnosis · RT-QuIC · Epidemiology</div><div style="background-color: white; font-family: arial; font-size: 16px;"><br /></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-3278493534118123972023-01-25T12:13:00.003-06:002023-01-25T15:02:52.986-06:00Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD<p>Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report steady rise in cases as of January 2023 and STILL NO CASES REPORTED OF VPSPr CJD</p><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div><div dir="ltr"><div><div dir="ltr">wow, just wow, i don't know where to start with Canada and CJD and strange neurological conditions mounting. </div><div dir="ltr"><br /></div><div dir="ltr">seems the New Brunswick strange neurological cases of unknown origin just still will not go away, and CJD cases in Canada keep rising.</div><div><br /></div><div>Canada Creutzfeldt-Jakob disease surveillance system (CJDSS) report Update January 2023</div><div><br /></div><div dir="ltr">(PLEASE NOTE, ''The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians.'' HAS BEEN USED 'ad nauseam' YEAR, AFTER YEAR, AFTER YEAR, OF RISKING CJD CASES.)</div><div><br /></div><div>(PLEASE NOTE, OCTOBER OF 2022 THERE WERE 65 CJD CASES REPORTED, TWO MONTHS LATER, DECEMBER 2022, CJD CASES JUMPED TO 154 CASES REPORTED...tss)</div><div><br /></div><div>(PLEASE NOTE, STILL, CANADA DOES NOT REPORT VPSPr CJD TSE Prion Cases to the public, see links below for this explanation and discussion. tss)</div><div><br /></div><div>Referrals of suspected CJD reported by CJDSS, 1998-2022 As of December 31, 2022</div><div><br /></div><div>Year of reporting Number of referrals</div><div><br /></div><div>1998 43</div><div><br /></div><div>1999 63</div><div><br /></div><div>2000 82</div><div><br /></div><div><span dir="ltr">2001 101</span></div><div><br /></div><div><span dir="ltr">2002 103</span></div><div><br /></div><div>2003 75</div><div><br /></div><div>2004 90</div><div><br /></div><div>2005 97</div><div><br /></div><div>2006 80</div><div><br /></div><div><span dir="ltr">2007 101</span></div><div><br /></div><div><span dir="ltr">2008 100</span></div><div><br /></div><div><span dir="ltr">2009 104</span></div><div><br /></div><div>2010 76</div><div><br /></div><div><span dir="ltr">2011 102</span></div><div><br /></div><div><span dir="ltr">2012 103</span></div><div><br /></div><div>2013 99</div><div><br /></div><div>2014 99</div><div><br /></div><div>2015 98</div><div><br /></div><div><span dir="ltr">2016 117</span></div><div><br /></div><div><span dir="ltr">2017 116</span></div><div><br /></div><div><span dir="ltr">2018 125</span></div><div><br /></div><div><span dir="ltr">2019 142</span></div><div><br /></div><div><span dir="ltr">2020 123</span></div><div><br /></div><div><span dir="ltr">2021 140</span></div><div><br /></div><div><span dir="ltr">2022 154</span></div><div><br /></div><div>Total 2533</div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html</a></div><div><br /></div><div><a href="https://www.canada.ca/content/dam/phac-aspc/documents/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system/cjd-surveillance-system.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/content/dam/phac-aspc/documents/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system/cjd-surveillance-system.pdf</a></div><div><br /></div><div>Canada Definite and probable CJD, 1998-2022 As of October 31, 2022</div><div><br /></div><div>Year Sporadic Iatrogenic CJD Genetic vCJD Total</div><div><br /></div><div><span dir="ltr">1998 22 1 1 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 3 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 3 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 3 0 30</span></div><div><br /></div><div><span dir="ltr">2002 30 0 5 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 4 0 44</span></div><div><br /></div><div><span dir="ltr">2005 42 0 2 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 5 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 4 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 5 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 38</span></div><div><br /></div><div><span dir="ltr">2011 46 0 4 1 51</span></div><div><br /></div><div><span dir="ltr">2012 62 0 1 0 63</span></div><div><br /></div><div><span dir="ltr">2013 50 0 1 0 51</span></div><div><br /></div><div><span dir="ltr">2014 51 0 5 0 56</span></div><div><br /></div><div><span dir="ltr">2015 44 0 8 0 52</span></div><div><br /></div><div><span dir="ltr">2016 57 1 6 0 64</span></div><div><br /></div><div><span dir="ltr">2017 82 0 5 0 87</span></div><div><br /></div><div><span dir="ltr">2018 75 1 5 0 81</span></div><div><br /></div><div><span dir="ltr">2019 76 0 2 0 78</span></div><div><br /></div><div><span dir="ltr">2020 65 0 4 0 69</span></div><div><br /></div><div><span dir="ltr">2021 56 0 3 0 59</span></div><div><br /></div><div><span dir="ltr">2022 64 0 1 0 65</span></div><div><br /></div><div>Total <span dir="ltr">1183 6 83 2 1274</span></div><div><br /></div><div>Cases with definite and probable diagnosis to date</div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a></div><div><br /></div><div>PLEASE NOTE, Canada does not mention VPSPr Variably protease-sensitive prionopathy and you can read why here ;</div><div><br /></div><div>WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div><br /></div><div>THIS problem must be addressed immediately imo.</div><div><br /></div><div>WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div><br /></div><div>QUOTE;</div><div><br /></div><div>''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''</div><div><br /></div><div>''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</div><div><br /></div><div>end</div><div><br /></div><div><a fg_scanned="1" href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow" style="color: #196ad4;" target="_blank">vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a></div><div><br /></div><div><a fg_scanned="1" href="https://vpspr.blogspot.com/" rel="nofollow" style="color: #196ad4;" target="_blank">vpspr.blogspot.com/</a></div><div><br /></div><div>Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</div><div><br /></div><div><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a><br /></div></div><div><br /></div><div dir="ltr"><div><div>CANADA CJD 2020</div><div><br /></div><div>***> CANADA, I find it very odd that Canada has NO recorded or documented cases of Variably Protease-Sensitive Prionopathy (VPSPr)?</div><div><br /></div><div>CANADA Creutzfeldt-Jakob disease surveillance system (CJDSS) report</div><div><br /></div><div>Definite and probable CJD, 1998-2020</div><div><br /></div><div>As of 31 October, 2020</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 2 1 0 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2005 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 0 0 38</span></div><div><br /></div><div><span dir="ltr">2011 46 0 3 1 0 1 51</span></div><div><br /></div><div><span dir="ltr">2012 62 0 1 0 0 0 63</span></div><div><br /></div><div><span dir="ltr">2013 50 0 0 0 1 0 51</span></div><div><br /></div><div><span dir="ltr">2014 51 0 4 0 1 0 56</span></div><div><br /></div><div><span dir="ltr">2015 44 0 5 1 2 0 52</span></div><div><br /></div><div><span dir="ltr">2016 57 1 5 1 0 0 64</span></div><div><br /></div><div><span dir="ltr">2017 82 0 2 1 1 0 86</span></div><div><br /></div><div><span dir="ltr">2018 74 1 4 0 1 0 80</span></div><div><br /></div><div><span dir="ltr">2019 76 0 2 0 0 0 78</span></div><div><br /></div><div><span dir="ltr">2020 30 0 2 0 0 0 32</span></div><div><br /></div><div>Total <span dir="ltr">1027 6 45 23 7 2 1110</span></div><div><br /></div><div>Cases with definite and probable diagnosis to date.</div><div><br /></div><div>Gerstmann-Sträussler-Scheinker disease (GSS)</div><div><br /></div><div>Fatal familial insomnia (FFI)</div><div><br /></div><div>Variant CJD (vCJD)</div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html</a><br /></div></div><div><br /></div><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2020/12/sporadic-creutzfeldt-jakob-disease-scjd.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2020/12/sporadic-creutzfeldt-jakob-disease-scjd.html</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><div><div>CANADA</div><div><br /></div><div>Creutzfeldt-Jakob Disease Surveillance System Report</div><div><br /></div><div>Definite and probable CJD, 1998-2019</div><div><br /></div><div>As of 31 July, 2019</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 2 1 0 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2005 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 0 0 38</span></div><div><br /></div><div><span dir="ltr">2011 46 0 3 1 0 1 51</span></div><div><br /></div><div><span dir="ltr">2012 62 0 1 0 0 0 63</span></div><div><br /></div><div><span dir="ltr">2013 50 0 0 0 1 0 51</span></div><div><br /></div><div><span dir="ltr">2014 51 0 4 0 1 0 56</span></div><div><br /></div><div><span dir="ltr">2015 44 0 5 1 2 0 52</span></div><div><br /></div><div><span dir="ltr">2016 55 1 5 1</span> - - 62</div><div><br /></div><div><span dir="ltr">2017 78 - 1 1 1 - 81</span></div><div><br /></div><div><span dir="ltr">2018 65 1 3 - 1 - 70</span></div><div><br /></div><div><span dir="ltr">2019 20 0</span> - - - - 20</div><div><br /></div><div>Total <span dir="ltr">926 6 39 23 7 2 1003</span></div><div><br /></div><div>Cases with definite & probable diagnosis to date.</div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a></div></div><br /></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2019/08/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2019/08/creutzfeldt-jakob-disease-cjd-tse-prion.html</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><div><div>***> CANADA CJD 2018</div><div><br /></div><div>see substantial increase in sporadic cjd in Canada 2017...terry</div><div><br /></div><div>Definite and probable CJD, 1998-2018</div><div><br /></div><div>As of 31 October, 2018</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 2 1 0 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2005 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 0 0 38</span></div><div><br /></div><div><span dir="ltr">2011 46 0 3 1 0 1 51</span></div><div><br /></div><div><span dir="ltr">2012 62 0 1 0 0 0 63</span></div><div><br /></div><div><span dir="ltr">2013 50 0 0 0 1 0 51</span></div><div><br /></div><div><span dir="ltr">2014 51 0 4 0 1 0 56</span></div><div><br /></div><div><span dir="ltr">2015 44 0 5 1 2 0 52</span></div><div><br /></div><div><span dir="ltr">2016 55 1 5 1 62</span></div><div><br /></div><div><span dir="ltr">2017 77 1 1 1 80</span></div><div><br /></div><div><span dir="ltr">2018 35 1 36</span></div><div><br /></div><div>Total <span dir="ltr">875 5 36 23 7 2 948</span> </div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#cases" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#cases</a> </div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html</a></div><div><br /></div><div><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow" style="color: #196ad4;" target="_blank">www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a></div><div><br /></div><div><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2018/12/creutzfeldt-jakob-disease-cjd-bse.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2018/12/creutzfeldt-jakob-disease-cjd-bse.html</a><br /></div></div><div><br /></div><div dir="ltr"><div><div>CANADA CJD As of October 31, 2015</div><div><br /></div><div>Referrals of Suspected CJD Reported by CJDSS, 1997-2015 As of October 31, 2015</div><div><br /></div><div>Year of Reporting Number of Referrals</div><div><br /></div><div>1997 4</div><div><br /></div><div>1998 43</div><div><br /></div><div>1999 63</div><div><br /></div><div>2000 82</div><div><br /></div><div><span dir="ltr">2001 101</span></div><div><br /></div><div><span dir="ltr">2002 103</span></div><div><br /></div><div>2003 75</div><div><br /></div><div>2004 90</div><div><br /></div><div>2005 97</div><div><br /></div><div>2006 80</div><div><br /></div><div><span dir="ltr">2007 101</span></div><div><br /></div><div><span dir="ltr">2008 100</span></div><div><br /></div><div><span dir="ltr">2009 104</span></div><div><br /></div><div>2010 76</div><div><br /></div><div><span dir="ltr">2011 102</span></div><div><br /></div><div><span dir="ltr">2012 103</span></div><div><br /></div><div>2013 99</div><div><br /></div><div>2014 99</div><div><br /></div><div>2015 80</div><div><br /></div><div>Total 1602</div><div><br /></div><div>CJD Deaths Reported by CJDSS, 1994-2015 As of October 31, 2015</div><div><br /></div><div>Deaths of Definite and Probable CJD</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div>Cases with definite & probable diagnosis to date.</div><div><br /></div><div><span dir="ltr">1994 2 0 0 1 0 0 3</span></div><div><br /></div><div><span dir="ltr">1995 3 0 0 0 0 0 3</span></div><div><br /></div><div><span dir="ltr">1996 13 0 0 0 0 0 13</span></div><div><br /></div><div><span dir="ltr">1997 16 0 1 1 0 0 18</span></div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 2 1 0 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2005 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 0 0 38</span></div><div><br /></div><div><span dir="ltr">2011 46 0 3 1 0 1 51</span></div><div><br /></div><div><span dir="ltr">2012 62 0 1 0 0 0 63</span></div><div><br /></div><div><span dir="ltr">2013 50 0 0 0 1 0 51</span></div><div><br /></div><div><span dir="ltr">2014 49 0 4 0 1 0 54</span></div><div><br /></div><div><span dir="ltr">2015 23 0 1 0 0 0 24</span></div><div><br /></div><div>Total <span dir="ltr">719 4 27 22 3 2 777</span></div><div><br /></div><div>CJD Cases by Province/Territory October 31, 2015</div><div><br /></div><div>CJD Cases by Province/Territory October 30, 2015</div><div><br /></div><div>Text Equivalent - CJD Cases by Province/Territory October 31, 2015</div><div><br /></div><div>Incidence of CJD Deaths Reported by CJDSS in Canada As of October 31, 2015</div><div><br /></div><div>Year of Death Total CJD Cases Population of Canada Incidence Rate</div><div><br /></div><div>Cases with definite & probable diagnosis to date.2014 Population Source External Link</div><div><br /></div><div><span dir="ltr">1999 32 30,492,106</span> 1.05</div><div><br /></div><div><span dir="ltr">2000 35 30,783,969</span> 1.14</div><div><br /></div><div><span dir="ltr">2001 30 31,130,030</span> 0.96</div><div><br /></div><div><span dir="ltr">2002 36 31,450,443</span> 1.14</div><div><br /></div><div><span dir="ltr">2003 29 31,734,851</span> 0.91</div><div><br /></div><div><span dir="ltr">2004 44 32,037,434</span> 1.37</div><div><br /></div><div><span dir="ltr">2005 44 32,352,233</span> 1.36</div><div><br /></div><div><span dir="ltr">2006 44 32,678,986</span> 1.35</div><div><br /></div><div><span dir="ltr">2007 39 33,001,076</span> 1.18</div><div><br /></div><div><span dir="ltr">2008 49 33,371,810</span> 1.47</div><div><br /></div><div><span dir="ltr">2009 53 33,756,714</span> 1.57</div><div><br /></div><div><span dir="ltr">2010 38 34,131,451</span> 1.11</div><div><br /></div><div><span dir="ltr">2011 51 34,472,304</span> 1.48</div><div><br /></div><div><span dir="ltr">2012 63 34,880,248</span> 1.81</div><div><br /></div><div><span dir="ltr">2013 51 35,289,003</span> 1.45</div><div><br /></div><div><span dir="ltr">2014 54 35,675,834</span> 1.51</div><div><br /></div><div><span dir="ltr">2015 24 35,702,707</span> 0.81</div><div><br /></div><div><span dir="ltr">http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php</span> </div></div><br /></div><div dir="ltr"><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html</a><br /></div></div><div dir="ltr"><br /></div><div dir="ltr"><div><div>P.179: Sporadic Creutzfeldt-Jakob disease in Canada</div><div><br /></div><div>Zheng Wang,1 Gerard Jansen,1,2 Stacy Sabourin,1 Rolande D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 David J Knox,3 Neil R Cashman,4 and Michael B Coulthart1 1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital; Ottawa, ON Canada; 3National Microbiology Laboratory; Public Health Agency of Canada; Winnipeg, MB Canada; 4Brain Research Centre; University of British Columbia; Vancouver, BC Canada</div><div><br /></div><div>Background. Sporadic Creutzfeldt-Jakob Disease (sCJD) is a fatal, transmissible neurodegenerative disease. Systematic surveillance has repeatedly shown annual mortality in the range 1 to 2 per million population, has elucidated key characteristics of sCJD, and led to recognition of a new form of CJD, variant CJD (vCJD), which is associated with BSE. In 1998, Canada launched comprehensive national CJD surveillance to assess the characteristics of CJD in Canada, identify any acquired cases of CJD (such as vCJD, of which 2 imported cases have been identified in Canada to date), and mitigate public health risks. This study describes the epidemiology of sCJD in Canada from 1998 to 2012.</div><div><br /></div><div>Methods. Case ascertainment was based on internationally accepted criteria. Demographic and medical information were collected by standardized questionnaire and medical chart review. Poisson regression and descriptive analysis were employed.</div><div><br /></div><div>Results. A total of 563 sCJD deaths (definite: 462, probable: 101) in Canadian residents were registered from 1998 to 2012. Average annual sCJD mortality was 1.2 per million population, increasing gradually from 0.9 in 1999 to 1.7 in 2012 (P = 0.0004). All provinces saw average annual mortalities ranging from 1.0 to 1.6 (P = 0.25), except three territories where population is small (~25,000 to ~45,000) and no cases were identified. sCJD occurred at similar rates in males (1.1) and females (1.2) (P = 0.21). sCJD was rare under 50 years of age with only 11 cases identified (2.7%). Mortality increased after 50 and peaked at 7.4 per million in the 70–74 age group. Median age at death was 69 and median duration of illness was 4 months. Genotype at codon 129 (N = 358) revealed that the MM subgroup accounted for 223 (62%, median age at death: 69, duration: 4), the MV subgroup was 82 (23%, median age at death: 68, duration: 9), and the VV subgroup was 53 (23%, median age at death: 66, duration: 5). Results of molecular typing (Parchi Scheme) for 256 cases are; MM1: 140, MM2: 11, MV1: 28, MV2: 18, VV1: 5, VV2: 25, Mixture: 29.</div><div><br /></div><div>Conclusion. Characteristics of sCJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be partly attributed to increased awareness of CJD among Canadian clinicians. These findings support the conclusion that Canadian CJD surveillance system is sufficiently sensitive to accurately characterize the epidemiology of sCJD in Canada, and to detect potential additional cases of acquired CJD such as vCJD or human chronic wasting disease.</div><div><br /></div><div>Conclusion. Characteristics of sCJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be partly attributed to increased awareness of CJD among Canadian clinicians. These findings support the conclusion that Canadian CJD surveillance system is sufficiently sensitive to accurately characterize the epidemiology of sCJD in Canada, and to detect potential additional cases of acquired CJD such as vCJD or human chronic wasting disease.</div><div><br /></div><div data-setdir="false" dir="ltr"><div><a fg_scanned="1" href="http://www.tandfonline.com/doi/pdf/10.4161/pri.29371" style="color: #196ad4;">www.tandfonline.com/doi/pdf/10.4161/pri.29371</a></div><div><br /></div></div><div>HD.18: Creutzfeldt-Jakob disease reporting in Canada</div><div><br /></div><div>Zheng Wang,1 Gerard H. Jansen,1, 2 Elina Olsen,1 Rolande D’Amour,1 Stacy Sabourin,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael Coulthart1 1Public Health Agency of Canada; Ottawa, ON CA; 2Department of Pathology; Ottawa Hospital; Ottawa, ON CA; 3Brain Research Centre; University of British Columbia; Vancouver, BC CA</div><div><br /></div><div>Background. To deal with risks of infectious transmission of Creutzfeldt-Jakob disease (CJD), in 1998 the Government of Canada launched a prospective national CJD surveillance system (CJDSS). In 2000, CJD became nationally notifiable in Canada, and since then all Canadian Provinces and Territories (P/Ts) have made CJD reportable. It has been recognized that the CJDSS registers more cases of CJD than are reported to P/T Ministries of Health (PTMH). Because the CJDSS may not legally share personal information with PTMH, in 2008 the CJDSS began to systematically discuss the issue of CJD reporting with referring health care professionals (HCP). The present study was undertaken to estimate any changes in P/T CJD reporting from 2008, and to identify possible areas for further improvement.</div><div><br /></div><div>Materials and Methods. P/T CJD data were retrieved from the Public Health Agency of Canada’s National Notifiable Disease System (NNDS) database, and compared with CJDSS data. CJDSS intake sheets were examined, to determine if the case had been reported to the PTMH at the time of notification.</div><div><br /></div><div>Results. From 2005 to 2010, NNDS received complete data on CJD from 5 P/Ts. During the same period, 134 cases of CJD (probable or neuropathologically confirmed) were reported by the 5 P/Ts while 210 CJD deaths (probable or definite) were recorded in the CJDSS from the same 5 P/Ts. Between 2008 and 2010 there was an increase of ~48% in P/T CJD reports compared with the period 2005–2007. In contrast, the CJDSS registered only ~12% more CJD deaths between 2008 and 2010 compared with 2005–2007, supporting an interpretation of improved P/T reporting. Examination of intake sheets from 172 notifications that were made to the CJDSS from the same 5 P/Ts between 2008 and 2010 revealed that 30 were known to have been reported to PTMH at the time of referring (24 were CJD, 5 were non-CJD, and 1 was unclassifiable). 142 were not reported or had unknown reporting status. Reasons cited by HCPs for not reporting included (1) uncertainty of the CJD diagnosis; (2) uncertainty regarding responsibility for reporting; (3) lack of awareness that CJD is reportable; and (4) uncertainty regarding when or how to report.</div><div><br /></div><div>Conclusion. The considerable increase of CJD reports in P/Ts since 2008 occurred concurrently with efforts of the CJDSS to engage HCPs on the issue of CJD reporting requirements. P/T CJD reports may include non-CJD cases. Inter-jurisdiction collaboration is underway to further improve CJD reporting.</div><div><br /></div><div><a fg_scanned="1" href="http://www.tandfonline.com/doi/pdf/10.4161/pri.24865" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.24865</a></div></div><div><br /></div></div><div dir="ltr"><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2015/12/creutzfeldt-jakob-disease-cjd-tse-prion.html</a><br /></div><div dir="ltr"><br /></div><div dir="ltr">Canada CJD 2015</div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2015/03/" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2015/03/</a></div><div><br /></div><div><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/06/transmissible-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">transmissiblespongiformencephalopathy.blogspot.com/2014/06/transmissible-spongiform-encephalopathy.html</a></div><div><br /></div><div dir="ltr"><div>2012</div><div><br /></div><div>CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss</div><div><br /></div><div>PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD) in Canada is also on a steady increase.</div><div><br /></div><div>please see ;</div><div><br /></div><div>> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.</div><div><br /></div><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20122</span></div><div><br /></div><div>As of May 31, 2012</div><div><br /></div><div>Deaths of Definite and Probable CJD</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1994 2 0 0 1 0 0 3</span></div><div><br /></div><div><span dir="ltr">1995 3 0 0 0 0 0 3</span></div><div><br /></div><div><span dir="ltr">1996 13 0 0 0 0 0 13</span></div><div><br /></div><div><span dir="ltr">1997 16 0 1 1 0 0 18</span></div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 26 2 2 1 0 0 31</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 0 0 0 43</span></div><div><br /></div><div><span dir="ltr">2005 42 0 0 2 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 34 0 3 0 0 0 37</span></div><div><br /></div><div><span dir="ltr">2011 37 0 2 1 0 1 41</span></div><div><br /></div><div><span dir="ltr">2012 1 0 0 0 0 0 1</span></div><div><br /></div><div>Total <span dir="ltr">525 4 19 22 1 2 573</span></div><div><br /></div><div>1. CJDSS began in 1998</div><div><br /></div><div>2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional</div><div><br /></div><div>3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.</div><div><br /></div><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20122</span></div><div><br /></div><div>As of May 31, 2012</div><div><br /></div><div><a href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0512-eng.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0512-eng.pdf</a></div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html</a></div><div><br /></div><div>SEE DECEMBER 2012 CANADA</div><div><br /></div><div><a fg_scanned="1" href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php#canada" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php#canada</a><br /></div><div><br /></div><div dir="ltr">UNITED STATES</div><div><br /></div><div>USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss</div><div><br /></div><div>National Prion Disease Pathology Surveillance Center</div><div><br /></div><div>Cases Examined1</div><div><br /></div><div>(May 18, 2012)</div><div><br /></div><div>Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD</div><div><br /></div><div>1996 & earlier <span dir="ltr">50 32 28 4 0 0</span></div><div><br /></div><div><span dir="ltr">1997 114 68 59 9 0 0</span></div><div><br /></div><div><span dir="ltr">1998 88 52 44 7 1 0</span></div><div><br /></div><div><span dir="ltr">1999 123 74 65 8 1 0</span></div><div><br /></div><div><span dir="ltr">2000 145 103 89 14 0 0</span></div><div><br /></div><div><span dir="ltr">2001 210 120 110 10 0</span> 0</div><div><br /></div><div><span dir="ltr">2002 248 149 125 22 2</span> 0</div><div><br /></div><div><span dir="ltr">2003 266 168 137 31 0</span> 0</div><div><br /></div><div><span dir="ltr">2004 326 187 164 22 0</span> 13</div><div><br /></div><div><span dir="ltr">2005 344 194 157 36 1</span> 0</div><div><br /></div><div><span dir="ltr">2006 382 196 166 28 0</span> 24</div><div><br /></div><div><span dir="ltr">2007 377 213 185 28 0</span> 0</div><div><br /></div><div><span dir="ltr">2008 396 232 206 26 0</span> 0</div><div><br /></div><div><span dir="ltr">2009 423 256 212 43 1</span> 0</div><div><br /></div><div><span dir="ltr">2010 413 257 216 41 0</span> 0</div><div><br /></div><div><span dir="ltr">2011 410 257 213 43 0</span> 0</div><div><br /></div><div><span dir="ltr">2012 153 82 51 15 0 0</span></div><div><br /></div><div>TOTAL <span dir="ltr">44685 26406</span> <span dir="ltr">2227 387 6 3</span></div><div><br /></div><div>1 Listed based on the year of death or, if not available, on year of referral;</div><div><br /></div><div>2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;</div><div><br /></div><div>3 Disease acquired in the United Kingdom;</div><div><br /></div><div>4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;</div><div><br /></div><div>5 Includes 14 cases in which the diagnosis is pending, and 18 inconclusive cases;</div><div><br /></div><div>6 Includes 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded. The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).</div><div><br /></div><div>Rev 5/18/2012</div><div><br /></div><div><a href="http://www.cjdsurveillance.com/pdf/case-table.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">www.cjdsurveillance.com/pdf/case-table.pdf</a></div><div><br /></div><div>> 6 Includes</div><div><br /></div><div>> 17 (16 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded.</div><div><br /></div><div>> The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).</div><div><br /></div><div>WELL, it seems the USA mad cow strains in humans classified as type determination pending tdpCJD, VPSPr, sFFI, and sCJD) have steadily increased over the years, and the same old song and dance continues with sporadic CJD cases $$$</div><div><br /></div><div><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/06/transmissible-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">transmissiblespongiformencephalopathy.blogspot.com/2014/06/transmissible-spongiform-encephalopathy.html</a></div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2012/06/creutzfeldt-jakob-disease-human-tse.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2012/06/creutzfeldt-jakob-disease-human-tse.html</a></div><div><br /></div><div>VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $</div><div><br /></div><div>OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles</div><div><br /></div><div>Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA</div><div><br /></div><div>Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.</div><div><br /></div><div>Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.</div><div><br /></div><div>Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.</div><div><br /></div><div>In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.</div><div><br /></div><div>Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.</div><div><br /></div><div>The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.</div><div><br /></div><div><a href="http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf" style="color: #196ad4;">www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf</a><br /></div><div><br /></div><div><a fg_scanned="1" href="https://wwwnc.cdc.gov/eid/article/25/1/18-0807_article" style="color: #196ad4;">wwwnc.cdc.gov/eid/article/25/1/18-0807_article</a></div><div><br /></div><div>Wednesday, March 28, 2012</div><div><br /></div><div>VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $</div><div><br /></div><div><a fg_scanned="1" href="http://prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html</a></div></div></div><div dir="ltr"><div><div><br /></div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-cases.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-cases.html</a></div></div><div><br /></div><div dir="ltr"><div><div>SEE DECEMBER 2012 CANADA</div><div dir="ltr"> <div><div>CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss</div><div><br /></div><div>PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD) in Canada is also on a steady increase.</div><div><br /></div><div> please see ;</div><div><br /></div><div>> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.</div><div><br /></div><div dir="ltr"><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20122</span></div><div><br /></div><div>As of Oct 31, 2012</div><div><br /></div><div>Deaths of Definite and Probable CJD</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1994 2 0 0 1 0 0 3</span></div><div><br /></div><div><span dir="ltr">1995 3 0 0 0 0 0 3</span></div><div><br /></div><div><span dir="ltr">1996 13 0 0 0 0 0 13</span></div><div><br /></div><div><span dir="ltr">1997 16 0 1 1 0 0 18</span></div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 27 2 2 1 0 0 32</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 1 0 0 44</span></div><div><br /></div><div><span dir="ltr">2005 41 0 1 1 0 0 43</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 35 0 3 0 0 0 38</span></div><div><br /></div><div><span dir="ltr">2011 41 0 2 1 0 1 45</span></div><div><br /></div><div><span dir="ltr">2012 20 0 0 0 0 0 20</span></div><div><br /></div><div>Total <span dir="ltr">549 4 20 22 1 2 598</span></div><div><br /></div><div>1. CJDSS began in 1998</div><div><br /></div><div>2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional</div><div><br /></div><div>3. Final classification of 48 cases from 2009, 2010, 2011 and 2012 is pending.</div><div><br /></div><div><a href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_1012-eng.pdf" style="color: #196ad4;">www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_1012-eng.pdf</a></div><div><br /></div><div>snip...see full text ;</div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2012/11/sporadic-creutzfeldt-jakob-disease.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2012/11/sporadic-creutzfeldt-jakob-disease.html</a></div></div><div><br /></div><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20122</span></div><div><br /></div><div>As of May 31, 2012</div><div><br /></div><div>Deaths of Definite and Probable CJD</div><div><br /></div><div>Year Sporadic Iatrogenic Familial GSS FFI vCJD Total</div><div><br /></div><div><span dir="ltr">1994 2 0 0 1 0 0 3</span></div><div><br /></div><div><span dir="ltr">1995 3 0 0 0 0 0 3</span></div><div><br /></div><div><span dir="ltr">1996 13 0 0 0 0 0 13</span></div><div><br /></div><div><span dir="ltr">1997 16 0 1 1 0 0 18</span></div><div><br /></div><div><span dir="ltr">1998 22 1 0 1 0 0 24</span></div><div><br /></div><div><span dir="ltr">1999 26 2 2 1 0 0 31</span></div><div><br /></div><div><span dir="ltr">2000 32 0 0 3 0 0 35</span></div><div><br /></div><div><span dir="ltr">2001 27 0 2 1 0 0 30</span></div><div><br /></div><div><span dir="ltr">2002 31 0 2 2 0 1 36</span></div><div><br /></div><div><span dir="ltr">2003 27 1 1 0 0 0 29</span></div><div><br /></div><div><span dir="ltr">2004 42 0 1 0 0 0 43</span></div><div><br /></div><div><span dir="ltr">2005 42 0 0 2 0 0 44</span></div><div><br /></div><div><span dir="ltr">2006 39 0 1 3 1 0 44</span></div><div><br /></div><div><span dir="ltr">2007 35 0 0 4 0 0 39</span></div><div><br /></div><div><span dir="ltr">2008 48 0 1 0 0 0 49</span></div><div><br /></div><div><span dir="ltr">2009 48 0 3 2 0 0 53</span></div><div><br /></div><div><span dir="ltr">2010 34 0 3 0 0 0 37</span></div><div><br /></div><div><span dir="ltr">2011 37 0 2 1 0 1 41</span></div><div><br /></div><div><span dir="ltr">2012 1 0 0 0 0 0 1</span></div><div><br /></div><div>Total <span dir="ltr">525 4 19 22 1 2 573</span></div><div><br /></div><div>1. CJDSS began in 1998</div><div><br /></div><div>2. Data before 1998 are retrospective and partial, data from 1998 to 2008 are complete, and data for 2009 - 2012 are provisional</div><div><br /></div><div>3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is pending.</div><div><br /></div><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20122</span></div><div><br /></div><div>As of May 31, 2012</div><div><br /></div><div><a href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0512-eng.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0512-eng.pdf</a></div><div><br /></div><div><a fg_scanned="1" href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php#canada" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats-eng.php#canada</a><br /></div></div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2012/12/creutzfeldt-jakob-tse-prion-disease.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2012/12/creutzfeldt-jakob-tse-prion-disease.html</a><br /></div><div><br /></div></div><div dir="ltr"><div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html</a></div><div><br /></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-cases.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-cases.html</a></div></div><div dir="ltr"><br /></div></div><div>USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic CJD’s). ...tss</div></div><br /></div><div dir="ltr">CANADA CJD 2011<br /></div></div><div dir="ltr"><br /></div><div dir="ltr"><div><div>Risk.49: Creutzfeldt-Jakob Disease in Canada, 1998–2009</div><div><br /></div><div>Zheng Wang,1,† Gerard Jansen,1, 2 Elina Olsen,1 Stacy Sabourin,1 Rolande D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael Coulthart1</div><div><br /></div><div>1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital; Ottawa, ON Canada; 3Brain Research Centre; University of British Columbia; Vancouver, BC Canada†Presenting author; Email: <span dir="ltr">zheng.wang@phac-aspc.gc.ca</span></div><div><br /></div><div>Background. Creutzfeldt-Jakob Disease (CJD) is a fatal, transmissible neurodegenerative disease with sporadic, genetic and acquired forms. In 1998, Canada launched comprehensive national CJD surveillance to assess the characteristics of CJD in Canada and its risks to the health of Canadians. This study describes the broad characteristics of CJD in Canada from 1998–2009.</div><div><br /></div><div>Methods. Case ascertainment was based on internationally accepted criteria. Demographic information and risk-factor data were collected by standardized questionnaire and medical chart review. Poisson regression, descriptive analysis, and case investigation were employed.</div><div><br /></div><div>Results. A total of 453 CJD deaths in Canadian residents were registered from 1998–2009. Four hundred and fifteen (92%) were sporadic (sCJD), 33 (7%) were genetic and five (1%) were acquired. Average annual sCJD mortality was 1.1 per million population, increasing gradually from 0.9 in 1999 to 1.4 in 2009 (P = 0.27). All provinces saw average annual mortalities ranging from 1.0 to 1.5 (P = 0.85), except three territories where population is small (~25,000 to ~45,000), sCJD occurred equally in both genders at 1.1. sCJD was rare under 50 years of age with only 11 cases identified (2.7%). Mortality increased after 50 and peaked at 8 per million in the 70–74 age group. Median age at death was 69 and median duration of illness was 4 months. Genetic TSE accounted for 33 deaths: 19 were GSS (P102L: 5, D202N: 2, P105T: 2, Q217R:1, A117V: 1, unknown mutation: 8); 13 were familial CJD (E200K: 9, D178N: 2, V203I: 1, V189I:1); one was FFI (D178N). Median age for genetic TSE was 59 and median duration of illness was 27 months. For the five acquired cases of CJD, four were associated with dura mater procedures (3 Lyodura, 1 Tutoplast) and were identified from 1998–2003 in patients aged 14–59. Investigation indicated the infections possibly occurred from 1981–1992 with incubation times <span dir="ltr">from 10–16</span> years. One biochemically and neuropathologically confirmed variant CJD death occurred in 2002 in a person under 40 years old, likely acquired overseas.</div><div><br /></div><div>Discussion and Conclusion. Characteristics of CJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians. The finding of four dura matter associated CJD cases and one imported vCJD case in Canada demonstrate risks to Canadians from acquired CJD exist. Continued surveillance for iatrogenic risks and novel forms of CJD is warranted.</div><div><br /></div><div><a href="http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf" style="color: #196ad4;">www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf</a></div></div><div><br /></div></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2016/08/creutzfeldt-jakob-disease-usa-2015.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2016/08/creutzfeldt-jakob-disease-usa-2015.html</a></div></div><br /></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div><div>CANADA CJD UPDATE 2011</div><div><br /></div><div>CJD Deaths Reported by CJDSS1, <span dir="ltr">1994-20112</span> As of January 31, 2011</div><div><br /></div><div>3. Final classification of 49 cases from 2009, 2010, 2011 is pending.</div><div><br /></div><div>snip...</div><div><br /></div><div><a href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf" style="color: #196ad4;">www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf</a></div><div><br /></div><div><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html" rel="nofollow" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html</a><br /></div></div><div><br /></div><div dir="ltr"><div><span style="background-color: #fff3db; color: #29303b;">Saturday, March 5, 2011</span><br style="background-color: #fff3db; color: #29303b;" /><br style="background-color: #fff3db; color: #29303b;" /><span style="background-color: #fff3db; color: #29303b;">MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA</span><br style="background-color: #fff3db; color: #29303b;" /><br style="background-color: #fff3db; color: #29303b;" /><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html" rel="nofollow" style="background-color: #fff3db; color: #473624;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html</a></div><br /></div><div dir="ltr"><div><div dir="ltr">CANADA CJD 2007</div><div><br /></div><div>Creutzfeldt-Jakob Disease Surveillance System (CJD-SS)</div><div><br /></div><div>Referrals of Suspected CJD Reported by CJD-SS(1), 1997-2007(2)</div><div><br /></div><div>Year of Reporting Numbers of Referrals</div><div><br /></div><div>1997 4 </div><div><br /></div><div>1998 43 </div><div><br /></div><div>1999 63 </div><div><br /></div><div>2000 82 </div><div><br /></div><div><span dir="ltr">2001 101</span> </div><div><br /></div><div><span dir="ltr">2002 103</span> </div><div><br /></div><div>2003 75 </div><div><br /></div><div>2004 89 </div><div><br /></div><div>2005 97 </div><div><br /></div><div>2006 78 </div><div><br /></div><div>2007 88</div><div><br /></div><div>Total 823</div><div><br /></div><div>1CJD-SS began in April 1998 2Data before April 1998 are retrospective and partial, data from 1999 to 2005 are complete, and data for 2006 and 2007 are provisional As of December 1, 2007</div><div><br /></div><div><a href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/refs1207_e.pdf" style="color: #196ad4;">www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/refs1207_e.pdf</a></div><div><br /></div><div><a fg_scanned="1" href="http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html" style="color: #196ad4;">www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html</a></div><div><br /></div><div><a fg_scanned="1" href="https://cjdmadcowbaseoct2007.blogspot.com/2008/01/cjd-human-tse-report-uk-usa-canada-and.html" rel="nofollow" style="color: #196ad4;" target="_blank">cjdmadcowbaseoct2007.blogspot.com/2008/01/cjd-human-tse-report-uk-usa-canada-and.html</a></div></div><div><br /></div></div><div><div>A simple look at sporadic CJD statistics in EU countries with BSE will reveal this;</div><div><br /></div><div>CANADA 2 IN 94 COMPARED TO 30 IN 2002</div><div><br /></div><div>FRANCE 35 IN 93 COMPARED TO 102 IN 2003</div><div><br /></div><div>GERMANY 21 IN 93 COMPARED TO 112 IN 2003</div><div><br /></div><div>ITALY 27 IN 93 TO COMPARED TO 75 IN 2003</div><div><br /></div><div>UK 37 IN 93 COMPARED TO 74 IN 2003</div><div><br /></div><div>USA (UNKNOWN...TSS)</div><div><br /></div><div><a fg_scanned="1" href="http://www.eurocjd.ed.ac.uk/sporadic.htm" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.eurocjd.ed.ac.uk/sporadic.htm</a><br /></div><div><br /></div><div><a fg_scanned="1" href="https://www.eurocjd.ed.ac.uk/data" style="color: #196ad4;">www.eurocjd.ed.ac.uk/data</a></div><div><br /></div><div><a fg_scanned="1" href="https://www.eurocjd.ed.ac.uk/data_tables" style="color: #196ad4;">www.eurocjd.ed.ac.uk/data_tables</a><br /></div><div><br /></div><div>snip...</div><div><br /></div><div>IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ;</div><div><br /></div><div>However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52).</div><div><br /></div><div>IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;</div><div><br /></div><div>Canada from 2 to 25</div><div><br /></div><div>France from 35 to 108</div><div><br /></div><div>Germany 21+ to 96</div><div><br /></div><div>Italy 27 to 76</div><div><br /></div><div><a fg_scanned="1" href="http://www.eurocjd.ed.ac.uk/sporadic.htm" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.eurocjd.ed.ac.uk/sporadic.htm</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://www.eurocjd.ed.ac.uk/data" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.eurocjd.ed.ac.uk/data</a><br /></div><div><br /></div><div><a fg_scanned="1" href="https://www.eurocjd.ed.ac.uk/data_tables" style="color: #196ad4;">www.eurocjd.ed.ac.uk/data_tables</a></div><div><br /></div><div><a fg_scanned="1" href="http://prionunitusaupdate.blogspot.com/2010/01/human-prion-diseases-in-united-states.html" rel="nofollow" style="color: #196ad4;" target="_blank">prionunitusaupdate.blogspot.com/2010/01/human-prion-diseases-in-united-states.html</a><br /></div></div><div><br /></div></div><div dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div>Government officials misled the public about the “mysterious” New Brunswick neurological disease, and we have the documents to prove it Here are the 10 biggest takeaways after pouring over thousands of pages of internal government documents obtained through access to information</div><div><br /></div><div>JANUARY 23, 2023</div><div><br /></div><div>ON THE MONDAY SHOW BY SARAH LAWRYNUIK</div><div><br /></div><div><a fg_scanned="1" href="https://www.canadaland.com/new-brunswick-mystery-illness-documents/" rel="nofollow" style="color: #196ad4;" target="_blank">https://www.canadaland.com/new-brunswick-mystery-illness-documents/</a></div><div><br /></div><div>WEDNESDAY, OCTOBER 27, 2021 </div><div><br /></div><div>New Brunswick October 27, 2021 - Health minister availability Report on cluster of unknown neurological disorders update </div><div><br /></div><div><a fg_scanned="1" href="https://cjdusa.blogspot.com/2021/10/new-brunswick-october-27-2021-health.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://cjdusa.blogspot.com/2021/10/new-brunswick-october-27-2021-health.html</a></div><div><br /></div><div><a fg_scanned="1" href="http://creutzfeldt-jakob-disease.blogspot.com/2022/09/neuropathology-of-8-patients-of-new.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://creutzfeldt-jakob-disease.blogspot.com/2022/09/neuropathology-of-8-patients-of-new.html</a></div><div><br /></div><div><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2021/03/new-brunswick-monitoring-more-than-40.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/03/new-brunswick-monitoring-more-than-40.html</a></div><div><br /></div><div dir="ltr"><div><div><div>WEDNESDAY, NOVEMBER 30, 2022 </div><div><br /></div><div>USDA Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, Testing and Surveillance 2022 A Review of History </div><div><br /></div></div><div><a fg_scanned="1" href="https://animalhealthreportpriontse.blogspot.com/2022/11/usda-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2022/11/usda-bovine-spongiform-encephalopathy.html</a><br /></div></div><div><br /></div><div dir="ltr"><div dir="ltr">FRIDAY, DECEMBER 02, 2022 <br /></div><div dir="ltr"><br /></div><div dir="ltr">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update<br /></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/12/creutzfeldt-jacob-disease-cjd-tse-prion.html</a><br /></div><div dir="ltr"><br /></div></div><div dir="ltr"><div><div style="font-size: 13.3333px; text-align: justify;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="https://www.nature.com/articles/srep11573" rel="nofollow" style="color: blue;" target="_blank">https://www.nature.com/articles/srep11573</a> </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***thus questioning the origin of human sporadic cases. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">=============== </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***thus questioning the origin of human sporadic cases*** </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">=============== </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">============== </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">PRION 2015 CONFERENCE</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow" style="color: blue;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a><br /></div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: blue;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">PRION <span dir="ltr">2016 TOKYO</span></div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Saturday, April 23, 2016</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">SCRAPIE <span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential 2016</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Prion. 10:S15-S21. 2016 ISSN: <span dir="ltr">1933-6896</span> printl 1933-690X online</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Taylor & Francis</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><span dir="ltr">WS-01</span>: Prion diseases in animals and zoonotic potential</div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow" style="color: blue;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br /></div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></div></div><div dir="ltr" style="font-size: 13.3333px; text-align: justify;"><div><div><br /></div><div dir="ltr"><div><div>TUESDAY, DECEMBER 21, 2021 </div><div><br /></div><div>OIE-WAHIS CANADA atypical BSE type H Bovine spongiform encephalopathy<br /></div><div><br /></div><div><a fg_scanned="1" href="https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html" rel="nofollow" style="color: blue;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2021/12/oie-wahis-canada-atypical-bse-type-h.html</a></div></div><div><br /></div><div><div>SATURDAY, DECEMBER 18, 2021 </div><div><br clear="none" /></div><div>CFIA Canada Alberta Laboratory detection of atypical bovine spongiform encephalopathy<br clear="none" /></div><div><br clear="none" /></div><div><a fg_scanned="1" href="https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html" rel="nofollow" shape="rect" style="color: blue;" target="_blank">https://bovineprp.blogspot.com/2021/12/cfia-canada-alberta-laboratory.html</a></div></div></div><div><br /></div></div><div><div>I remember another famous quote;</div><div><br /></div><div>Alberta Premier Ralph Klein has taken aim at the owner of the province's infamous mad cow, saying a "self-respecting" rancher would not have taken the animal to slaughter but instead would have simply "shot, shovelled and shut up."<br /></div><div><br /></div><div><a fg_scanned="1" href="https://www.theglobeandmail.com/news/national/farmer-should-have-covered-up-mad-cow-klein-says/article1166750/" style="color: #196ad4;">www.theglobeandmail.com/news/national/farmer-should-have-covered-up-mad-cow-klein-says/article1166750/</a></div><div><br /></div><div>OIE ATYPICAL BSE 2019</div><div><br /></div><div><div><div>the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided. The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.</div><div><br /></div><div>4. Definitions of meat-and-bone meal (MBM) and greaves</div><div><span style="background-color: transparent;"><br /></span></div><div><span style="background-color: transparent;">snip...</span><br /></div><div><br /></div><div>REFERENCES</div><div><br /></div><div>SNIP...END SEE FULL TEXT;</div><div><br /></div><div><a href="http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf</a><br /></div><div><br /></div><div>As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.</div><div><br /></div></div><div><a href="http://%20https//www.oie.int/.../AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http:// https://www.oie.int/.../AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf</a></div><div><br /></div><div><div class="ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626cxmmr5t8 ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626oygrvhab ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626hcukyx3x ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626c1et5uql ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626o9v6fnle" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;">TUESDAY, SEPTEMBER 07, 2021</div></div><div class="ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626cxmmr5t8 ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626oygrvhab ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626hcukyx3x ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626c1et5uql ydp397e9dcyiv8679458604ydp8e84daa6yiv2687684626o9v6fnle" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;">Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom</div><div style="font-family: inherit;"><br /></div><div style="font-family: inherit;"><a fg_scanned="1" href="https://bse-atypical.blogspot.com/2021/09/atypical-bovine-spongiform.html" rel="nofollow" style="color: #196ad4; font-family: inherit;" target="_blank">https://bse-atypical.blogspot.com/2021/09/atypical-bovine-spongiform.html</a></div></div></div><div><div><div><br /></div><div data-setdir="false" dir="ltr"><div>BSE in North America</div><div><br /></div><div>Through August 2018, BSE surveillance has identified 26 cases in North America: </div><div><br /></div><div>6 BSE cases in the United States and 20 in Canada. </div><div><br /></div><div>Of the 6 cases identified in the United States, one was born in Canada; of the 20 cases identified in Canada, one was imported from the United Kingdom (see graph below).</div><div><br /></div><div><a fg_scanned="1" href="https://www.cdc.gov/prions/bse/bse-north-america.html" style="color: #196ad4;">www.cdc.gov/prions/bse/bse-north-america.html</a></div></div><div><br /></div><div>BSE Cases Identified in Canadian-born Cattle</div><div><br /></div><div>Update: February 12, 2015 a New Case of BSE Detected in Canada</div></div><div><br /></div><div>The Canadian Food Inspection Agency (CFIA) external icon announced the confirmation of another bovine spongiform encephalopathy (BSE) in a beef cow from Alberta born in March 2009. See the CFIAexternal icon notice.</div></div><div><br /></div><div>Based on the known or most likely year of birth, an average of 1.4 cases of BSE occurred among the group of animals born each year in Canada from 1991 through 2004. The highest reported number of cases by birth year in a single year, 3 BSE cases, occurred in 2000, 2001 and 2002. The most recently reported case extends the period of BSE transmission in Canada through at least the early half of 2009.</div><div><br /></div><div><a fg_scanned="1" href="https://www.cdc.gov/prions/bse/case-canadian-cattle.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://www.cdc.gov/prions/bse/case-canadian-cattle.html</a></div></div><div><br /></div><div><a fg_scanned="1" href="https://www.cdc.gov/prions/bse/bse-north-america.html" style="color: #196ad4;">www.cdc.gov/prions/bse/bse-north-america.html</a></div><div><br /></div><div>MONDAY, NOVEMBER 30, 2015 </div><div><br /></div><div>Report on the Investigation of the Nineteenth Case of Bovine Spongiform Encephalopathy (BSE) in Canada November 2015<br /></div><div><br /></div><div><a fg_scanned="1" href="https://bovineprp.blogspot.com/2015/11/report-on-investigation-of-nineteenth.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://bovineprp.blogspot.com/2015/11/report-on-investigation-of-nineteenth.html</a><br /></div><div><br /></div><div>FRIDAY, FEBRUARY 20, 2015 </div><div><br /></div><div>A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)</div><div> </div><div><a fg_scanned="1" href="https://transmissiblespongiformencephalopathy.blogspot.com/2015/02/a-bse-canadian-cow-mad-cow-update.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2015/02/a-bse-canadian-cow-mad-cow-update.html</a><br /></div><div><br /></div><div><div>SATURDAY, FEBRUARY 14, 2015 </div><div><br /></div><div>Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta </div><div><br /></div><div>Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta The Canadian Food Inspection Agency (CFIA) has confirmed bovine spongiform encephalopathy (BSE) in a beef cow from Alberta. No part of the animal's carcass entered the human food or animal feed systems.</div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The Government of Canada is committed to protecting human and animal health and takes the management of BSE very seriously. Immediately upon confirmation of this case, the CFIA launched an investigation and is working closely with provincial and industry partners.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">BSE is a progressive, fatal neurological disease in cattle. Canada's last confirmed BSE case was reported in 2011. This latest case was detected through the national BSE surveillance program, which continues to play an important role in Canada's strategy to manage BSE.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">As part of the investigation, the CFIA is seeking to confirm the age of the animal, its history and how it became infected. The investigation will focus in on the feed supplied to this animal during the first year of its life. The Agency will also trace out all animals of equivalent risk. Equivalent risk animals will be ordered destroyed and tested for BSE.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Canada remains a "controlled BSE risk" country, as recognized by the World Organisation for Animal Health (OIE). Accordingly, this case should not affect current exports of Canadian cattle or beef.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The case will be reported to the OIE, in line with Canada's international obligations and our commitment to transparency. It will be reported on the CFIA website, as part of the Agency's monthly reportable diseases update.</span></div><div><br /></div><div><a fg_scanned="1" href="http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/cfia-confirms-bse-in-alberta/eng/1423797248015/1423797327027" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/cfia-confirms-bse-in-alberta/eng/1423797248015/1423797327027</a><br /></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Transcript - Briefing (February 13, 2015) Date/Date: February 13, 2015 4:00 p.m.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Location/Endroit: Teleconference, Ottawa, Ontario</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Principal(s)/Principaux:</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Denis Schryburt, Media Relations Officer, Canadian Food Inspection Agency Paul Mayers, Vice-President, Policy and Programs, CFIA Dr. Martine Dubuc, Vice-President, Science, CFIA, and Delegate for Canada for the World Organization for Animal Health Nathalie Durand, Agriculture and Agri-Food Canada Subject/Sujet: The Canadian Food Inspection Agency Holds a Technical Briefing to Provide More Information on a BSE (Bovine Spongiform Encephalopathy) Find in Alberta.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Operator: Good afternoon, ladies and gentlemen. Bonjour, mesdames et messieurs. Welcome to the Canadian Food Inspection Agency's technical briefing on Bovine Spongiform Encephalopathy in Alberta. Bienvenue à la séance d'information technique sur le cas d'Encéphalopathie spongiforme bovine en Alberta. I would like to turn the meeting over to the technical briefing operator, Mr. Denis Schryburt. J'aimerais maintenant céder la parole au modérateur de cette séance, M. Denis Schryburt. À vous la parole, M. Schryburt. Please go ahead, sir.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Denis Schryburt: Thank you very much. Good afternoon and thank you for joining us today. My name is Denis Schryburt, Media Relations Officer at the Canadian Food Inspection Agency, and I'll be moderating today's technical briefing. I will begin by introducing our speakers who will make a short statement in both official languages and then open it up to the media for questions.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Our first speaker is Paul Mayers, Vice-President, Policy and Programs, followed by Dr. Martine Dubuc, Vice-President, Science, and Delegate for Canada for the World Organization for Animal Health.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Bonjour et merci de vous joindre à nous aujourd'hui. Mon nom est Denis Schryburt, agent des Relations avec les médias à l'Agence canadienne d'inspection des aliments, et j'animerais la séance d'information technique aujourd'hui. Je vais débuter par présenter nos porte-parole qui feront une brève déclaration dans les deux langues officielles et ensuite répondre à vos questions.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Notre premier porte-parole est Paul Mayers, vice-président, Politique et Programmes, suivi par Martine Dubuc, vice-présidente, science, et la délégué pour Canada pour l'Organisation mondiale de la santé animale.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">I will now invite Paul Mayers to make a brief statement in English. Mr. Mayers.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Paul Mayers: Thank you, Denis. Good afternoon, everyone, and thank you for calling in today. We'd like to provide some information today on a developing animal health situation. The Canadian Food Inspection Agency has confirmed Bovine Spongiform Encephalopathy, also known as BSE, in a beef cow from Alberta.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">First of all, no part of the animal's carcass entered the human food or animal feed system. Canada's suite of internationally recognized safeguards effectively protects the safety of food and animal feed. There is no risk to food safety.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The Government of Canada is committed to protecting human and animal health and takes the management of BSE very seriously. Immediately upon confirmation of this case, the CFIA launched an investigation and is working closely with provincial and industry partners. This investigation will follow the well-developed procedures we've employed in response to previous BSE cases.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Canada's last confirmed BSE case was reported in 2011. This latest case in Alberta was detected through the National BSE Surveillance Program, which is a program that continues to play an important part in Canada's strategy to manage BSE. The fact that we continue to see very high levels of producer participation in the surveillance program underscores the commitment present throughout the cattle and beef sectors to responsibly manage BSE.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The detection of a small number of additional BSE cases is not unexpected in the context of the 30,000 samples we take annually, as Canada continues our ongoing management of this disease.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">As has been our practice for CFIA investigations of BSE cases, the Agency is seeking to confirm the age of the animal, its history and how it may have become infected. We're also working to trace out all animals of equivalent risk such as the animals that may have been exposed to the same feed as the infected animal in the first year of its life. Equivalent risk animals will be ordered destroyed, and they will be tested for BSE.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA will notify the World Organization for Animal Health, also known as the OIE, in line with Canada's international obligations and our commitment to transparency.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">This finding should not affect Canada's status as a controlled BSE risk country as recognized by the OIE. Canada continues to effectively manage BSE through a series of integrated safeguards designed to protect both human and animal health. These include prohibiting risk materials from entering the human food and animal feed chains and testing cattle for BSE.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Again, the CFIA is strongly committed to protecting animal health. Our investigation is underway, and we are mobilizing all necessary resources to address this situation.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Thank you.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Denis Schryburt: Thank you, Mr. Mayers. Et maintenant j'invite Martine Dubuc à faire une déclaration en français. Mme Dubuc, s'il-vous-plaît.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Dr Martine Dubuc: Merci. Bonjour à tous, et merci de vous être joints à la téléconférence aujourd'hui. Nous aimerions vous donner aujourd'hui des renseignements sur une situation de santé animale en évolution.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">L'Agence canadienne d'inspection des aliments a confirmé un cas d'Encéphalopathie spongiforme bovine, aussi connu sous le nom d'ESB, chez une vache de boucherie provenant de l'Alberta.</span></div><div><br /></div><div> </div><div><span style="background-color: transparent;">snip... </span><br /></div><div><br /></div><div>-30-</div><div><br /></div><div><a fg_scanned="1" href="http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/transcript/eng/1423881622681/1423881709500" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/transcript/eng/1423881622681/1423881709500</a><br /></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Timeline of Events: Bovine Spongiform Encephalopathy – Alberta – February 2015</span><br /></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 13</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The Canadian Food Inspection Agency (CFIA) holds a technical briefing related to the Bovine Spongiform Encephalopathy (BSE) positive case found in Alberta.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 12</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA notifies key trading partners of the new finding and posts the information on its website.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 11</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA confirms BSE in one beef cow in Alberta.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA continue to gather information on the animal's herd of origin and to trace the suspect animal's offspring.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 10</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA gather preliminary information on the suspect animal's herd of origin.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 9</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The CFIA receives a tissue sample from the affected animal and begins confirmatory testing at its laboratory in Lethbridge.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">CFIA inspectors follow up at the farm, obtain additional samples, discuss next steps with producer and begin the investigation.</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">February 7</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">The province of Alberta reports a non-negative test for BSE to the CFIA.</span></div><div><br /></div><div><a fg_scanned="1" href="http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/cfia-confirms-bse-in-alberta/timeline/eng/1423937283891/1423937285813" style="color: #196ad4;">www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/bse/cfia-confirms-bse-in-alberta/timeline/eng/1423937283891/1423937285813</a><br /></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">Friday, February 20, 2015</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;">A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015)</span></div><div><br /></div><div><span style="background-color: transparent; font-size: 10pt;"><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2015/02/a-bse-canadian-cow-mad-cow-update.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2015/02/a-bse-canadian-cow-mad-cow-update.html</a><br /></span></div><div><br /></div><div><a fg_scanned="1" href="https://bovineprp.blogspot.com/2015/02/oie-bovine-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://bovineprp.blogspot.com/2015/02/oie-bovine-spongiform-encephalopathy.html</a><br /></div><div><br /></div><div><div>Saturday, February 14, 2015</div><div><br /></div><div>Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta</div><div><br /></div><div><a fg_scanned="1" href="http://madcowusda.blogspot.com/2015/02/canadian-food-inspection-agency.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowusda.blogspot.com/2015/02/canadian-food-inspection-agency.html</a></div></div><div><div><br /></div><div>Current as of: 2015-01-31</div><div><br /></div><div>Sheep flocks and/or goat herds confirmed to be infected with classical scrapie in Canada in 2015 Date confirmed Location Animal type infected January 5 Ontario Goat</div><div><br /></div><div></div><a fg_scanned="1" href="http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/2015/scrapie-2015-/eng/1423162035296/1423162036030" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/2015/scrapie-2015-/eng/1423162035296/1423162036030</a><div><br /></div><div></div><a fg_scanned="1" href="http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/scrapie/eng/1329723409732/1329723572482" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/animals/terrestrial-animals/diseases/reportable/scrapie/eng/1329723409732/1329723572482</a><div><br /></div><div>Tuesday, February 10, 2015</div><div><br /></div><div>Alberta Canada First case of chronic wasting disease found in farm elk since 2002</div><div><br /></div><div><a fg_scanned="1" href="http://chronic-wasting-disease.blogspot.com/2015/02/alberta-canada-first-case-of-chronic.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://chronic-wasting-disease.blogspot.com/2015/02/alberta-canada-first-case-of-chronic.html</a><br /></div></div><div><br /></div><div><div>Tuesday, May 21, 2013</div><div><br /></div><div>Canada, USA, Bad feed, mad cows: Why we know three BSE cases had a common origin and why the SSS policy is in full force $$$</div><div><br /></div><div><a fg_scanned="1" href="http://madcowusda.blogspot.com/2013/05/canada-usa-bad-feed-mad-cows-why-we.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowusda.blogspot.com/2013/05/canada-usa-bad-feed-mad-cows-why-we.html</a><br /></div></div><div><br /></div><div><div>Thursday, January 17, 2013</div><div><br /></div><div>Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection</div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2013/01/canada-us-agree-on-animal-disease.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2013/01/canada-us-agree-on-animal-disease.html</a></div><div><br /></div><div>Sunday, December 2, 2012</div><div><br /></div><div>CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’</div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2012/12/canada-19-cases-of-mad-cow-disease.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2012/12/canada-19-cases-of-mad-cow-disease.html</a></div><div><br /></div><div>Tuesday, October 2, 2012</div><div><br /></div><div>Canadian veterinarian fined after approving banned BSE high risk cattle for export to U.S.A.</div><div><br /></div><div><a fg_scanned="1" href="http://madcowusda.blogspot.com/2012/10/canadian-veterinarian-fined-after.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowusda.blogspot.com/2012/10/canadian-veterinarian-fined-after.html</a></div><div><br /></div><div>Monday, April 23, 2012</div><div><br /></div><div>BOVINE SPONGIFORM ENCEPHALOPATHY BSE CJD TSE PRION DISEASE UPDATE CANADA 2012</div><div><br /></div><div><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/bovine-spongiform-encephalopathy-bse.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/bovine-spongiform-encephalopathy-bse.html</a></div></div><div><br /></div><div><div>Thursday, February 10, 2011</div><div><br /></div><div>TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31</div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2011/02/transmissible-spongiform-encephalopathy.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2011/02/transmissible-spongiform-encephalopathy.html</a><br /></div><div><br /></div><div><div>Friday, March 4, 2011 </div><div><br /></div><div>Alberta dairy cow found with mad cow disease</div><div><br /></div><div><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/alberta-dairy-cow-found-with-mad-cow.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/alberta-dairy-cow-found-with-mad-cow.html</a><br /></div></div><div><br /></div><div>Wednesday, August 11, 2010</div><div><br /></div><div>REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA</div><div><br /></div><div><a fg_scanned="1" href="http://bse-atypical.blogspot.com/2010/08/report-on-investigation-of-sixteenth.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://bse-atypical.blogspot.com/2010/08/report-on-investigation-of-sixteenth.html</a></div><div> </div><div>Thursday, August 19, 2010</div><div><br /></div><div>REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA</div><div><br /></div><div><a fg_scanned="1" href="http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html</a></div><div><br /></div></div><div><span style="background-color: transparent; font-size: 10pt;">Published Date: 2010-03-11 19:00:03 </span><br /></div></div><div><div><br /></div><div>Subject: PRO/AH/EDR> BSE, bovine - Canada: (AB) Archive Number: 20100311.0792</div><div><br /></div><div>BSE, BOVINE - CANADA: (ALBERTA) </div><div><br /></div><div>******************************* </div><div><br /></div><div>A ProMED-mail post <http://www.promedmail.org> ProMED-mail is a program of the International Society for Infectious Diseases <http://www.isid.org></div><div><br /></div><div>[1] Date: 25 Feb 2010 Source: Canadian Food Inspection Agency [edited]</div><div><br /></div><div> <<a fg_scanned="1" href="http://www.inspection.gc.ca/english/anima/disemala/rep/2010bseesbe.shtml" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/english/anima/disemala/rep/2010bseesbe.shtml</a>> </div><div><br /></div><div>Bovine spongiform encephalopathy (BSE) cases have been confirmed in Canada in 2010. BSE is a reportable disease under the "Health of Animals Regulations." This means that all suspected cases must be reported to the CFIA. The following table lists individual animals confirmed to be infected with BSE in Canada in 2010, updated 28 Feb 2010:</div><div><br /></div><div>Date confirmed: 25 Feb 2010 Location: Alberta Animal type infected: Beef cow Age of animal: 72 months</div><div><br /></div><div>-- Communicated by: Terry S. Singeltary Sr. <flounder9@verizon.net></div><div><br /></div><div>****** [2] Date: 11 Mar 2010 Source: Meat Trade News [edited]</div><div><br /></div><div> <http://www.meattradenewsdaily.co.uk/news/100310/canada___case_of_bse_mad_cow_disease_in__year_old_cow.aspx> </div><div><br /></div><div>The Badger has learned a new case of BSE was discovered 2 weeks ago, but the public was not informed as part of the government's new communication strategy. The decision not to announce new cases of BSE was made in August 2009, and the public was informed by the Canadian Food Inspection Agency (CFIA) online.</div><div><br /></div><div>"The CFIA is committed to providing all stakeholders, including the general public, media and trading partners, with timely information about disease detections in farmed animals. As such, we have revised how we report online for disease detections in farmed animals to provide a more comprehensive view of Canada's animal health status. All confirmed cases of federally reportable diseases in farmed animals will be centrally located on our website. This information will be updated monthly," explained CFIA spokesperson Jenn Gearey.</div><div><br /></div><div>The new communication strategy means journalists will not be notified when any new cases of BSE are discovered.</div><div><br /></div><div>The latest finding of BSE -- Canada's 17th domestic case -- was announced to industry stakeholders, such as processors, on 25 Feb 2010, but not to the media or general public. And while the CFIA claims its reportable diseases page will be updated monthly, no new information has been posted since 31 Jan 2010.</div><div><br /></div><div>The infection was detected through the national surveillance program in a 6-year-old black angus cow in the same general area of Alberta, home to most of Canada's BSE activity.</div><div><br /></div><div>The last case discovered in Canada was in May 2009, the only occurrence that year. In 2008, there were 4 incidents; in 2007, there were 3, and in 2006, there were 5 cases of BSE.</div><div><br /></div><div>Canada's international risk status has not been affected by the latest case.</div><div><br /></div><div>-- Communicated by: Terry S. Singeltary Sr. <flounder9@verizon.net></div><div><br /></div><div>****** [3] Date: 10 Mar 2010 Source: Reuters Canada [edited] <http://ca.reuters.com/article/domesticNews/idCATRE6295A420100310> </div><div><br /></div><div>Canada has confirmed its 17th case of mad cow disease, a finding that will delay any upgrade to its international risk status by one year, a top industry official said on Wednesday [10 Mar 2010].</div><div><br /></div><div>The animal was born in February 2004, making it Canada's latest-born case of bovine spongiform encephalopathy (BSE). The new case pushes back the earliest date for an upgrade to Canada's controlled risk status from the World Organization for Animal Health (OIE) to 2016, said Ted Haney, president of the Canada Beef Export Federation.</div><div><br /></div><div>A country cannot apply to upgrade to negligible status sooner than 11 years after the latest-born case of BSE. The process then takes about one year.</div><div><br /></div><div>Canada, along with many other countries with controlled risk status from the OIE, can ship beef as long as it meets conditions such as disease surveillance.</div><div><br /></div><div>The infected animal, which has been slaughtered, has not affected trade, Haney said.</div><div><br /></div><div>The 2003 discovery of the 1st case of mad cow disease on a Canadian farm caused many countries to halt imports of Canadian beef. Most markets have since reopened, but the cattle industry remains in a slump due to other factors such as a strong Canadian dollar.</div><div><br /></div><div>Mad cow disease is believed to be spread when cattle eat protein rendered from the brains and spines of infected cattle or sheep. Canada banned that practice in 1997.</div><div><br /></div><div>The Canadian Food Inspection Agency tightened feed rules further in 2007 and said the moves should help eliminate the disease nationally within a decade, although the agency cautioned it still expected to discover the occasional new case.</div><div><br /></div><div>CFIA spokeswoman Julie LePage confirmed the 17th case but could not provide details of the new case.</div><div><br /></div><div>The CFIA notified cattle industry officials of the new case late last month [February 2010] but did not issue a news release, Haney said.</div><div><br /></div><div>[Byline: Rod Nickel]</div><div><br /></div><div>-- Communicated by: ProMED-mail <promed@promedmail.org></div><div><br /></div><div>[While it may be CFIA's decision on how to notify the public, it may not be in the best interest as far as public relations are concerned. Also, the Reuters article does not make much sense. How can the OIE status change be delayed to 2016 if it is 11 years from the last case? From the CFIA website [article [1] in this posting], it appears the animal was confirmed positive on 25 Feb 2010. Thus, 11 years would be 2021. - Mod.TG] See Also 2009 ---- BSE, bovine - Canada (AB) 20090517.1841 BSE, bovine, 2008 - Canada: (AB, BC) CFIA reports 20090417.1459 2008 ---- BSE, bovine - Canada (04): (BC) 20081119.3648 BSE, bovine - Canada (03): (AB) 20080819.2580 BSE, bovine - Canada (02): (BC) 20080623.1941 BSE, bovine - Canada (AB) 20080226.0786 2007 ---- BSE, bovine - Canada (AB) (03) 20071218.4076 BSE, bovine - Canada (BC) 20070502.1430 BSE, bovine - Canada (AB) (02) 20070308.0813 BSE, bovine - Canada (AB) 20070208.0499 2006 ---- BSE, bovine - Canada (AB)(06) 20061227.3621 BSE, bovine - Canada (AB)(05) 20060825.2413 BSE, bovine - Canada (AB)(04) 20060823.2384 ...................................................tg/msp/lm</div><div><br /></div><div>*##########################################################* </div></div><div><br /></div><div><a fg_scanned="1" href="https://promedmail.org/promed-post/?id=20100311.0792" rel="nofollow" style="color: #196ad4;" target="_blank">https://promedmail.org/promed-post/?id=20100311.0792</a><br /></div><div><br /></div><div><div>Increased Atypical Scrapie Detections</div><div><br /></div><div>Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.</div><div><br /></div><div><a href="http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://gain.fas.usda.gov/Recent%20GAIN%20Publications/This%20Week%20in%20Canadian%20Agriculture%20%20%20%20%20Issue%2028_Ottawa_Canada_11-6-2009.pdf</a></div></div><div><br /></div><div><div>Published Date: 2008-08-19 11:00:29 Subject: PRO/AH/EDR> BSE, bovine - Canada (03): (AB) Archive Number: 20080819.2580</div><div><br /></div><div>BSE, BOVINE - CANADA (03): (ALBERTA) </div><div><br /></div><div>************************************ </div><div><br /></div><div>A ProMED-mail post <http://www.promedmail.org> ProMED-mail is a program of the International Society for Infectious Diseases <http://www.isid.org></div><div><br /></div><div>[1] Date: Fri 15 Aug 2008 Source: Canadian Food Inspection Agency (CFIA) [edited]</div><div><br /></div><div> < <a fg_scanned="1" href="http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/ab2008/14notavie.shtml" rel="nofollow" style="color: #196ad4;" target="_blank">http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/ab2008/14notavie.shtml</a> > </div><div><br /></div><div>BSE [bovine spongiform encephalopathy] case confirmed in Alberta</div><div><br /></div><div>----------------------------------------------------------------</div><div><br /></div><div>The Canadian Food Inspection Agency (CFIA) has confirmed bovine spongiform encephalopathy (BSE) in a 6-year-old beef cow from Alberta. No part of the animal's carcass entered the human food or animal feed systems.</div><div><br /></div><div>The animal's birth farm has been identified, and an investigation is underway. The CFIA is tracing the animal's herdmates at the time of birth and examining possible sources of infection. The age and location of the infected animal are consistent with previous cases detected in Canada.</div><div><br /></div><div>This case was detected through the national BSE surveillance program, which has been highly successful in demonstrating the low level of BSE in Canada. The program continues to play an important role in Canada's strategy to manage BSE.</div><div><br /></div><div>Canada remains a Controlled Risk country for BSE, as recognized by the World Organisation for Animal Health (OIE). Accordingly, this case should not affect exports of Canadian cattle or beef.</div><div><br /></div><div>For information: Canadian Food Inspection Agency Media relations: 613-228-6682</div><div><br /></div><div>-- Communicated by: Terry S Singeltary Sr <flounder9@verizon.net></div><div><br /></div><div>****** [2] Date: Sat 16 Aug 2008 Source: Montana News Station, Associated Press (AP) report [edited]</div><div><br /></div><div> <http://www.montanasnewsstation.com/Global/story.asp?S=8851685> </div><div><br /></div><div>New mad cow case found in Canada </div><div><br /></div><div>--------------------------------</div><div><br /></div><div>A new case of mad cow disease was confirmed in Canada, its 14th case since 2003.</div><div><br /></div><div>Government inspectors say no part of the animal entered the human food system. The Canadian Food Inspection Agency (CFIA) says the disease was found in a 6-year-old beef cow. The agency did not say where the cow was born. The agency says it is tracing other cattle in the herd and is trying to determine how the cow became infected with the disease. They say the new case should not affect exports of Canadian cattle or beef.</div><div><br /></div><div>Mad cow disease causes spongy holes in the brain. In people, a rare but fatal form of the disease has been linked to eating infected tissue from cows.</div><div><br /></div><div>The inspection agency has said a ban on using animal materials in feed products has virtually eliminated the spread of BSE in Canada, but it said a small number of mad cow cases are still expected to surface.</div><div><br /></div><div>-- Communicated by: ProMED-mail Rapporteur Brent Barrett</div><div><br /></div><div>****** [3] Date: Sat 16 Aug 2008 Source: The Edmonton Journal [edited] <http://www.canada.com/edmontonjournal/news/story.html?id=e1dd935b-43dc-417a-a76e-6376990ba413></div><div><br /></div><div>Another mad cow case confirmed </div><div><br /></div><div>------------------------------</div><div><br /></div><div>A 6-year-old beef cow was confirmed Friday [15 Aug 2008] as the 13th case of mad cow disease in Alberta, the Canadian Food Inspection Agency said.</div><div><br /></div><div>It is the 14th confirmed case of bovine spongiform encephalopathy, or BSE, in Canada.</div><div><br /></div><div>"At this point, it is too early to say how it could have been infected," said Natalie Bragg, a veterinary program specialist with the food inspection agency.</div><div><br /></div><div>The cow was born, raised, and died on the same farm in northern Alberta, Bragg said. The agency does not release specific locations of infected animals. The animal was euthanized after it became sick. A sample from the animal was tested twice and confirmed as carrying BSE on Friday [15 Aug 2008].</div><div><br /></div><div>The agency said no traces of BSE made it into either human or animal food supplies. Bragg said the investigation is now focused on the feed on the farm, including how it was transported and stored. The animal was born after a 1997 ban on feed containing cattle or other ruminant parts was introduced.</div><div><br /></div><div>The agency is also tracking all other animals that were born within a year, and on the same farm, as the dead cow.</div><div><br /></div><div>Last month [July 2008], it was revealed that Alberta plans to test 50 per cent fewer cattle for BSE by stopping targeted tests of elderly bovines or those without proper documentation. The step was taken because animals that are 9 years old or older are far less likely to contract the diseases, the Alberta government said. Between 2004 and mid-2006, 54 per cent of cattle tested in the province were 9 years or older. 2 of those cows tested positive.</div><div><br /></div><div>Alberta tests up to 30 000 cattle a year, roughly half of the national BSE monitoring.</div><div><br /></div><div>Cattle industry officials downplayed the latest BSE discovery, saying it should have little impact on their business, including international beef exports. "So far there has been no major reaction and no markets have closed, and that's because we have kept our international clients educated," said Cam Daniels of Canada Beef Export Federation.</div><div><br /></div><div>Daniels regularly travels to Japan, China, Mexico, Macau, the Middle East, and other places to talk to importers and distributors. "They understand clearly the control measures we have in place, and they know we're expecting more cases because we are working diligently to find them," he said.</div><div><br /></div><div>While everyone looks forward to the time when mad cow is eradicated in Canada, some overseas clients view the climbing number of cases as positive right now because it means our surveillance programs are working, said Alberta Beef Producers spokeswoman Lori Creech. "They prefer we find them, rather than in the words of Ralph Klein, 'Shoot, shovel and shut up,' " she said.</div><div><br /></div><div>"We are very transparent and open as a country. This latest case doesn't affect our status in the world at all because it's not unexpected that they would find another animal with BSE."</div><div><br /></div><div>The World Organization for Animal Health (OIE) lists Canada as a controlled risk country for BSE.</div><div><br /></div><div>[Byline: Ryan Cormier and Keith Gerein]</div><div><br /></div><div>-- Communicated by: ProMED-mail <promed@promedmail.org></div><div><br /></div><div>[Although Canada is proposing a decrease of its surveillance their system is clearly working. The system may be expensive, but it appears to be a model for others. - Mod.TG</div><div><br /></div><div>Alberta can be located on the HealthMap/ProMED-mail interactive map of Canada at <http://healthmap.org/promed?v=55.4,-101.9,4>. - CopyEd.MJ] See Also BSE, bovine - Canada (02): (BC) 20080623.1941 BSE, bovine - Canada (AB) 20080226.0786 2007 ---- BSE, bovine - Canada (AB) (03) 20071218.4076 BSE, bovine - Canada (BC) 20070502.1430 BSE, bovine - Canada (AB) (02) 20070308.0813 BSE, bovine - Canada (AB) 20070208.0499 ...................................tg/mj/dk</div><div><br /></div><div>*##########################################################*</div></div><div><br /></div><div><a fg_scanned="1" href="https://promedmail.org/promed-post/?id=20080819.2580" rel="nofollow" style="color: #196ad4;" target="_blank">https://promedmail.org/promed-post/?id=20080819.2580</a><br /></div><div><br /></div><div><div>MONDAY, JUNE 23, 2008</div><div><br /></div><div>BSE CASE CONFIRMED IN BRITISH COLUMBIA OTTAWA </div><div><br /></div><div></div><a fg_scanned="1" href="https://bovineprp.blogspot.com/2008/06/bse-case-confirmed-in-british-columbia.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://bovineprp.blogspot.com/2008/06/bse-case-confirmed-in-british-columbia.html</a><div><br /></div><div>MAD COW TESTING USDA AND CANADA</div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2008/04/mbm-greaves-meat-offal-live-cattle.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2008/04/mbm-greaves-meat-offal-live-cattle.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2008/04/report-on-investigation-of-eleventh.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2008/04/report-on-investigation-of-eleventh.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2008/03/rapid-typing-of-transmissible.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2008/03/rapid-typing-of-transmissible.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2008/01/docket-no-aphis-2006-0026-rin-0579-ac45.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2008/01/docket-no-aphis-2006-0026-rin-0579-ac45.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2008/01/bse-oie-usda.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2008/01/bse-oie-usda.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2007/12/bse-case-confirmed-in-alberta-december.html" rel="nofollow" style="color: #338fe9;" target="_blank">http://madcowtesting.blogspot.com/2007/12/bse-case-confirmed-in-alberta-december.html</a><br /></div><div><br /></div><div><a fg_scanned="1" href="http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html" rel="nofollow" style="color: #196ad4;" target="_blank">http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html</a></div></div></div><br /></div><div data-setdir="false" dir="ltr" style="font-size: 13.3333px; text-align: justify;">CANADA BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION DISEASE</div><div data-setdir="false" dir="ltr" style="font-size: 13.3333px; text-align: justify;"><br /></div><div data-setdir="false" dir="ltr" style="font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="https://inspection.canada.ca/animal-health/terrestrial-animals/diseases/reportable/federally-reportable-diseases-for-terrestrial-anim/eng/1329499145620/1329499272021" style="color: #196ad4;">inspection.canada.ca/animal-health/terrestrial-animals/diseases/reportable/federally-reportable-diseases-for-terrestrial-anim/eng/1329499145620/1329499272021</a></div><div style="font-size: 13.3333px; text-align: justify;"><br /></div><div dir="ltr" style="font-size: 13.3333px; text-align: justify;"><div><div dir="ltr" style="font-size: 16px;">MONDAY, JANUARY 09, 2023 CANADA </div><div dir="ltr" style="font-size: 16px;"><br /></div><div dir="ltr" style="font-size: 16px;">Canada Unstable funding threatens ‘zombie deer’ CWD TSE PRION research in the Prairies </div><div dir="ltr" style="font-size: 16px;"><br /></div><div dir="ltr" style="font-size: 16px;"><a fg_scanned="1" href="https://chronic-wasting-disease.blogspot.com/2023/01/canada-unstable-funding-threatens.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://chronic-wasting-disease.blogspot.com/2023/01/canada-unstable-funding-threatens.html</a></div></div><br /></div><div dir="ltr" style="font-size: 13.3333px; text-align: justify;"><div><div dir="ltr">FRIDAY, DECEMBER 09, 2022 </div><div dir="ltr"><br /></div><div dir="ltr">Manitoba, Canada PROVINCE ADVISES ADDITIONAL CASES OF CHRONIC WASTING DISEASE DETECTED<br /></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://chronic-wasting-disease.blogspot.com/2022/12/manitoba-canada-province-advises.html" rel="nofollow" style="color: #196ad4;" target="_blank">https://chronic-wasting-disease.blogspot.com/2022/12/manitoba-canada-province-advises.html</a><br /></div><div dir="ltr"><br style="font-size: 16px;" /></div></div><div><div><div>THURSDAY, OCTOBER 06, 2022 </div><div><br /></div><div>CANADA, Alberta, Québec, Manitoba, Saskatchewan, CWD UPDATE 2022 </div><div><br /></div><div><a fg_scanned="1" href="https://chronic-wasting-disease.blogspot.com/2022/10/canada-alberta-quebec-manitoba.html" rel="nofollow" style="color: #196ad4;" target="_blank">chronic-wasting-disease.blogspot.com/2022/10/canada-alberta-quebec-manitoba.html</a><br /></div></div><div><br style="font-size: 16px;" /></div></div></div><div dir="ltr">USA BSE</div><div dir="ltr"><br /></div><div dir="ltr"><div dir="ltr"><div><span face="Helvetica, Arial, sans-serif" style="color: #333333; font-size: 14px;">Notice of Request To Renew an Approved Information Collection: Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission</span><br /></div><div dir="ltr"><span face="Helvetica, Arial, sans-serif" style="color: #333333; font-size: 14px;"><br /></span><a fg_scanned="1" href="https://www.regulations.gov/comment/FSIS-2022-0027-0002" rel="nofollow" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/FSIS-2022-0027-0002</a><br /></div></div><div dir="ltr"><br /></div><div dir="ltr">Singeltary further comments in attachment;</div><div dir="ltr"><br /></div><div dir="ltr"><h3 class="ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0h5 ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0mt-0 ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0mb-1" style="color: #333333; font-family: Helvetica, Arial, sans-serif; font-size: 16px; font-weight: 500; line-height: 1.42858; margin-top: 0px;">Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment</h3><h3 class="ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0h5 ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0mt-0 ydp397e9dcyiv8679458604ydp66c71383yiv2024154281ydpc9767bf3yiv0599487745ydp88fbb9f8yiv0939494003ydpab7bd21cyiv3403474399ydp363dd048yiv8787012848ydp8ce40537yiv1003256124ydp422011c0mb-1" style="color: #333333; font-family: Helvetica, Arial, sans-serif; font-size: 16px; font-weight: 500; line-height: 1.42858; margin-top: 0px;"><a href="https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/FSIS-2022-0027-0002/attachment_1.pdf</a><br /></h3><div dir="ltr"><pre style="font-size: 13.3333px; text-align: justify; white-space: pre-wrap;"><div style="background-color: #f0f2f5; color: #050505; font-family: arial; font-size: 10pt; margin-bottom: 2em; margin-top: 0.5em;"><span style="color: #29303b; font-size: 10pt;">Sunday, January 10, 2021 </span><br /></div><div style="background-color: #f0f2f5; color: #050505; font-family: arial; font-size: 10pt; margin-bottom: 2em; margin-top: 0.5em;"><div dir="ltr" style="font-size: 10pt;"><div style="background-color: white; color: #29303b;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission June 17, 2019</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">Greetings APHIS et al, </div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal. </div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban. </div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.</div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;">AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...</div><div style="background-color: white; color: #29303b;"><br /></div><div style="background-color: white; color: #29303b;"><a fg_scanned="1" href="https://www.regulations.gov/comment/APHIS-2018-0087-0002" rel="nofollow" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/APHIS-2018-0087-0002</a><br /></div><div style="background-color: white; color: #29303b;"><br clear="none" /></div><div style="background-color: white; color: #29303b;"><a href="https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf" rel="nofollow" shape="rect" style="color: blue;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf</a></div></div></div></pre><pre style="font-size: 13.3333px; text-align: justify; white-space: pre-wrap;"><div>APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</div><div><br /></div><div>Comment from Singeltary Sr., Terry</div><div><br /></div><div>Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022</div><div><br /></div><div><a fg_scanned="1" href="http://https//www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow" style="color: #196ad4;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a></div><div><br /></div><div><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a><br /></div><div><span style="font-family: arial; font-size: 16px;"><br /></span></div><div><span style="font-family: arial; font-size: 16px;"><a fg_scanned="1" href="http://https//usdasearch.usda.gov/search?utf8=%E2%9C%93&affiliate=usda&query=2005+bse&commit=Search" rel="nofollow" style="color: #196ad4;" target="_blank">https://usdasearch.usda.gov/search?utf8=%E2%9C%93&affiliate=usda&query=2005+bse&commit=Search</a></span></div></pre></div><div dir="ltr"><div><div dir="ltr" style="font-family: Arial, Helvetica, sans-serif; font-size: 12px; line-height: 1.6em; margin: 0px 0px 0.75em;">Suppressed peer review of Harvard study October 31, 2002</div><div dir="ltr" style="font-family: Arial, Helvetica, sans-serif; font-size: 12px; line-height: 1.6em; margin: 0px 0px 0.75em;"><div>October 31, 2002</div><div><br /></div><div>Review of the Evaluation of the Potential for Bovine Spongiform Encephalopathy in the United States Conducted by the Harvard Center for Risk Analysis, Harvard School of Public Health and Center for Computational Epidemiology, College of Veterinary Medicine, Tuskegee University</div><div><br /></div><div>Final Report </div><div><br /></div><div><a href="http://web.archive.org/web/20061005020902/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20061005020902/http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf</a></div></div><div dir="ltr" style="font-family: Arial, Helvetica, sans-serif; font-size: 12px; line-height: 1.6em; margin: 0px 0px 0.75em;"><div><div>Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Update; Notice of Availability and Technical Meeting </div><div><br /></div><div>Owens, Julie</div><div><br /></div><div>From: Terry S. Singeltary Sr. [<span dir="ltr">flounder9@verizon.net</span>]</div><div><br /></div><div>Sent: Monday, July 24, 2006 1:09 PM</div><div><br /></div><div>To: FSIS RegulationsComments</div><div><br /></div><div>Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)</div><div><br /></div><div><a href="http://web.archive.org/web/20090424070523/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090424070523/http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf</a><br /></div><div><br /></div><div>Response to Public Comments on the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update,</div><div><br /></div><div>October 31, 2005</div><div><br /></div><div>INTRODUCTION</div><div><br /></div><div>The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website: <span dir="ltr">http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp</span>). Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, RCALF USA, Linda A Detwiler, and Terry S. Singeltary. This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind: </div><div><br /></div><div><a href="http://http//web.archive.org/web/20090412200037/http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090412200037/http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf</a></div><div><br /></div><div>03-025IFA</div><div><br /></div><div>03-025IFA-2</div><div><br /></div><div>Terry S. Singeltary</div><div><br /></div><div>From: Terry S. Singeltary Sr. [<span dir="ltr">flounder9@verizon.net</span>]</div><div><br /></div><div>Sent: Thursday, September 08, 2005 6:17 PM</div><div><br /></div><div>To: <span dir="ltr">fsis.regulationscomments@fsis.usda.gov</span></div><div><br /></div><div>Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle</div><div><br /></div><div><a href="http://web.archive.org/web/20060316114732/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20060316114732/http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf</a></div><div dir="ltr"><div><br /></div><div>ONE final comment tonight, i just cannot take anymore. well, ill just let the facts speak for themselves, no need to even comment ;<br /></div><div><br /></div><div>Section 2. Testing Protocols and Quality Assurance Controls<br /></div><div><br /></div><div>In November 2004, USDA announced that its rapid screening test, Bio-Rad Enzyme Linked Immunosorbent Assay (ELISA), produced an inconclusive BSE test result as part of its enhanced BSE surveillance program. The ELISA rapid screening test performed at a BSE contract laboratory produced three high positive reactive results.40 As required,41 the contract laboratory forwarded the inconclusive sample to the APHIS National Veterinary Services Laboratories (NVSL) for confirmatory testing. NVSL repeated the ELISA testing and again produced three high positive reactive results.42 In accordance with its established protocol, NVSL ran its confirmatory test, an immunohistochemistry (IHC) test, which was interpreted as negative for BSE. In addition, NVSL performed a histological43 examination of the tissue and did not detect lesions44 consistent with BSE.</div><div><br /></div><div>Faced with conflicting results, NVSL scientists recommended additional testing to resolve the discrepancy but APHIS headquarters officials concluded no further testing was necessary because testing protocols were followed. In our discussions with APHIS officials, they justified their decision not to do additional testing because the IHC is internationally recognized as the "gold standard." Also, they believed that conducting additional tests would undermine confidence in USDA’s established testing protocols.</div><div><br /></div><div>full text 130 pages ;</div><div><br /></div><div><a href="http://web.archive.org/web/20090411173629/http://www.usda.gov/oig/webdocs/50601-10-KC.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20090411173629/http://www.usda.gov/oig/webdocs/50601-10-KC.pdf</a><br /></div><div><br /></div></div><div><span style="font-size: 13.3333px; text-align: justify; white-space: pre-wrap;">PDF]Freas, William TSS SUBMISSION</span><br /></div></div><div dir="ltr"><div dir="ltr"><pre style="font-size: 13.3333px; text-align: justify; white-space: pre-wrap;">File Format: PDF/Adobe Acrobat -
Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary
Sr. [<span dir="ltr">flounder@wt.net</span>] Monday, January 08,200l <span dir="ltr">3:03 PM</span> freas ...
<a href="http://web.archive.org/web/20170301223601/https://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf" rel="nofollow" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20170301223601/https://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf</a></pre></div></div></div></div><br /></div><div dir="ltr">Terry S. Singeltary Sr.</div><div><br /></div></div></div></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-49955963432674257442023-01-23T11:28:00.000-06:002023-01-23T11:28:05.388-06:00Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection?<p><span style="background-color: white; font-family: arial; font-size: 16px;">Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection?</span></p><div style="background-color: white; font-family: arial; font-size: 16px;"><div data-setdir="false" dir="ltr"><div><div data-setdir="false" dir="ltr">THIS STUDY SHOULD BE PULLED IMMEDIATLEY FOR RISKING HUMAN LIFE DUE TO JUNK SCIENCE!</div><div><br /></div><div>Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection</div><div><br /></div><div>Jean-Claude Perez, PhD 1, Claire Moret-Chalmin, MD 2, Luc Montagnier, MD, RIP31 Biomathematics; Luc Montagnier Foundation; corresponding author, jeanclaudeperez2@gmail.com2 Neurology; Luc Montagnier Foundation; clmoret@gmail.com3 Virology; discoverer of the human immunodeficiency virus and Nobel Laureate 2008;the website for the Luc Montagnier Foundation ishttps://montagnier.net/flm/fr/accueil/Luc Montagnier, MD, and Nobel Laureate, esteemed colleague and friend, passed from this world on February 8, 2022 not long after the completion of the preliminary draft of this work which his co-authors have carried forward to this updated report with some additional cases and new information. Perhaps this may be the most important work of Luc’s lifetime expressing his incredible genius and spirit. While hospitalized, he continued to attach the greatest importance to the publication of this article. He is honored by the Luc Montagnier Foundation Quai Gustave-Ador 62 1207, Geneva, Switzerland.</div><div><br /></div><div>ABSTRACT</div><div><br /></div><div>Creutzfeldt-Jakob Disease, the formerly rare but universally fatal prion disease in humans, normally progresses over several decades before it leads to death. In the Appendix to this paper, we highlight the presence of a prion region in the spike protein of the original SARS-CoV-2,and in all the “vaccine” variants built from the Wuhan virus. The prion region in the spike of SARS-CoV-2 has a density of mutations eight times greater than that of the rest of the spike, and, yet, strangely that entire prion region disappears completely in the Omicron variant. In the main body of our text, we present 26 cases of Creuzfeldt-Jacob Disease, all diagnosed in 2021 with the first symptoms appearing within an average of 11.38 days after a Pfizer, Moderna, or AstraZeneca COVID-19 injection. Because the causal progression, the etiopathogenesis, of these atypical and new cases of human prion disease—cases of what is apparently a totally new form of rapidly developing Creuzfeldt-Jacob Disease—we focus on the chronology of the symptomatic development. We consider it from an anamnestic point of view —one in which we compare the typical development of pre-COVID cases of Creuzfeldt-Jacob Disease to the extremely accelerated development of similar symptoms in the 26 cases under examination. By such an approach, we hope to work out the etiopathogenesis critical to understanding this new and much more rapidly developing human prion disease. By recalling the sequential pathway of that the formerly subacute and slowly developing disease followed in the past, and by comparing it with this new, extremely acute, rapidly developing prion disease—one following closely usually after two of the COVID-19 injections—we believe it is correct to infer that the injections caused the disease in these 26 cases. If so, they have probably also caused a many other cases that have gone undiagnosed because of their rapid progression to death. By late 2021, 20 had died within 4.76 months of the offending injection. Of those, 8 died suddenly within 2.5 months confirming the rapid progression of this accelerated form of Creuzfeldt-Jacob Disease. By June 2022, 5 more patients had died, and at the time of this current writing, only 1 remains still alive.</div><div><br /></div><div>Keywords: Creutzfeldt-Jacob Disease, onset of CJD, prion protein, SARS-CoV-2 variants, spike protein, COVID-19 mRNA vaccines, neuropsychiatric disease, evolution of the COVID virus</div><div><br /></div><div data-setdir="false" dir="ltr"><a fg_scanned="1" href="https://ijvtpr.com/index.php/IJVTPR/article/view/66/179" rel="nofollow" style="color: #196ad4;" target="_blank">https://ijvtpr.com/index.php/IJVTPR/article/view/66/179</a><br /></div><div><br /></div><div>''we believe it is correct to infer that the injections caused the disease in these 26 cases. If so, they have probably also caused a many other cases that have gone undiagnosed because of their rapid progression to death'' </div></div><div><br /></div><div data-setdir="false" dir="ltr">Greetings Everyone,</div></div><div><br /></div><div data-setdir="false" dir="ltr">i said i was not going to comment on this junk science, i was just going to ignore it, but i just can't, i thought i must respond now. too many lives are at stake from Covid, (NOT THE VACCINE), and too many families and friends of victims of creutzfeldt jakob disease are going to read this, and then refuse to get a Covid vaccine, then some might die, more so than from the CJD itself, and they could die from this junk science, imo.</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr">the word 'SPONTANEOUS' was used 44 times in this paper. </div><div><br /></div><div>How does horse shit like this even get published? This is from a Nobel Laureate.</div><div><br /></div><div>I see no scientific evidence, just supposition…am I missing something?</div><div><br /></div><div>People are going to read this junk science and they are going to run with it, and a lot of folks that would have gotten a covid vaccine, will now refuse to get one after reading this. i have written about the risk factors from vaccines and TSE prion disease (BSE), from back in the BSE days. i see no risk factors from covid vaccines, it's not a live vaccine from any animal tissue as far as i know. </div><div><br /></div><div>Seems with a disease such as cjd tse prion disease, of very long disease course, with as many Covid vaccines given out, there is of course humans incubating the cjd tse prion disease when they get Covid vaccine, so imo, these are case of cjd that got their Covid vaccines around the same time or before, while cjd was incubating, and afterward they became clinical with new cjd like disease, a terrible happenstance of bad luck scenarios. I see no scientific link of Covid vaccines to any CJD TSE Prion disease, imo.</div><div><br /></div><div data-setdir="false" dir="ltr">There needs to be some Coalition of Transmissible Spongiform Encephalopathy TSE, Creutzfeldt Jakob Disease CJD, Prion Scientist from around the world and make a statement condemning this study ''Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection'' for the junk science it is. </div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr">This study and the anti-vaccine groups that push them are dangerous, and this study should be pulled ASAP, imo.</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr">It upset me so bad that i have written some of the top CJD TSE Prion Scientist around the globe, and express my concern with this study, and ask them what they thought of my concerns, and i thought i might post a few of them without divulging their identity. now most all of these comments were from Professors of TSE Prion science at top Universities. i hope they come forward and condemn this study for what it is, and just how dangerous crap like this can be once it's published...</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr">disgusted again in Bacliff, Texas USA 77518</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr">1. comment</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr"><div class="ydpb7618fcfyiv4762150982gmail_attr" dir="ltr">On Thu, Jan 19, 2023 at 2:31 PM Terry Singeltary <<a href="mailto:flounder9@verizon.net" rel="nofollow" shape="rect" style="color: #196ad4;" target="_blank">flounder9@verizon.net</a>> wrote:<br clear="none" /></div><blockquote class="ydpb7618fcfyiv4762150982gmail_quote" style="border-left: 1px solid rgb(204, 204, 204); margin: 0px 0px 0px 0.8ex; padding: 8px 8px 8px 1ex;"><div dir="ltr"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">re-Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Greetings Professor xxxxx et al, </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">How does horse shit like this even get published? This is from a Nobel Laureate.</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">I see no scientific evidence, just supposition…am I missing something?<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">snip...</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div>Hi Terry,</div><div><br /></div><div>Oh man---this is such a ridiculous article! The journal is not a valid journal--among the worst of the predatory journals---but unfortunately, the anti-vaxxers don't pay much attention to how credible a source is. You are absolutely correct re: incubation periods for CJD etc--arghhh! Prion diseases are complicated enough on their own---but then when people put out this garbage--deflects time and energy to argue against it. </div><div><br /></div><div>Thank you for bringing this to my attention.</div></div></div></blockquote></div><div data-setdir="false" dir="ltr"><br /></div></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;">2. comment</div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div class="ydp92a6a55cyiv0209950288gmail_attr" dir="ltr">On Thu, Jan 19, 2023 at 4:38 PM Terry Singeltary <<a href="mailto:flounder9@verizon.net" rel="nofollow" shape="rect" style="color: #196ad4;" target="_blank">flounder9@verizon.net</a>> wrote:<br clear="none" /></div><blockquote class="ydp92a6a55cyiv0209950288gmail_quote" style="border-left: 1px solid rgb(204, 204, 204); margin: 0px 0px 0px 0.8ex; padding: 8px 8px 8px 1ex;"><div dir="ltr"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">re-Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Greetings Professor xxxxx et al, </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">How does horse shit like this even get published? This is from a Nobel Laureate.</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">I see no scientific evidence, just supposition…am I missing something?<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">snip...</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><span style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">Hi Terry,</span><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;"><br clear="none" /></div><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">I agree with you that this paper has lots of problems....snip... It is sad that some journals will publish anything.</div><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;"><br clear="none" /></div><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">Best,</div></div></div></blockquote></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;">3. comment</div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div class="ydpd06a3b68yiv3926506931gmail_attr" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">On Thu, Jan 19, 2023 at 5:11 PM Terry Singeltary <<a href="mailto:flounder9@verizon.net" rel="nofollow" shape="rect" style="color: #196ad4;" target="_blank">flounder9@verizon.net</a>> wrote:<br clear="none" /></div><blockquote class="ydpd06a3b68yiv3926506931gmail_quote" style="border-left: 1px solid rgb(204, 204, 204); font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; margin: 0px 0px 0px 0.8ex; padding: 8px 8px 8px 1ex;"><div style="font-family: arial;"><div dir="ltr"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">re-Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Greetings xxxxxx and xxxxxx, </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div dir="ltr">i have gone down a list of prominent cjd tse prion scientist, and none...NONE! see this study as valid, and all seem to agree with what i said below...this publication is dangerous imo.</div><div dir="ltr"><br clear="none" /></div><div dir="ltr">How does horse shit like this even get published? This is from a Nobel Laureate.</div><div dir="ltr"><br clear="none" /></div><div dir="ltr">I see no scientific evidence, just supposition…am I missing something?</div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">snip...</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">You are speaking to the choir my friend. By the way, it's from a deceased Nobel laureate! You really can't make this stuff up...<br clear="none" /></div><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;"><br /></div></div></div></div></blockquote></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;">4. comment</div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">re-Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Greetings Professor xxxxxxx et al, </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">How does horse shit like this even get published? This is from a Nobel Laureate.</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">I see no scientific evidence, just supposition…am I missing something?<br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br clear="none" /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">People are going to read this junk science and ...</div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">snip...</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div><p style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; margin-bottom: 0px; margin-top: 0px;">Thanks for your message Terry. I also feel distressed with the incredibly lack of responsibility in publishing such an article. I myself have been trapped into writing excuse letters for the vaccine because of the fear engendered by this and other articles. The genie is out of the bottle, and he is not going back in...</p><p style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; margin-bottom: 0px; margin-top: 0px;"><br clear="none" /></p><p style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; margin-bottom: 0px; margin-top: 0px;">Best</p></div><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">end...TSS</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">5. comment</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div><div>re-Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection</div><div><br /></div><div>Greetings Professor xxxxx et al, </div><div><br /></div><div>How does horse shit like this even get published?</div><div><br /></div><div>I see no scientific evidence, just supposition…am I missing something?</div><div><br /></div><div>snip...</div><div><br /></div><div>Thu, Jan 19 at 3:36 PM</div><div><br /></div><div>I agree. I read the paper and I do not see a convincing case for the relationship of covid vaccines and CJD.</div></div><div><br /></div><div data-setdir="false" dir="ltr">6. comment</div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr"><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">> On 18 Jan 2023, at 23:10, TERRY SINGELTARY <<a href="mailto:flounder9@verizon.net" rel="nofollow" style="color: #196ad4;" target="_blank">flounder9@verizon.net</a>> wrote:<br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">><br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">> Greetings Professor xxxxxxx,<br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">><br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">> This looks like horse shit to me, but how does this get published, I see no evidence?<br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">><br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">> Kind regards, terry<br /></div><div dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif;">snip...</div></div><div data-setdir="false" dir="ltr"><br /></div><div data-setdir="false" dir="ltr"><div data-test-id="focus-group"><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dem_N ydpbc0d2c2do_h ydpbc0d2c2dA_6FsP ydpbc0d2c2dP_3Y3Gk"><span class="ydpbc0d2c2den_N ydpbc0d2c2di_3mCHE ydpbc0d2c2dc1AVi73_6LUG ydpbc0d2c2dc1AVi7H_6MWH" data-test-id="message-group-subject" title="Re: Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection">Re: Emergence of a New Creutzfeldt-Jakob Disease: 26 Cases of the Human Version of Mad-Cow Disease, Days After a COVID-19 Injection</span></div><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dY_eTB ydpbc0d2c2den_0"><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dem_N ydpbc0d2c2den_0 ydpbc0d2c2do_h ydpbc0d2c2dY_dm5 ydpbc0d2c2dE_fq7 ydpbc0d2c2dU_dm5 ydpbc0d2c2dQ_72FG"><br /></div><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dem_N ydpbc0d2c2den_0 ydpbc0d2c2do_h ydpbc0d2c2dY_dm5 ydpbc0d2c2dE_fq7 ydpbc0d2c2dU_dm5 ydpbc0d2c2dQ_72FG">I agree!</div><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dem_N ydpbc0d2c2den_0 ydpbc0d2c2do_h ydpbc0d2c2dY_dm5 ydpbc0d2c2dE_fq7 ydpbc0d2c2dU_dm5 ydpbc0d2c2dQ_72FG"><br /></div><div class="ydpbc0d2c2dD_F ydpbc0d2c2dab_C ydpbc0d2c2dem_N ydpbc0d2c2den_0 ydpbc0d2c2do_h ydpbc0d2c2dY_dm5 ydpbc0d2c2dE_fq7 ydpbc0d2c2dU_dm5 ydpbc0d2c2dQ_72FG" data-setdir="false" dir="ltr">END</div></div></div></div></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">I believe that this study should be pulled immediately for the trash it is, nothing, NOTHING, shows any link from the covid vaccine to _CJD_ cases, just supposition. </div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">AS dangerous as this study is, i hope that CJD TSE Prion Scientist around the Globe come forth publicly with their concerns, and the study is pulled...terry</div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div><div style="color: #222222; font-family: arial, helvetica; font-size: 12px;"><span style="font-family: Georgia, serif; font-size: 10pt;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</span></div><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px;"><span style="font-size: 10pt; line-height: 1.22em;"><span style="background-color: inherit; font-family: Georgia, serif;"><br clear="none" /></span></span></div><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px;"><span style="font-size: 10pt; line-height: 1.22em;"></span><div style="font-family: arial, helvetica; line-height: 1.22em;"><div style="line-height: 1.22em;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div></div><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a fg_scanned="1" href="https://www..nature.com/articles/srep11573" rel="nofollow" shape="rect" style="color: purple; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a fg_scanned="1" href="https://www.nature.com/articles/srep11573" rel="nofollow" shape="rect" style="color: purple; line-height: 1.22em;" target="_blank">https://www.nature.com/articles/srep11573</a></span><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"> </span></div></div><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div style="font-family: arial;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA </div><div style="font-family: arial;"><br /></div><div style="font-family: arial;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </div><div style="font-family: arial;"><br /></div><div style="font-family: arial;">To the Editor: </div><div style="font-family: arial;"><br /></div><div style="font-family: arial;">In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.. </div><div style="font-family: arial;"><br /></div><div style="font-family: arial;">Terry S. Singeltary, Sr Bacliff, Tex </div><div style="font-family: arial;"><br /></div><div style="font-family: arial;">1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.</div><div style="font-family: arial;"><br /></div><div style="font-family: arial;"><a fg_scanned="1" href="http://jama.jamanetwork.com/article.aspx?articleid=1031186" rel="nofollow" style="color: #196ad4;" target="_blank">jama.jamanetwork.com/article.aspx?articleid=1031186</a></div></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div data-setdir="false" dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Terry S. Singeltary Sr., flounder9@verizon.net Bacliff, Texas, 77518</div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-2986256688827230312022-12-02T15:29:00.000-06:002022-12-02T15:29:04.310-06:00Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update<p><span style="background-color: white; color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;">Creutzfeldt Jacob Disease CJD TSE Prion December 2022 Annual Update</span></p><div style="background-color: white; color: #1d2228; font-family: arial; font-size: 16px;"><div dir="ltr"><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><div><div dir="ltr"><br /></div><div>National Prion Disease Pathology Surveillance Center Cases Examined1</div><div><br /></div><div>(September 20, 2022)</div><div><br /></div><div>Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD</div><div><br /></div><div>1999 & earlier 383 232 202 27 3 0</div><div><br /></div><div>2000 145 102 90 12 0 0</div><div><br /></div><div>2001 209 118 110 8 0 0</div><div><br /></div><div>2002 241 144 124 18 2 0</div><div><br /></div><div>2003 259 160 137 21 2 0</div><div><br /></div><div>2004 315 180 163 16 0 13</div><div><br /></div><div>2005 328 179 157 21 1 0</div><div><br /></div><div>2006 365 179 159 17 1 24</div><div><br /></div><div>2007 374 210 191 19 0 0</div><div><br /></div><div>2008 384 221 205 16 0 0</div><div><br /></div><div>2009 397 231 210 20 1 0</div><div><br /></div><div>2010 401 246 218 28 0 0</div><div><br /></div><div>2011 392 238 214 24 0 0</div><div><br /></div><div>2012 413 244 221 23 0 0</div><div><br /></div><div>2013 416 258 223 34 1 0</div><div><br /></div><div>2014 355 208 185 21 1 15</div><div><br /></div><div>2015 401 263 243 20 0 0</div><div><br /></div><div>2016 395 277 248 29 0 0</div><div><br /></div><div>2017 375 266 247 19 0 0</div><div><br /></div><div>2018 308 221 202 18 1 0</div><div><br /></div><div>2019 434 281 259 22 0 0</div><div><br /></div><div>2020 365 252 227 24 1 0</div><div><br /></div><div>2021 343 248 223 22 0 0</div><div><br /></div><div>2022 213 124 98 9 0 0</div><div><br /></div><div>TOTAL 82116 50827 45568 4889 14 4</div><div><br /></div><div>Year CSF Only & RT-QuIC Positive10</div><div><br /></div><div>2015 140</div><div><br /></div><div>2016 183</div><div><br /></div><div>2017 227</div><div><br /></div><div>2018 266</div><div><br /></div><div>2019 311</div><div><br /></div><div>2020 310</div><div><br /></div><div>2021 341</div><div><br /></div><div>2022 262</div><div><br /></div><div>TOTAL 2040</div><div><br /></div><div>1 Listed based on the year of death or, if not available, on year of referral; </div><div><br /></div><div>2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; </div><div><br /></div><div>3 Disease acquired in the United Kingdom; </div><div><br /></div><div>4 Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; </div><div><br /></div><div>5 Disease possibly acquired in a Middle Eastern or Eastern European country; </div><div><br /></div><div>6 Includes 28 cases in which the diagnosis is pending (1 from 2020, 3 from 2021 and 24 from 2022), and 20 inconclusive cases;</div><div><br /></div><div>7 Includes 20 (3 from 2021 and 17 from 2022) cases with type determination pending in which the diagnosis of vCJD has been excluded. </div><div><br /></div><div>8 The sporadic cases include 4437 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 82 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 37 cases of sporadic Fatal Insomnia (sFI). </div><div><br /></div><div>9 Total does not include 300 Familial cases diagnosed by blood only. </div><div><br /></div><div>10 Lists number of patients (deceased and alive) who have had a positive RT-QuIC and no neuropath examination.</div></div><div><br /></div><div dir="ltr"><a href="https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://case.edu/medicine/pathology/sites/case.edu.pathology/files/2022-10/WebTable%20NPDPSC.pdf</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><br /></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;">SUNDAY, MAY 08, 2022 </span></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;"><br /></span></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;">USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022 </span></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/05/usa-national-prion-disease-pathology.html</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;"><br /></span></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;">Tuesday APRIL 05, 2022 </span></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;"><br /></span></div><div dir="ltr"><span style="font-family: Helvetica, Arial, sans-serif;">Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014 </span></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html" rel="nofollow noopener noreferrer" style="color: #196ad4; font-family: Helvetica, Arial, sans-serif;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><br /></div><div dir="ltr"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">TUESDAY, MAY 24, 2022 </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022<br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><a fg_scanned="1" href="https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://cjdtexas.blogspot.com/2022/05/texas-creutzfeldt-jakob-disease-cjd-tse.html</a></div></div></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">TUESDAY, MAY 10, 2022 <br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network<br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">creutzfeldt-jakob-disease.blogspot.com/2022/05/concordance-of-csf-rt-quic-across.html</a><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Friday, DECEMBER 24, 2021 </div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021<br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2021/12/creutzfeldt-jakob-disease-cjd-tse-prion.html</a></div></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><div><div dir="ltr"><div><div><br /></div><div>Canada Definite and probable CJD, 1998-2022 As of October 31, 2022</div><div><br /></div><div>Year Sporadic Iatrogenic CJD Genetic vCJD Total</div><div><br /></div><div>1998 22 1 1 0 24</div><div><br /></div><div>1999 27 2 3 0 32</div><div><br /></div><div>2000 32 0 3 0 35</div><div><br /></div><div>2001 27 0 3 0 30</div><div><br /></div><div>2002 30 0 5 1 36</div><div><br /></div><div>2003 27 1 1 0 29</div><div><br /></div><div>2004 42 0 4 0 44</div><div><br /></div><div>2005 42 0 2 0 44</div><div><br /></div><div>2006 39 0 5 0 44</div><div><br /></div><div>2007 35 0 4 0 39</div><div><br /></div><div>2008 48 0 1 0 49</div><div><br /></div><div>2009 48 0 5 0 53</div><div><br /></div><div>2010 35 0 3 0 38</div><div><br /></div><div>2011 46 0 4 1 51</div><div><br /></div><div>2012 62 0 1 0 63</div><div><br /></div><div>2013 50 0 1 0 51</div><div><br /></div><div>2014 51 0 5 0 56</div><div><br /></div><div>2015 44 0 8 0 52</div><div><br /></div><div>2016 57 1 6 0 64</div><div><br /></div><div>2017 82 0 5 0 87</div><div><br /></div><div>2018 75 1 5 0 81</div><div><br /></div><div>2019 76 0 2 0 78</div><div><br /></div><div>2020 65 0 4 0 69</div><div><br /></div><div>2021 56 0 3 0 59</div><div><br /></div><div>2022 64 0 1 0 65</div><div><br /></div><div>Total 1183 6 83 2 1274</div><div><br /></div><div>Cases with definite and probable diagnosis to date</div></div><br /></div><div dir="ltr"><a fg_scanned="1" href="https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.canada.ca/en/public-health/services/surveillance/blood-safety-contribution-program/creutzfeldt-jakob-disease/cjd-surveillance-system.html#ref</a><br /></div><div dir="ltr"><br /></div><div dir="ltr">PLEASE NOTE, Canada does not mention VPSPr <b style="color: #202124; font-family: Roboto, arial, sans-serif; font-size: 16px;">Variably protease-sensitive prionopathy</b><span style="color: #202124; font-family: Roboto, arial, sans-serif; font-size: 16px;"> and you can read why here ;</span></div><div dir="ltr"><span style="color: #202124; font-family: Roboto, arial, sans-serif; font-size: 16px;"><br /></span></div><div dir="ltr"><div><div style="color: #333333; font-family: arial; font-size: 13.3333px;">WHY do some countries count vpspr as sporadic cjd tse prion, and some countries don't?</div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;">THIS problem must be addressed immediately imo.</div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;">WE have the USA classifying Variably protease-sensitive prionopathy (VPSPr) (formerly known as Protease Sensitive Prionopathy) as sporadic Creutzfeldt Jakob Disease sCJD, and we have Canada not even mentioning in on there statistics links, like vpspr does not even exist, so this is a problem for any valid surveillance imo. IN fact, personal communication from Canada Surveillance et al;</div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;">QUOTE;</div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;">''Well Terry, we have the data. We simply do not report it separately because we do not believe it has any specific epidemiologic significance, including zoonotic transmission (this opinion is shared unanimously by the international CJD surveillance community, and was established very quickly after the discovery of VPSPr). The key reason in my mind why the US system reports it – in a footnote to their sporadic CJD data – is that they discovered it, and want to follow up on it publicly to validate the reality of their finding scientifically (which is distinct from its significance).''<br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><span style="font-family: sans-serif; font-size: 13px;">''The simple answer to your question is that we do not track VPSPr separately, as we view is as a form of sporadic CJD with an unusual phenotype but no specific epidemiological significance. Even the USA surveillance figures do not report it separately.''</span><br /></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><span style="font-family: sans-serif; font-size: 13px;"><br /></span></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: 13px;">end</span></div></div><div style="color: #333333; font-family: arial; font-size: 13.3333px;"><span style="font-family: Arial, Helvetica, sans-serif; font-size: 13px;"><br /></span></div><div dir="ltr" style="color: #333333; font-family: arial; font-size: 13.3333px;"><a fg_scanned="1" href="https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://vpspr.blogspot.com/2022/04/phenotypic-heterogeneity-of-variably.html</a><span style="font-family: Arial, Helvetica, sans-serif; font-size: 13px;"><br /></span></div></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://vpspr.blogspot.com/" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://vpspr.blogspot.com/</a><br /></div><div dir="ltr"><br /></div><div dir="ltr"><span style="color: #333333; font-family: Arial, Helvetica, sans-serif;">Hell of a way for a surveillance system for any country to look for any suspect unusual zoonosis zoonotic disease from any mutated TSE Prion strain from any species. ...terry</span><br /></div><div dir="ltr"><span style="color: #333333; font-family: Arial, Helvetica, sans-serif;"><br /></span></div><div>United Kingdom</div><div><br /></div><div>CREUTZFELDT-JAKOB DISEASE IN THE UK (By Calendar Year)</div><div><br /></div><div>Source: NCJDRSU website www.cjd.ed.ac.uk - updated 07/11/2022</div><div><br /></div><div>REFERRALS OF SUSPECT CJD DEATHS OF DEFINITE AND PROBABLE CJD</div><div><br /></div><div>Year Referrals Year Sporadic1 Iatrogenic Genetic2 vCJD Total Deaths</div><div><br /></div><div>1990 [53]† 1990 28 5 0 - 33</div><div><br /></div><div>1991 75 1991 31 1 4 - 36</div><div><br /></div><div>1992 96 1992 45 2 6 - 53</div><div><br /></div><div>1993 79 1993 36 4 7 - 47</div><div><br /></div><div>1994 119 1994 53 1 9 - 63</div><div><br /></div><div>1995 87 1995 35 4 5 3 47</div><div><br /></div><div>1996 132 1996 40 4 6 10 60</div><div><br /></div><div>1997 163 1997 59 6 7 10 82</div><div><br /></div><div>1998 155 1998 64 3 5 18 90</div><div><br /></div><div>1999 170 1999 62 6 2 15 85</div><div><br /></div><div>2000 178 2000 48 1 3 28 80</div><div><br /></div><div>2001 179 2001 58 4 6 20 88</div><div><br /></div><div>2002 164 2002 73 0 5 17 95</div><div><br /></div><div>2003 163 2003 79 5 7 18 109</div><div><br /></div><div>2004 114 2004 50 2 6 9 67</div><div><br /></div><div>2005 124 2005 67 4 13 5 89</div><div><br /></div><div>2006 112 2006 68 1 9 5 83</div><div><br /></div><div>2007 119 2007 63 2 11 5 81</div><div><br /></div><div>2008 150 2008 84 5 6 2 97</div><div><br /></div><div>2009 153 2009 78 2 8 3 91</div><div><br /></div><div>2010 150 2010 85 3 6 3 97</div><div><br /></div><div>2011 158 2011 91 4 14 5 114</div><div><br /></div><div>2012 127 2012 92 5 12 0 109</div><div><br /></div><div>2013 152 2013 108 2 10 1 121</div><div><br /></div><div>2014 130 2014 100 3 13 0 116</div><div><br /></div><div>2015 140 2015 105 0 4 0 109</div><div><br /></div><div>2016 148 2016 118 1 6 1 126</div><div><br /></div><div>2017 159 2017 122 0 12 0 134</div><div><br /></div><div>2018 167 2018 137 2 12 0 151</div><div><br /></div><div>2019 147 2019 128 1 7 0 136</div><div><br /></div><div>2020 172 2020 135 1 8 0 144</div><div><br /></div><div>2021 179 2021 132 0 6 0 138</div><div><br /></div><div>2022 148 2022 113 0 3 0 116</div><div><br /></div><div>Total Referrals 4562 Total Deaths 2587 84 238 178 3087</div><div><br /></div><div>† Referral figure for 1990 is from 1 May onwards *As at 7 th November 2022</div><div><br /></div><div>Summary of vCJD cases</div><div><br /></div><div>Deaths from definite vCJD (confirmed): 123</div><div><br /></div><div>Deaths from probable vCJD (without neuropathological confirmation): 55</div><div><br /></div><div>Number of deaths from definite or probable vCJD (as above): 178</div><div><br /></div><div>Number of definite/probable vCJD cases still alive: 0</div><div><br /></div><div>Total number of definite or probable vCJD (dead and alive): 178</div><div><br /></div><div>1 There are, in addition, a total of 20 cases of vPSPr (death in 1997(1 case), 2004(1), 2006(1), 2008(3), 2010(1), 2012(4), 2013(1), 2016(3), 2017(1), 2018(1), 2019(1), 2020(2)) not included in the above figures.</div><div><br /></div><div>2 includes all genetic prion disease, including GSS.</div><div><br /></div><div>Source: NCJDRSU website www.cjd.ed.ac.uk - updated 07/11/2022</div><div><br /></div></div><div dir="ltr"><a href="https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.cjd.ed.ac.uk/sites/default/files/figs.pdf</a><br /></div><div dir="ltr"><br /></div></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><a href="https://www.cjd.ed.ac.uk/sites/default/files/report30.pdf" rel="nofollow noopener noreferrer" style="color: #6283a6; font-family: Arial, Helvetica, sans-serif; font-size: 14.4px;" target="_blank">Latest NCJDRSU annual report, covering the period 1990-2021</a><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><a href="http://www.cjd.ed.ac.uk/sites/default/files/report30.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">http://www.cjd.ed.ac.uk/sites/default/files/report30.pdf</a><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;">Safe laboratory management of prions and proteopathic seeds<br /></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">CORRESPONDENCE| VOLUME 20, ISSUE 12, P981, DECEMBER 01, 2021</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">Safe laboratory management of prions and proteopathic seeds</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">Simon Mead Thomas Evans</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">on behalf of the Advisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup Published:December, 2021DOI: <a fg_scanned="1" href="https://doi.org/10.1016/S1474-4422(21)00379-3" rel="nofollow noopener noreferrer" style="color: #cc6611;" target="_blank">https://doi.org/10.1016/S1474-4422(21)00379-3</a></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">Prions, the infectious agents of fatal and transmissible neurodegenerative disorders in humans and animals, are comprised of assemblies of misfolded forms of prion protein (PrP). The death of a 33-year-old researcher of prion diseases from variant Creutzfeldt-Jakob disease (ie, the strain of disease that is derived from bovine spongiform encephalopathy) 9 years after a percutaneous exposure to prion-contaminated material, and the death from or diagnosis of prion disease in two other people in Europe after working in prion research, emphasises the importance of statutory guidance for laboratory safety when working with dangerous pathogens.1 People in numerous laboratories handling diagnostic blood, CSF, and other low-risk biofluid samples from patients with or suspected to have Creutzfeldt-Jakob disease have contacted us to suggest that the existing guidance was not sufficiently clear or proportionate.</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">Evidence has accrued for the potential for proteins that are linked to neurodegenerative diseases, other than PrP, to adopt abnormal conformations, self-propagate, and cause transmissible pathologies and diseases in humans and laboratory animals.2, 3 These proteins share a range of pathological properties but are also distinct from prions in important ways, including that there are no known animal or human epidemics or established occupational risks. Experiments that involve inoculating, concentrating, or synthesising these so-called proteopathic seeds have become routine in the past decade, but no statutory guidance is available for safety. Human–human transmission of amyloid β proteopathic seeds has been observed in some specific circumstances that were also shown to transmit prion infection (eg, use of cadaver-derived human pituitary hormones or dura mater in neurosurgery) and can cause iatrogenic cerebral amyloid angiopathy and fatal brain haemorrhage after long latencies.4 The popularity of this field of research, and the long latencies that are to be expected for diseases that are caused by these proteopathic seeds, mean that occupational exposures might not yet have resulted in any clinical consequences. It is prudent, therefore, to consider potential risks from laboratory work involving these agents.</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">The UK's Advisory Committee for Dangerous Pathogens convened a subgroup to revise guidance for safe working with prions and to consider whether any measures were needed for work with proteopathic seeds, involving experts from research laboratories for prion and other neurodegenerative diseases, infectious disease specialists, pathologists, veterinarians, and health and safety experts. In the new guidance, we emphasise a distinction between high-risk CNS tissues and research samples that contain high concentrations of prions, which need to be managed in specialised laboratories with strict policies, and low-risk biofluids, such as blood and CSF, from patients who are suspected to have Creutzfeldt-Jakob disease with no or low concentrations of prions, which can be managed in a high-throughput diagnostic laboratory setting through adherence to appropriate general laboratory practices. We also concluded that the poorly defined pathogenicity in humans of proteopathic seeds when prepared in concentrated forms for biochemical, structural, or transmission studies means that they should now be considered as hazard group 2 pathogens, necessitating work in a containment level 2 facility. We recommend a range of safety measures,5 including special attention to risk assessment and staff training; recording of accidental exposures; special caution with the use of any sharp tools to avoid percutaneous injury; work inside a microbiological safety cabinet; and the use of spill trays, absorbent material, and defined procedures to decontaminate equipment and spills to avoid contamination of the laboratory environment.</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">Importantly, we do not recommend any changes to existing procedures for the routine handling of tissues and biofluids from patients with non-prion neurodegenerative conditions for diagnostic or research purposes. We hope that this new guidance will be seen as proportionate and precautionary and help organisations to have increased confidence about the safety of their employees.5</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;">We declare no competing interests. Members of the Advisory Committee for Dangerous Pathogens Transmissible Spongiform Encephalopathy Subgroup are listed in the appendix.</span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><span style="font-size: 13.3333px;"><br /></span></div><div dir="ltr" style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><a fg_scanned="1" href="https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(21)00379-3/fulltext" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(21)00379-3/fulltext</a><span style="font-size: 13.3333px;"><br /></span></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><br /></div><div style="color: #222222; font-family: Arial, Tahoma, Helvetica, FreeSans, sans-serif; font-size: 13.2px;"><a fg_scanned="1" href="https://www.thelancet.com/action/showPdf?pii=S1474-4422%2821%2900379-3" rel="nofollow noopener noreferrer" style="color: #0096ef; cursor: pointer;" target="_blank">https://www.thelancet.com/action/showPdf?pii=S1474-4422%2821%2900379-3</a></div></div><div dir="ltr"><br /></div><div dir="ltr"><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x11i5rnm yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xat24cr yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1mh8g0r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1vvkbs yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xtlvy1s" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;">The importance of ongoing international surveillance for Creutzfeldt–Jakob disease</div><div style="font-family: inherit;"><br /></div></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x11i5rnm yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xat24cr yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1mh8g0r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1vvkbs yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xtlvy1s" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;">Neil Watson1, Jean-Philippe Brandel2, Alison Green1, Peter Hermann3, Anna Ladogana 4, Terri Lindsay1, Janet Mackenzie1, Maurizio Pocchiari 4, Colin Smith1, Inga Zerr3 and Suvankar Pal 1 <span class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x3nfvp2 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1j61x8r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1fcty0u yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xdj266r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xhhsvwb yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xat24cr yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xgzva0m yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xxymvpz yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xlup9mm yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1kky2od" style="font-family: inherit; margin: 0px 1px; min-height: 16px; vertical-align: middle; width: 16px;"><img alt="✉" class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617" fg_scanned="1" height="16" src="https://ecp.yusercontent.com/mail?url=https%3A%2F%2Fstatic.xx.fbcdn.net%2Fimages%2Femoji.php%2Fv9%2Ft37%2F1%2F16%2F2709.png&t=1670016388&ymreqid=d41d8cd9-8f00-b204-1c96-860006016a00&sig=n4rGiK8VG959ljvcqCUiIA--~D" style="border: 0px; text-indent: -9999px;" width="16" /></span></div></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x11i5rnm yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xat24cr yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1mh8g0r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1vvkbs yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xtlvy1s" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;">Abstract | Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.</div><div style="font-family: inherit;"><br /></div><div style="font-family: inherit;"><a href="https://www.nature.com/articles/s41582-021-00488-7.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.nature.com/articles/s41582-021-00488-7.pdf</a><br /></div><div style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;">see also;</div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><a fg_scanned="1" href="https://thedaily.case.edu/researchers-find-infectious-prions-creutzfeldt-jakob-disease-patient-skin/" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://thedaily.case.edu/researchers-find-infectious-prions-creutzfeldt-jakob-disease-patient-skin/</a><br /></div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><a fg_scanned="1" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187701/" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187701/</a><br /></div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><a fg_scanned="1" href="https://www.nih.gov/news-events/nih-research-matters/prions-found-skin-people-creutzfeldt-jakob-disease" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.nih.gov/news-events/nih-research-matters/prions-found-skin-people-creutzfeldt-jakob-disease</a></div></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x11i5rnm yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xat24cr yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1mh8g0r yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268x1vvkbs yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp5ea9139fyiv8772872617ydp42168268xtlvy1s" style="background-color: #f0f2f5; color: #050505; font-family: Arial, sans-serif; font-size: 15px; margin: 0.5em 0px 0px;"><div style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;">ACDP TSE subgroup minutes, agendas and papers, history<br /></div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><a fg_scanned="1" href="https://app.box.com/s/hhhhg857fjpu2bnxhv6e/folder/2936396377" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://app.box.com/s/hhhhg857fjpu2bnxhv6e/folder/2936396377</a><br /></div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><div dir="ltr">TUESDAY, NOVEMBER 1, 2022 <br /></div><div dir="ltr"><br /></div><div dir="ltr">SEAC Scientific Steering Committee on TSE Prion<br /></div><div dir="ltr"><br /></div><div dir="ltr"><a fg_scanned="1" href="https://bovineprp.blogspot.com/2022/11/seac-scientific-steering-committee-on.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://bovineprp.blogspot.com/2022/11/seac-scientific-steering-committee-on.html</a><br /></div><div dir="ltr"><br /></div></div><div dir="ltr" style="font-family: inherit;">iatrogenic TSE PrP</div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><a fg_scanned="1" href="https://itseprion.blogspot.com/" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://itseprion.blogspot.com/</a><br /></div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;">NOW LET'S LOOK AT THE LATEST ON TSE PRION ZOONOSIS RISK FACTORS</div><div dir="ltr" style="font-family: inherit;"><br /></div><div dir="ltr" style="font-family: inherit;"><div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***thus questioning the origin of human sporadic cases. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">=============== </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***thus questioning the origin of human sporadic cases*** </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">=============== </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">============== </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">PRION 2015 CONFERENCE</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/</a><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a> </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">PRION 2016 TOKYO</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Saturday, April 23, 2016</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Taylor & Francis</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Prion 2016 Animal Prion Disease Workshop Abstracts</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">WS-01: Prion diseases in animals and zoonotic potential</div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><a fg_scanned="1" href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a><br /></div></div><div style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><br /></div><div dir="ltr" style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS <br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla <br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;">Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.<br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><br /></div><div style="color: #111111; font-family: sans-serif; font-size: 16px; min-height: 1.25em;"><a fg_scanned="1" href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><br /></div><div dir="ltr" style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><div><div><div>PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS </div><div><br /></div><div>Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.</div><div><br /></div><div>Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.</div><div><br /></div><div>Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.</div><div><br /></div><div>Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.</div><div><br /></div><div>Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.</div><div><br /></div><div>Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada. Grant number: 1R01NS121016-01; 201,600,023</div><div><br /></div><div>Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.</div><div><br /></div><div>Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD</div><div><br /></div><div>Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha</div><div><br /></div><div>aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada</div><div><br /></div><div>Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.</div><div><br /></div><div>Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.</div><div><br /></div><div>Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.</div><div><br /></div><div>Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.</div><div><br /></div><div>Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.</div><div><br /></div><div>Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.</div><div><br /></div><div>Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.</div><div><br /></div><div>The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons</div><div><br /></div><div>Eric Cassmanna, Xu Qib, Qingzhong Kongb, and Justin Greenleea</div><div><br /></div><div>aNational Animal Disease Center, Agricultural Research Service, US Department of Agriculture, Ames, IA, USA bDepartments of Pathology, Neurology, National Center for Regenerative Medicine, and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA</div><div><br /></div><div>Aims: Evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer host.</div><div><br /></div><div>Material and Methods: Pooled brain material (GG96) from a CWD positive herd was used to oronasally inoculate two white-tailed deer with wild-type prion protein genotype and intracranially inoculate a raccoon. Brain homogenates (10% w/v) from the raccoon and the two white-tailed deer were used to intracranially inoculate separate groups of transgenic mice that express human prion protein with methionine (M) at codon 129 (Tg40h). Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue.</div><div><br /></div><div>Results: Humanized transgenic mice inoculated with the raccoon passaged CWD agent from white-tailed deer exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPScwas detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPScalso was detected in brain tissue by western blot and immunohistochemistry. No PrPScwas detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from white-tailed deer did not have detectable PrPScusing conventional immunoassay techniques.</div><div><br /></div><div>Conclusions: The host range of the CWD agent from white-tailed deer was expanded in our experimental model after one passage through raccoons.</div><div><br /></div><div>Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.</div><div><br /></div><div>Acknowledgement: We thank Quazetta Brown, Lexi Frese, Rylie Frese, Kevin Hassall, Leisa Mandell, and Trudy Tatum for providing excellent technical support to this project.</div><div><br /></div><div>Stable and highly zoonotic cervid prion strain is possible</div><div><br /></div><div>Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, and Qingzhong Kong Department of Pathology, Case Western Reserve University, Cleveland, USA</div><div><br /></div><div>Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in some areas. Multiple in vitro conversion experiments and in vivo animal studies suggest that the CWD-to-human transmission barrier is not unbreakable. A major public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.</div><div><br /></div><div>Material and Methods: We inoculated a few sCJD brain samples into cervidized transgenic mice, which were intended as negative controls for bioassays of brain tissues from sCJD cases who had hunted or consumed vension from CWD-endemic states. Some of these mice became infected and their brain tissues were further examined by serial passages in humanized or cervidized mice.</div><div><br /></div><div>Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a ‘cervidized’ CJD strain that we termed CJDElkPrP. We observed 100% transmission of CJDElkPrPin transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrPtwo more times in the Tg12 mice. We found that such second and third passage CJDElkPrPprions also led to 100% infection in the Tg40h mice. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice, despite that natural elk CWD isolates and CJDElkPrPshare the same elk PrP sequence.</div><div><br /></div><div>Conclusions: Our data demonstrate that highly zoonotic cervid prion strains are not only possible but also can be stably maintained in cervids and that CWD zoonosis is prion strain-dependent.</div><div><br /></div><div>Funded by: NIH</div><div><br /></div><div>Grant number: R01NS052319, R01NS088604, R01NS109532</div><div><br /></div><div>Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O’Rourke for providing the sCJD samples and the CWD samples, respectively.</div><div><br /></div><div>Adaptation of chronic wasting disease (CWD) prion strains in hosts with different PRNP genotypes</div><div><br /></div><div>Camilo Duque Velasqueza,c, Elizabeth Triscotta,c, Chiye Kima,c, Diana Morenoa,c, Judd Aikenb,c, and Debbie McKenziea,c</div><div><br /></div><div>aDepartment of Biological Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; bDepartment of Agriculture, Food & Nutritional Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; cCentre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2M8, Canada</div><div><br /></div><div>Aims: The contagious nature of CWD epizootics and the PrPCamino acid variation of cervids (and susceptible sympatric species) guarantee the expansion of prion conformational diversity and selective landscapes where new strains can arise. CWD strains can have novel transmission properties including altered host range that may increase zoonotic risk as circulating strains diversify and evolve. We are characterizing the host adaptability of characterized CWD strains as well as CWD isolates from different cervid species in various enzootic regions.</div><div><br /></div><div>Material and Methods: Characterized CWD strains as well as a number of isolates from hunter-harvested deer were bioassayed in our rodent panel (transgenic mice expressing cervid alleles G96, S96 and H95-PrPC, elk PrPC, bovine PrPC, and both hamsters and non-transgenic laboratory mice). Strain characteristics were compared using computer based scoring of brain pathology (e.g. PrPCWDbrain distribution), western blot and protein misfolding cyclic amplification (PMCA).</div><div><br /></div><div>Results: Transmission of various isolates resulted in the selection of strain mixtures in hosts expressing similar PrPC, particularly for polymorphic white-tailed deer and for Norwegian reindeer. As of the second passage, transmission of P153 moose prions from Norway has not resulted in emergence of strains with properties similar to any North American CWD strains in our taxonomic collection (Wisc-1, CWD2, H95+and 116AG).</div><div><br /></div><div>Conclusions: Our data indicates polymorphic white-tailed deer can favor infection with more than one strain. Similar to transmission studies of Colorado CWD isolates from cervids expressing a single PrPCprimary structure, the isolate from Norway reindeer (V214) represents a strain mixture, suggesting intrinsic strain diversity in the Nordfjella epizootic. The diversity of CWD strains with distinct transmission characteristics represents a threat to wildlife, sympatric domestic animals and public health.</div><div><br /></div><div>Funded by: Genome Canada and Genome Alberta (Alberta Prion Research Institute and Alberta Agriculture & Forestry); NSERC Grant number: #LSARP 10205; NSERC RGPIN-2017-05539</div><div><br /></div><div>Acknowledgement: We would like to thank Margo Pybus (Alberta Environment and Parks) Trent Bollinger (University of Saskatchewan) for providing us with tissue samples from hunter-harvested deer and Sylvie Benestad for providing moose and reindeer samples.</div><div><br /></div><div>Application of PMCA to understand CWD prion strains, species barrier and zoonotic potential</div><div><br /></div><div>Sandra Pritzkowa, Damian Gorskia, Frank Ramireza, Fei Wanga, Glenn C. Tellingb, Justin J. Greenleec, Sylvie L. Benestadd, and Claudio Sotoa aDepartment of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA; bDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA; cVirus and Prion Research Unit, United States Department of Agriculture, Ames, Iowa, USA; dNorwegian Veterinary Institute, OIE Reference Laboratory for CWD, Ås, Norway</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) is a prion disease affecting various species of cervids that continues to spread uncontrollably across North America and has recently been detected in Scandinavia (Norway, Sweden and Finland). The mechanisms responsible for the natural transmission of CWD are largely unknown. Furthermore, the risk of CWD transmission to other species, including humans, is also unknown and remains a dangerous enigma. In this study, we investigated the potential of CWD prions to infect several other animal species (sheep, cattle, pig, hamster, and mouse) including humans, by examining their capacity to convert the normal prion protein of distinct species in a PMCA reaction. Moreover, we also investigated whether the in vivo passage of CWD through intermediate species alters their capacity for zoonotic transmission, which may represent a major hazard to human health.</div><div><br /></div><div>Material and Methods: For these studies, we used brain material from CWD-infected white-tailed deer (Odocoileus virginianus), elk (Cervus canadensis), and mule deer (Odocoileus hemionus) as species native to North America. We also used CWD-infected Moose (Alces alces), reindeer (Rangifer tarandus) and red deer (Cervus elaphus) as Norwegian cervids. We also used brains from cattle, sheep and pigs experimentally infected by CWD. To study interspecies-transmission and zoonotic potential, samples were tested via PMCA for the conversion of PrPCinto PrPScusing different combinations of inoculum and host species. Based on these analyses we estimated the spillover and zoonotic potential for different CWD isolates. We define and quantify spillover and zoonotic potential indices as the efficiency by which CWD prions sustain prion generation in vitro at the expense of normal prion proteins from various mammals and human, respectively.</div><div><br /></div><div>Results: Our results show that prions from some cervid species, especially those found in Northern Europe, have a higher potential to transmit disease characteristics to other animals. Conversely, CWD-infected cervids originated in North America appear to have a greater potential to generate human PrPSc. We also found that in vivo transmission of CWD to cattle, but not to sheep or pigs substantially increases the ability of these prions to convert human PrPCby PMCA.</div><div><br /></div><div>Conclusions: Our findings support the existence of different CWD prion strains with distinct spillover and zoonotic potentials. We also conclude that transmission of CWD to other animal species may increase the risk for CWD transmission to humans. Our studies may provide a tool to predict the array of animal species that a given CWD prion could affect and may contribute to understanding the risk of CWD for human health.</div><div><br /></div><div>Funded by: National Institute of Health Grant number: P01 AI077774</div><div><br /></div><div>Generation of human chronic wasting disease in transgenic mice</div><div><br /></div><div>Zerui Wanga, Kefeng Qinb, Manuel V. Camachoa, Ignazio Cali a,c, Jue Yuana, Pingping Shena, Tricia Gillilanda, Syed Zahid Ali Shaha, Maria Gerasimenkoa, Michelle Tanga, Sarada Rajamanickama, Anika Yadatia, Lawrence B. Schonbergerd, Justin Greenleee, Qingzhong Konga,c, James A. Mastriannib, and Wen-Quan Zoua,c</div><div><br /></div><div>aDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA; bDepartment of Neurology and Center for Comprehensive Care and Research on Memory Disorders, the University of Chicago Pritzker School of Medicine, Chicago, USA; cNational Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; dDivision of High-Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA, USA; eVirus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, Ames, IA, USA</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) results from the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC) in the brains of deer and elk. It has been spreading rapidly throughout many regions of North America, exported inadvertently to South Korea, and more recently identified in Europe. Mad cow disease has caused variant Creutzfeldt-Jakob disease (vCJD) in humans and is currently the only known zoonotic prion disease. Whether CWD is transmissible to humans remains uncertain. The aims of our study were not only to confirm whether CWD prion isolates can convert human brain PrPCinto PrPScin vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPScis infectious.</div><div><br /></div><div>Material and Methods: Eight CWD prion isolates from 7 elks and 1 deer were used as the seeds while normal human brain homogenates containing either PrP-129 MM (n = 2) or PrP-129 VV (n = 1) were used as the substrates for sPMCA assay. A normal elk brain tissue sample was used as a negative control seed. Two lines of humanized transgenic (Tg) mice expressing either human PrP-129VV or −129 MM polymorphism were included for transmission studies to determine the infectivity of PMCA-amplified PrPSc. Wester blotting and immunohistochemistry and hematoxylin & eosin staining were used for determining PrPScand neuropathological changes of inoculated animals.</div><div><br /></div><div>Results: We report here the generation of the first CWD-derived infectious human PrPScusing elk CWD PrPScto initiate conversion of human PrPCfrom normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPScwas derived from the human brain PrPCsubstrate. Two lines of humanized transgenic mice expressing human PrPCwith either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPScpatterns and neuropathological changes in the brain.</div><div><br /></div><div>Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSchas the potential to overcome the species barrier and directly convert human PrPCinto infectious PrPScthat can produce bona fide prion disease when inoculated into humanized transgenic mice.</div><div><br /></div><div>Funded by: CJD Foundation and NIH</div><div><br /></div><div>Mortality surveillance of persons potentially exposed to chronic wasting disease</div><div><br /></div><div>R.A. Maddoxa, R.F. Klosb, L.R. Willb, S.N. Gibbons-Burgenerb, A. Mvilongoa, J.Y. Abramsa, B.S. Applebyc, L.B. Schonbergera, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bWisconsin Department of Health Services (WDHS), Division of Public Health, Madison, USA; cNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div><br /></div><div>Aims: It is unknown whether chronic wasting disease (CWD), a prion disease of cervids, can infect people, but consumption of meat from infected animals would be the most likely route of transmission. Wisconsin Department of Health Services, Division of Public Health (WDHS) personnel maintain a database consisting of information collected from hunters who reported eating, or an intention to eat, venison from CWD-positive cervids. These data, collected since 2003, allow for the evaluation of causes of mortality in individuals potentially exposed to CWD.</div><div><br /></div><div>Material and Methods: The WDHS database contains the name, date of birth, when available, year of CWD-positive deer harvest, and city and state of residence for each potentially exposed individual. The database also includes information on how the deer was processed (self-processed or by a commercial operator) and when applicable, names of others with whom the venison was shared. Duplicate entries (i.e., those who consumed venison from CWD-positive deer in multiple hunt years) are determined by first name, last name, and date of birth. All names in the database are cross-checked with reported cases of human prion disease in Wisconsin and cases in the National Prion Disease Pathology Surveillance Center (NPDPSC) diagnostic testing database. Persons with date of birth available are also cross-checked with prion disease decedents identified through restricted-use national multiple cause-of-death data via a data use agreement with the National Center for Health Statistics (NCHS).</div><div><br /></div><div>Results: The database currently consists of 1561 records for hunt years 2003–2017 and 87 additional records for 2018–2019. Of these, 657 records have accompanying date of birth; 15 entries were removed as duplicates leaving 642 unique individuals. Of these individuals, 278 of 426 (66%) who ate venison from a CWD-positive deer and provided processing information reported self-processing. No matches were found among any persons in the database cross-checked with WDHS human prion disease surveillance data, NPDPSC data (February 2022 update), and NCHS data through 2020.</div><div><br /></div><div>Conclusions: Because of the linkage of person and CWD-positive animal in the WDHS database, reviewing the cause of mortality in potentially exposed persons is possible. The number of individuals cross-checked so far is likely only a small percentage of those potentially exposed to CWD in Wisconsin, and many more years of vital status tracking are needed given an expected long incubation period should transmission to humans occur. Nevertheless, the findings of this ongoing review are thus far reassuring.</div><div><br /></div><div>Prion disease incidence, United States, 2003–2020</div><div><br /></div><div>R.A. Maddoxa, M.K. Persona, K. Kotobellib, A. Mvilongoa, B.S. Applebyb, L.B. Schonbergera, T.A. Hammetta, J.Y. Abramsa, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA</div><div><br /></div><div>Aims: Mortality data, in conjunction with neuropathological and genetic testing results, are used to estimate prion disease incidence in the United States.</div><div><br /></div><div>Material and Methods: Prion disease decedents for 2003–2020 were identified from restricted-use U.S. national multiple cause-of-death data, via a data use agreement with the National Center for Health Statistics, and from the National Prion Disease Pathology Surveillance Center (NPDPSC) database. NPDPSC decedents with neuropathological or genetic test results positive for prion disease for whom no likely match was found in the NCHS multiple cause-of-death data were added as cases for incidence calculations, while those with negative neuropathology results but with cause-of-death data indicating prion disease were removed. Unmatched cases in the NPDPSC database lacking neuropathological testing but with a positive real-time quaking-induced conversion (RT-QuIC) test result were additionally assessed. Age-specific and age-adjusted average annual incidence rates were calculated from the combined data; the year 2000 as the standard population and the direct method were used for age-adjustment.</div><div><br /></div><div>Results: A total of 7,921 decedents were identified as having prion disease during 2003–2020 for an age-adjusted average annual incidence of 1.2 per million population. The age-adjusted incidence between males and females (1.3 and 1.1 per million, respectively) differed significantly (p < 0.0001). The age-specific average annual incidence among those <55 and ≥55 years of age was 0.2 and 4.8 per million, respectively; incidence among those ≥65 was 6.1 per million. Eighteen cases were <30 years of age for an age-specific incidence of 8.0 per billion; only 6 of these very young cases were sporadic (3 sporadic CJD, 3 sporadic fatal insomnia), with the rest being familial (9), variant (2), or iatrogenic (1). The age-adjusted annual incidence for the most recent year of data, 2020, was 1.3 per million. However, assessment of RT-QuIC positive cases lacking neuropathology in the NPDPSC database suggested that approximately 20% more cases may have occurred in that year; the addition of a subset of these cases that had date of death information available (n = 44) increased the 2020 rate to 1.4 per million.</div><div><br /></div><div>Conclusions: Mortality data supplemented with the results of neuropathological, CSF RT-QuIC, and genetic testing can be used to estimate prion disease incidence. However, the identification in the NPDPSC database of RT-QuIC-positive cases lacking date of death information suggests that this strategy may exclude a number of probable prion disease cases. Prion disease cases <30 years of age, especially those lacking a pathogenic mutation, continue to be very rare.</div><div><br /></div><div>Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer</div><div><br /></div><div>Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera</div><div><br /></div><div>aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.</div><div><br /></div><div>Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.</div><div><br /></div><div>Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.</div><div><br /></div><div>Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.</div><div><br /></div><div>Funded by: National Institutes of Health (NIH)</div><div><br /></div><div>Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM</div><div><br /></div><div>Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies</div><div><br /></div><div>Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study</div><div><br /></div><div>Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa</div><div><br /></div><div>aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA</div><div><br /></div><div>Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.</div><div><br /></div><div>Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.</div><div><br /></div><div>Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.</div><div><br /></div><div>Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.</div><div><br /></div><div>Funded by: USDA</div><div><br /></div><div>Grant number: AP20VSSPRS00C143</div><div><br /></div><div>ATYPRION project: assessing the zoonotic potential of interspecies transmission of CWD isolates to livestock (preliminary results).</div><div><br /></div><div>Enric Vidala,b, Juan Carlos Espinosac, Samanta Gilera,b, Montserrat Ordóñeza,b, Guillermo Canteroa,b, Vincent Béringued, Justin J. Greenleee, and Juan Maria Torresc</div><div><br /></div><div>aUnitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; bIRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; cCentro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid, Spain; dMolecular Virology and Immunology, French National Research Institute for Agriculture, Food and Environment (INRAE), Université Paris-Saclay, Jouy-en-Josas, France; eVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA</div><div><br /></div><div>Aims: Since variant Creutzfeldt-Jackob disease was linked to the consumption of bovine spongiform encephalopathy prions, the study of the pathobiological features of animal prions, particularly their zoonotic potential, is of great concern to the scientific community and public health authorities. Furthermore, interspecies transmission of prions has been demonstrated as a putative evolutionary mechanism for prions, that can lead to the emergence of new features including the ability to infect humans. For instance, small ruminants’ atypical scrapie prions, when propagated in a bovine or porcine host, can shift to a classical BSE phenotype thus posing a potential risk in case of human exposure. So far, no hard evidence of zoonotic transmission of cervids’ chronic wasting disease (CWD) to humans has been published, however experimental transmission to bovine, ovine and caprine hosts has been achieved. Our goal is to investigate if, once passaged through these domestic species, CWD prions might become infectious to humans.</div><div><br /></div><div>Material and Methods: Different CWD isolates experimentally adapted to cattle, sheep and goat (Hamir et al, 2005, 2006, 2007, Greenlee et al 2012) have been intracerebrally inoculated to transgenic mouse models expressing the human cellular prion protein either homozygous for methionine or valine at codon 129 (Tg340-Met129 and Tg362-Val129). Additionally, inocula obtained from experimental transmission of elk CWD to ovinized (Tg501) and bovinized (BoTg110) transgenic mice, as well as white-tailed deer CWD to BoTg110 mice, are currently being bioassayed in both human PrPCtransgenic models.</div><div><br /></div><div>Results and conclusions: No evidence of transmission has been found on first passage for bovine adapted elk and mule deer CWD to none of the humanized models. The remaining bioassays are ongoing without showing clinical signs yet, as well as second passages for the negative 1stpassages.</div><div><br /></div><div>Funded by: La Marató de TV3 foundation. Grant number: ATYPRION (201,821–30-31-32)</div><div><br /></div><div><a fg_scanned="1" href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div><br /></div><div><div>PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS and ENVIRONMENTAL FACTORS </div><div><br /></div><div>Chronic wasting disease detection in environmental and biological samples from a taxidermy site</div><div><br /></div><div>Paulina Sotoa,b, J. Hunter Reedc, Mitch Lockwoodc, and Rodrigo Moralesa,b aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bUniversidad Bernardo O’Higgins, Santiago, Chile; cTexas Parks and Wildlife Department, Texas, USA </div><div><br /></div><div>Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy affecting captive and free-ranging cervids (e.g., mule deer, white-tailed deer, elk, reindeer, and moose). Nowadays, CWD is widely distributed in North America. It is suggested that CWD spreads due to direct animal contact or through exposure to contaminated environments previously inhabited by infected animals. CWD may also be spread through the movement of infected animals and carcasses. Taxidermy practices involve processing deer tissues (or whole animal carcasses). In many cases, the CWD status of processed animals is unknown. This can generate risks of disease spread and transmission. Taxidermy practices include different steps involving physical, chemical, and biological procedures. Without proper tissue handling or disposal practices, taxidermist facilities may become a focus of prion infectivity. Aims: In this study, we evaluated the presence of infectious prions in a taxidermy facility believed to be exposed to CWD. Detection was performed using the Protein Misfolding Cyclic Amplification (PMCA) technique in biological and inert environmental samples. Methods: We collected biological and environmental samples (plants, soils, insects, excreta, and others) from a taxidermy facility, and we tested these samples using the PMCA technique. In addition, we swabbed different surfaces possibly exposed to CWD-infected animals. For the PMCA reaction, we directly used a swab piece or 10 µL of 20% w/v homogenized samples. Results: The PMCA analysis demonstrated CWD seeding activity in some of the components of this facility, including insects involved in head processing, soils, and a trash dumpster. Conclusions: Different areas of this property were used for various taxidermy procedures. We were able to detect the presence of prions in i) soils that were in contact with the heads of dead animals, ii) insects involved in the cleaning of skulls, and iii) an empty dumpster where animal carcasses were previously placed. This is the first report demonstrating that swabbing is a helpful method to screen for prion infectivity on surfaces potentially contaminated with CWD. These findings are relevant as this swabbing and amplification strategy may be used to evaluate the disease status of other free-ranging and captive settings where there is a concern for CWD transmissions, such as at feeders and water troughs with CWD-exposed properties. This approach could have substantial implications for free-ranging cervid surveillance as well as in epidemiological investigations of CWD. </div><div><br /></div><div>Funded by: USDA Grant number: AP20VSSPRS00C143 </div><div><br /></div><div>Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study </div><div><br /></div><div>Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA </div><div><br /></div><div>Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported. Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques. Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas. Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening. </div><div><br /></div><div>Funded by: USDA Grant number: AP20VSSPRS00C143 </div><div><br /></div><div>Protein misfolding cyclic amplification (PMCA) as an ultra-sensitive technique for the screening of CWD prions in different sample types </div><div><br /></div><div>Francisca Bravo‐Risia,b, Paulina Sotoa,b, Rebeca Benaventea, Hunter Reedc, Mitch Lockwoodc, Tracy Nicholsd, and Rodrigo Moralesa,b aDepartment of Neurology, The University of Texas Health Science Center at Houston, Houston, TX, USA; bCentro Integrativo de Biologia y Quimica Aplicada (CIBQA), Universidad Bernardo O’Higgins, Santiago, Chile; cTexas Park and Wildlife Department, Texas, USA; dVeterinary Services Cervid Health Program, United States Department of Agriculture, Animal and Plant Health Inspection Service, Fort Collins, Colorado, USA </div><div><br /></div><div>Chronic wasting disease (CWD) is a prion disease that affects farmed and free-ranging cervids. The infectious agent in CWD is a misfolded form of the prion protein (PrPSc) that promotes conformational changes in the host’s cellular prion protein (PrPC). Currently, definitive CWD status is confirmed in the brain and lymphoid tissues by immunohistochemistry. The limitation of this technique is its poor sensitivity. Protein misfolding cyclic amplification (PMCA) and real-time quaking-induced conversion (RT- QuIC) are ultra-sensitive techniques that overcome these issues. PMCA mimics the self- propagation of infectious prions in vitro through multiple incubation/sonication cycles, increasing the number of prion particles present in a given sample. The detection of proteinase K (PK) -resistant PrPScby PMCA has been performed in experimental and natural samples that might harbor subclinical levels of prions. These samples include several tissues, bodily fluids, excreta, and different manmade and natural materials, including mineral licks, soils, and plants. Aims: In this study, we highlight recent advances and contributions that our group has performed in the detection of CWD prions from samples collected in farmed and free-ranging cervids, as well as other specimens involving the environment that contains CWD-infected deer. Material and Methods: A set of diverse samples analyzed in this study were collected by USDA and TPWD personnel in breeding and taxidermy facilities, and deer breeding facilities. These included animal and environmental samples. Additional samples from free-ranging animals were provided by hunters. Results: The diverse range of samples successfully detected for CWD prion infection in this study include blood, semen, feces, obex, retropharyngeal lymph node, fetuses (neural and peripheral tissues) and gestational tissues, parasites, insects, plants, compost/soil mixtures, and swabs from trash containers. Importantly, these results helped to identify seeding-competent prions in places reported to be free of CWD. The levels of prion infectivity in most of these samples are currently being investigated. Conclusions: Our findings contribute to the understanding of the transmission dynamics and prevalence of CWD. In addition, our data have helped to identify CWD in areas previously considered to be free of CWD. We also demonstrate that PMCA is a powerful technique for the screening of biological and environmental samples. Overall, our research suggests that PMCA may be a useful tool to implement for the surveillance and management of CWD. Funded by: NIH/NIAID and USDA Grant number: 1R01AI132695 (NIH) and AP20VSSPRS00C143 (USDA) </div><div><br /></div><div>Nasal bot: an emerging vector for natural chronic wasting disease transmission </div><div><br /></div><div>Paulina Sotoa,b, Francisca Bravo-Risia,b, Carlos Kramma, Nelson Pereza, Rebeca Benaventea, J. Hunter Reedc, Mitch Lockwoodc, Tracy A. Nicholsd, and Rodrigo Moralesa,b aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bUniversidad Bernardo O’Higgins, Santiago, Chile; cTexas Park and Wildlife Department, Texas, USA; dVeterinary Services Cervid Health Program, United States Department of Agriculture, Animal and Plant Health Inspection Service, Fort Collins, Colorado, USA </div><div><br /></div><div>Chronic wasting disease (CWD) is a fatal neurodegenerative disease that affects farmed and free-ranging cervids populations. The spread of CWD in cervids is thought to occur through the direct contact between cervids or through the exposure of naïve animals to contaminated environments. Parasites are known vectors of multiple diseases in animals. However, the potential role of parasites in CWD transmission remains unclear. Aims: The main objective of this study was to determine if CWD prions could be detected in the larvae of deer nasal bot flies, a common deer parasite, taken from CWD-infected white-tailed deer (Odocoileus virginianus). Methods: Bot fly larvae were collected from the nasal cavity of naturally infected CWD- positive or CWD non-detect white-tailed deer. The CWD seeding activity of the larvae was interrogated by PMCA. Prion infectivity was also evaluated in cervidized transgenic mouse bioassay (intra-cerebral administration in Tg1536 mice). Mice inoculated with bot larvae homogenate were sacrificed when they showed established signs of prion disease, or at extended periods after treatment (600 days). All inoculated mouse brains were evaluated for protease resistant prions to confirm clinical or sub-clinical infection. Bot larvae from CWD non-detect deer were used as controls. To further mimic environmental transmission, bot larvae homogenates were mixed with soils and plants were grown on them. Both plants and soils were tested for prion seeding activity. Results: PMCA analysis demonstrated CWD seeding activity in nasal bot larvae from captive and free-ranging white-tailed deer. CWD-contaminated bots efficiently infected transgenic mice, with attack rates and incubation periods suggesting high infectivity titers. Further analyses of treated animals (biochemical characterization of protease resistant prions and immunohistochemistry) confirmed prion infection. Analyses on dissected parts of the bot larvae demonstrate that the infectivity is concentrated in the larvae cuticle (outer part). Nasal bot larvae extracts mixed with</div><div><br /></div><div> soils showed seeding activity by PMCA. Interestingly, plants grown in soil contaminated with the nasal bot larvae extract were found to produce seeding activity by PMCA. Conclusion: In this study we described for the first time that deer nasal bot larvae from CWD-infected deer carry high CWD infectivity titers. We also demonstrate that CWD prions in these parasites can interact with other environmental components relevant for disease transmission. Considering this information, we propose that deer nasal bot larvae could act as vectors for CWD transmission in wild and farming settings. Funded by: NIH/NIAID and USDA/APHIS Grant number: R01AI132695 and AP20VSSPRS00C143 PRION 2022 ABSTRACTS, AND A BIG THANK YOU TO On behalf of the Prion2020/2022 Congress Organizing Committee and the NeuroPrion Association, we heartily invite you to join us for the International Conference Prion2020/2022 from 13.-16. September 2022 in Göttingen.</div><div><br /></div><div>Prion 2022 Conference abstracts: pushing the boundaries</div><div><br /></div><div><a fg_scanned="1" href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a><br /></div><div><br /></div><div>Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer</div><div><br /></div><div>Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera</div><div><br /></div><div>aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK</div><div><br /></div><div>Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.</div><div><br /></div><div>Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.</div><div><br /></div><div>Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.</div><div><br /></div><div>Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.</div><div><br /></div><div>Funded by: National Institutes of Health (NIH)</div><div><br /></div><div>Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM</div><div><br /></div><div>Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies</div><div><br /></div><div>Carrot plants as potential vectors for CWD transmission</div><div><br /></div><div>Paulina Sotoa,b, Francisca Bravo-Risia,b, Claudio Sotoa, and Rodrigo Moralesa,b</div><div><br /></div><div>aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bUniversidad Bernardo O’Higgins, Santiago, Chile</div><div><br /></div><div>Prion diseases are infectious neurodegenerative disorders afflicting humans and other mammals. These diseases are generated by the misfolding of the cellular prion protein into a disease-causing isoform. Chronic wasting disease (CWD) is a prevalent prion disease affecting cervids (captive and free-range). CWD is thought to be transmitted through direct animal contact or by indirect exposure to contaminated environments. Many studies have shown that infectious prions can enter the environment through saliva, feces, or urine from infected animals and decaying carcasses. However, we do not fully understand the specific contribution of each component to disease transmission events. Plants are logical environmental components to be evaluated since they grow in environments contaminated with CWD prions and are relevant for animal and human nutrition.</div><div><br /></div><div>Aims: The main objective of this study is to study whether prions are transported to the roots and leaves of carrots, an edible plant commonly used in the human diet and as deer bait.</div><div><br /></div><div>Methods: We have grown carrot plants in CWD-infected soils. After 90 days, we harvested the carrots and separated them from the leaves. The experiment was controlled by growing plants in soil samples treated with brain extracts from healthy animals. These materials were interrogated for their prion seeding activity using the Protein Misfolding Cyclic Amplification (PMCA) technique. Infectivity was evaluated in mouse bioassays (intracerebral injections in Tg1536 mice). The animals were sacrificed when they showed established signs of prion disease. Animals not displaying clinical signs were sacrificed at 600 days post-inoculation.</div><div><br /></div><div>Results: The PMCA analysis demonstrated CWD seeding activity in soils contaminated with CWD prions, as well as in carrot plants (leaves and roots) grown on them. Bioassays demonstrated that both leaves and roots contained CWD prions in sufficient quantities to induce disease (92% attack rate). As expected, animals treated with prion-infected soils developed prion disease at shorter incubation periods (and complete attack rates) compared to plant components. Animals treated with soil and plant components exposed with CWD-free brain extracts did not display prion-associated clinical signs or evidence of sub-clinical prion infection.</div><div><br /></div><div>Conclusions: We show that edible plant components can absorb prions from CWD contaminated soils and transport them to their aerial parts. Our results indicate that plants could participate as vectors of CWD transmission. Importantly, plants designated for human consumption represent a risk of introducing CWD prions into the human food chain.</div><div><br /></div><div>Funded by: NIH</div><div><br /></div><div>Grant number: R01AI132695</div><div><br /></div><div><a fg_scanned="1" href="https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286</a></div></div><div><br /></div><div><div><div><div>8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div><br /></div><div><a fg_scanned="1" href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a><br /></div></div><div><br /></div><div><a fg_scanned="1" href="https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://efsaopinioncwd.blogspot.com/2022/04/efsa-eu-request-for-scientific-opinion.html</a><br /></div><div><br /></div></div><div><div>''These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.''</div><div><br /></div><div><div style="font-size: 10pt;">Prion Conference 2018 Abstracts</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">BSE aka MAD COW DISEASE, was first discovered in 1984, and it took until 1995 to finally admit that BSE was causing nvCJD, the rest there is history, but that science is still evolving i.e. science now shows that indeed atypical L-type BSE, atypical Nor-98 Scrapie, and typical Scrapie are all zoonosis, zoonotic for humans, there from. </div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">HOW long are we going to wait for Chronic Wasting Disease, CWD TSE Prion of Cervid, and zoonosis, zoonotic tranmission to humans there from?</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Studies have shown since 1994 that humans are susceptible to CWD TSE Prion, so, what's the hold up with making CWD a zoonotic zoonosis disease, the iatrogenic transmissions there from is not waiting for someone to make a decision.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Prion Conference 2018 Abstracts</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Background</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Methods</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Results</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Conclusions</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">=====</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">P172 Peripheral Neuropathy in Patients with Prion Disease</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Wang H(1), Cohen M(1), Appleby BS(1,2)</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">=====</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Background</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Methods</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Results</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Conclusions</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">=====</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">P180 Clinico-pathological analysis of human prion diseases in a brain bank series</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Background and objective:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Methods:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Results:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Discussion:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html</a><br clear="none" /></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">=====</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Aims:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Methods:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Results:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Conclusions:</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">=====</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><div>WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice</div><div><br clear="none" /></div><div>Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)</div><div><br clear="none" /></div><div>(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.</div><div><br clear="none" /></div><div>To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.</div><div><br clear="none" /></div><div>See also poster P103</div><div><br clear="none" /></div><div>***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.<br clear="none" /></div><div><br clear="none" /></div><div>=====</div><div><br clear="none" /></div><div>WA16 Monitoring Potential CWD Transmission to Humans</div><div><br clear="none" /></div><div>Belay ED</div><div><br clear="none" /></div><div>Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.</div><div><br clear="none" /></div><div>The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.</div><div><br clear="none" /></div><div>=====</div><div><br clear="none" /></div><div>P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan</div><div><br clear="none" /></div><div>Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)</div><div><br clear="none" /></div><div>(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.</div><div><br clear="none" /></div><div>Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.</div><div><br clear="none" /></div><div>=====</div></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;">Source Prion Conference 2018 Abstracts</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html</a><br clear="none" /></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html</a><br clear="none" /></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="http://prionconference.blogspot.com/2018/" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">http://prionconference.blogspot.com/2018/</a></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><div>Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions</div><div><br clear="none" /></div><div>Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)</div><div><br clear="none" /></div><div>Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.</div><div><br clear="none" /></div><div>snip...</div><div><br clear="none" /></div><div>Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).</div><div><br clear="none" /></div><div>A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.</div><div><br clear="none" /></div><div>The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.</div><div><br clear="none" /></div><div>In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).</div><div><br clear="none" /></div><div>The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.</div><div><br clear="none" /></div><div>Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.</div><div><br clear="none" /></div><div>Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.</div><div><br clear="none" /></div><div>This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.</div><div><br clear="none" /></div><div>Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.</div><div><br clear="none" /></div><div><a fg_scanned="1" href="https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article</a><br clear="none" /></div><div><br clear="none" /></div><div><a fg_scanned="1" href="https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion</a><br clear="none" /></div></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><div style="background-color: whitesmoke; color: #222222; font-family: arial, helvetica; font-size: 16px; margin-bottom: 24px;">Prion 2017 Conference Abstracts</div><div style="background-color: whitesmoke; font-family: arial, helvetica; font-size: 12px; margin-bottom: 24px;"><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 </span></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen </span><br clear="none" /></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">This is a progress report of a project which started in 2009. </span></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. </span><br clear="none" /></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. </span><br clear="none" /></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. </span><br clear="none" /></div><div style="margin-bottom: 24px;"><span style="color: #222222; font-size: 16px;">PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE</span></div></div></div><div style="font-size: 10pt;">8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.</div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132</a></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;">SATURDAY, FEBRUARY 23, 2019 </div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;">Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><a fg_scanned="1" href="https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html</a><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;">TUESDAY, NOVEMBER 04, 2014 </div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;">Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;">Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><a fg_scanned="1" href="http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html</a> </div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: small; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="background-color: whitesmoke; color: #222222; font-size: small; line-height: 1.22em;"><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Transmission Studies</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">snip.... </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><a href="https://web.archive.org/web/20090506002237/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species. </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><a fg_scanned="1" href="http://jvi.asm.org/content/83/18/9608.full" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">http://jvi.asm.org/content/83/18/9608.full</a> </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Prions in Skeletal Muscles of Deer with Chronic Wasting Disease </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure. </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><a fg_scanned="1" href="http://science.sciencemag.org/content/311/5764/1117..long" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">http://science.sciencemag.org/content/311/5764/1117..long</a> </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">From: TSS </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Subject: CWD aka MAD DEER/ELK TO HUMANS ???</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Date: September 30, 2002 at 7:06 am PST</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">From: "Belay, Ermias"</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Sent: Monday, September 30, 2002 9:22 AM</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Dear Sir/Madam,</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Ermias Belay, M.D. Centers for Disease Control and Prevention</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">-----Original Message-----</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">From: Sent: Sunday, September 29, 2002 10:15 AM</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">To: <a href="mailto:rr26k@nih.gov" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank" ymailto="mailto:rr26k@nih.gov">rr26k@nih.gov</a>; <a href="mailto:rrace@niaid.nih.gov" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank" ymailto="mailto:rrace@niaid.nih.gov">rrace@niaid.nih.gov</a>; <a href="mailto:ebb8@CDC.GOV" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank" ymailto="mailto:ebb8@CDC.GOV">ebb8@CDC.GOV</a></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">Thursday, April 03, 2008</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">snip...</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">snip... full text ; </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><a fg_scanned="1" href="http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html</a> </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">> However, to date, no CWD infections have been reported in people. </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-size: 12px; line-height: 1.22em;">sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;</div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">sporadic = 54,983 hits <a fg_scanned="1" href="https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic</a></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">spontaneous = 325,650 hits <a fg_scanned="1" href="https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; line-height: 1.22em;" target="_blank">https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous</a></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry </div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /></div><div style="font-family: Roboto, sans-serif; font-size: 12px; line-height: 1.22em;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div></div></div><div style="font-size: 10pt;"><br clear="none" /></div><div style="font-size: 10pt;"><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">> However, to date, no CWD infections have been reported in people.<br clear="none" /></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;">*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***</div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys" rel="nofollow noopener noreferrer" shape="rect" style="background-color: inherit; color: #222222;" target="_blank">http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys</a></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a fg_scanned="1" href="http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true" rel="nofollow noopener noreferrer" shape="rect" style="background-color: inherit; color: #222222;" target="_blank">http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true</a></div><div style="background-color: whitesmoke; color: #222222; font-family: Roboto, sans-serif; font-size: 16px; margin-bottom: 24px;"><a fg_scanned="1" href="https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article" rel="nofollow noopener noreferrer" shape="rect" style="background-color: inherit; color: #222222;" target="_blank">https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article</a></div></div></div><div><div style="font-size: 10pt;">CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Date: Fri, 18 Oct 2002 23:12:22 +0100 </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">From: Steve Dealler </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">To: BSE-L@ References: </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Dear Terry,</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">An excellent piece of review as this literature is desperately difficult to get back from Government sites.</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Steve Dealler =============== </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Table 9 presents the results of an analysis of these data.</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip...</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip...</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip...</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip...see full report ;</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a href="http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"> <a href="http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a href="http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Stephen Dealler is a consultant medical microbiologist <a href="mailto:deal@airtime.co.uk" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank" ymailto="mailto:deal@airtime.co.uk">deal@airtime.co.uk</a> </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">BSE Inquiry Steve Dealler</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Management In Confidence</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">BSE: Private Submission of Bovine Brain Dealler</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip...see full text;</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">MONDAY, FEBRUARY 25, 2019</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;">2004</span></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;"><br /></span></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;">Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD </span><br /></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;"><br /></span></span></div><div style="text-align: left;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium;">????: CBCnews</strong><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;"><br /></span></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;"><br /></span></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><span style="font-size: medium; font-weight: 700;"><a fg_scanned="1" href="https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-004/video-004.html</a><br /></span></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><br /></span></div><div style="text-align: left;"><span style="background-color: whitesmoke;">2004 </span></div><div style="text-align: left;"><span style="background-color: whitesmoke;"><br /></span></div><div style="text-align: left;"><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529article-body__date-source" style="color: #555555; font-size: 12px; letter-spacing: -0.04em; line-height: 1.5; margin: 0px;">April 22, 2004, 10:30 AM CDT</div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529article-body__date-source" style="color: #555555; font-size: 12px; letter-spacing: -0.04em; line-height: 1.5; margin: 0px;"><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529article-body__content" style="color: #2a2a2a; font-family: PublicoText, Georgia, Baskerville; font-size: 18px;"><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529">Guests: Patrick Singh, Terry Schwan, Janet Skarbek, Bill Fielding</div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529"><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529">(BEGIN VIDEOTAPE)</div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529"><br /></div><div id="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529taboolaReadMoreBelow"></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529">ANNOUNCER: DEBORAH NORVILLE TONIGHT.</div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529"><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp54d68d0eyiv2143574529"><a fg_scanned="1" href="https://www.nbcnews.com/id/wbna4806886" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://www.nbcnews.com/id/wbna4806886</a><br /></div></div></div><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium; text-align: left;"><div><strong><br /></strong></div>1997-11-10: Panorama - The British disease</strong><span style="text-align: left;"></span><div style="text-align: left;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium;"><br /></strong></div><div style="text-align: left;"><strong style="background-color: whitesmoke; font-family: Arial, Helvetica, sans-serif; font-size: medium;"><a fg_scanned="1" href="https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://histodb15.usz.ch/pages/Images/videos/video-009/video-009.html</a></strong></div></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a fg_scanned="1" href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a><br /></div><div style="font-size: 10pt;"><br /></div></div></div><div style="font-size: 10pt;"><br /></div></div><div><div>***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</div><div><br /></div><div>Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div><div><br /></div><div><a fg_scanned="1" href="https://www.nature.com/articles/srep11573" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.nature.com/articles/srep11573</a> </div></div></div><br /></div><div dir="ltr" style="color: black; font-family: arial; font-size: 13.3333px; text-align: justify;"><div><div style="font-size: 16px;"><div style="font-size: 13.3333px;"><div dir="ltr"><div><br /></div><div>Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission</div><div><br clear="none" /></div><div><a fg_scanned="1" href="https://www.regulations.gov/comment/APHIS-2021-0004-0002" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0004-0002</a></div><div><br clear="none" /></div><div><a href="https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf</a></div><div><br clear="none" /></div><div>Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification</div><div><br clear="none" /></div><div><a fg_scanned="1" href="https://www.regulations.gov/document/APHIS-2018-0011-0003" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://www.regulations.gov/document/APHIS-2018-0011-0003</a></div><div><br clear="none" /></div><div><a href="https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf</a></div></div><div dir="ltr"><br /></div></div></div><div style="font-size: 16px;"><pre style="font-size: 13.3333px; white-space: pre-wrap;"><div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; white-space: normal;"><span style="color: #333333; font-size: 14px;">APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission</span></div>
<div style="font-family: Helvetica, Arial, sans-serif; font-size: 16px; white-space: normal;"><h1 class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bh3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmt-0 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-1 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bfont-weight-bold yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-title" style="color: #333333; font-size: 24px; line-height: 1.42858; margin-left: 0px; margin-right: 0px; margin-top: 0px !important;">Comment from Singeltary Sr., Terry</h1><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-posted-text" style="color: #666666; font-size: 18px; line-height: 1.5;">Posted by the <span style="font-weight: 700;">Animal and Plant Health Inspection Service</span> on Sep 8, 2022</div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-posted-text" style="color: #666666; font-size: 18px; line-height: 1.5;"><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-posted-text" style="color: #666666; font-size: 18px; line-height: 1.5;"><a fg_scanned="1" href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank"></a><a fg_scanned="1" href="https://www.regulations.gov/comment/APHIS-2021-0010-0003" rel="nofollow noopener noreferrer" style="color: blue;" target="_blank">https://www.regulations.gov/comment/APHIS-2021-0010-0003</a><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-posted-text" style="color: #666666; font-size: 18px; line-height: 1.5;"><br /></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydp11c3e6a9yiv5901295807ydp82638dd5yiv1992879354ydp8cd4892fyiv4497094869ydp18f08d8fyiv8704346921ydpdb664e44yiv3324510546ydpcba0497byiv6076594264ydp474c308bjs-posted-text" style="color: #666666; font-size: 18px; line-height: 1.5;"><a href="https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://downloads.regulations.gov/APHIS-2021-0010-0003/attachment_1.pdf</a></div><div><br /></div></div></pre></div></div><div><div dir="ltr" style="font-family: Arial, Helvetica, sans-serif; font-size: 12px;"><pre style="font-size: 13.3333px; white-space: pre-wrap;"><div dir="ltr" style="font-family: arial;"><div dir="ltr"><div dir="ltr"><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div style="font-family: arial;">TUESDAY, OCTOBER 18, 2022 </div><div style="font-family: arial;"><br clear="none" /></div><div style="font-family: arial;">Assessing the Potential Transmissibility of Bovine and Cervid Prions with a Human Prion Protein-based Model ARS RESEARCH </div><div style="font-family: arial;"><br clear="none" /></div><div style="font-family: arial;"><a fg_scanned="1" href="https://nam11.safelinks.protection.outlook.com/?url=https%3A%2F%2Ftransmissiblespongiformencephalopathy.blogspot.com%2F2022%2F10%2Fassessing-potential-transmissibility-of.html&data=05%7C01%7CGStockburger%40abc27.com%7C7f4857b548ca4c57579308daccc305ce%7C9e5488e2e83844f6886cc7608242767e%7C0%7C1%7C638047435400177665%7CUnknown%7CTWFpbGZsb3d8eyJWIjoiMC4wLjAwMDAiLCJQIjoiV2luMzIiLCJBTiI6Ik1haWwiLCJXVCI6Mn0%3D%7C1000%7C%7C%7C&sdata=%2FkRtUDpQrbghIbnk7MSauru9GbZ6bEa8lJc1Iz2mejY%3D&reserved=0" rel="nofollow noopener noreferrer" shape="rect" style="color: blue;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/10/assessing-potential-transmissibility-of.html</a></div><div style="font-family: arial;"><br /></div></div><pre style="white-space: pre-wrap;"><div style="background-color: white; color: #1d2228; font-family: arial; font-size: 16px; text-align: left; white-space: normal;"><div>WEDNESDAY, NOVEMBER 30, 2022 </div><div><br /></div><div>USDA Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, Testing and Surveillance 2022 A Review of History </div><div><br /></div></div><div style="background-color: white; color: #1d2228; font-family: arial; font-size: 16px; text-align: left; white-space: normal;"><a fg_scanned="1" href="https://animalhealthreportpriontse.blogspot.com/2022/11/usda-bovine-spongiform-encephalopathy.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2022/11/usda-bovine-spongiform-encephalopathy.html</a></div><div><br /></div><div><br /></div></pre><pre style="white-space: pre-wrap;"><span style="background-color: transparent; font-family: Arial, Helvetica, sans-serif;">Diagnosis and Reporting of Creutzfeldt-Jakob Disease </span></pre><pre style="white-space: pre-wrap;"><span style="font-family: Arial, Helvetica, sans-serif;">Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor:
In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally..
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
</span></pre><pre style="white-space: pre-wrap;"><a fg_scanned="1" href="https://nam11.safelinks.protection.outlook.com/?url=http%3A%2F%2Fjama.jamanetwork.com%2Farticle.aspx%3Farticleid%3D1031186&data=05%7C01%7CGStockburger%40abc27.com%7C7f4857b548ca4c57579308daccc305ce%7C9e5488e2e83844f6886cc7608242767e%7C0%7C1%7C638047435400177665%7CUnknown%7CTWFpbGZsb3d8eyJWIjoiMC4wLjAwMDAiLCJQIjoiV2luMzIiLCJBTiI6Ik1haWwiLCJXVCI6Mn0%3D%7C1000%7C%7C%7C&sdata=BZfI3lpvnnqo7vKRTrg4Y2SKC0KmzFW3UjyG%2F8jUZIo%3D&reserved=0" rel="nofollow noopener noreferrer" shape="rect" style="color: #196ad4;" target="_blank">http://jama.jamanetwork.com/article.aspx?articleid=1031186</a></pre><pre style="white-space: pre-wrap;"></pre><div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Elsevier Editorial System(tm) for The Lancet Infectious Diseases</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Manuscript Draft</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Manuscript Number:</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Article Type: Personal View</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Corresponding Author: Mr. Terry S. Singeltary,</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Corresponding Author's Institution: na</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">First Author: Terry S Singeltary, none</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Order of Authors: Terry S Singeltary, none; Terry S. Singeltary</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory August 2007</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">August 2007</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007. With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE (one strain TSE in cows), and the nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human.</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Manuscript</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;">Terry S. Singeltary Sr. P.O. Box Bacliff, Texas USA 77518 flounder9@verizon.net</span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><br clear="none" /></span></div><div class="yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308blead yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308btext-muted yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bmb-3 yiv1113859732ydp46f79823yiv5180884413ydp6649cbbfyiv4488276153ydp490aa3cfyiv4058845648ydpf578f24byiv5901295807ydpe309fc02yiv7943631203ydpb4a00985yiv6154604746ydpec9c27f7yiv4374385699x_ydpec28ce7dyiv4426730788ydp474c308bjs-posted-text" style="line-height: 1.5;"><span style="color: #666666; font-family: Arial, Helvetica, sans-serif; font-size: 18px;"><a fg_scanned="1" href="https://nam11.safelinks.protection.outlook.com/?url=http%3A%2F%2Fweb.archive.org%2Fweb%2F20110507181935%2Fhttp%3A%2F%2Fwww.regulations.gov%2Ffdmspublic%2FContentViewer%3FobjectId%3D090000648027c28e%26disposition%3Dattachment%26contentType%3Dpdf&data=05%7C01%7CGStockburger%40abc27.com%7C7f4857b548ca4c57579308daccc305ce%7C9e5488e2e83844f6886cc7608242767e%7C0%7C1%7C638047435400177665%7CUnknown%7CTWFpbGZsb3d8eyJWIjoiMC4wLjAwMDAiLCJQIjoiV2luMzIiLCJBTiI6Ik1haWwiLCJXVCI6Mn0%3D%7C1000%7C%7C%7C&sdata=qOOFSOTB2u5FE6CitQPNKjiplm39aUXdw1e6K8Q2uvI%3D&reserved=0" rel="nofollow noopener noreferrer" shape="rect" style="color: #196ad4;" target="_blank">http://web.archive.org/web/20110507181935/http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf</a></span></div></div></div></div></div></pre></div><div><br /></div></div><div><br /></div></div></div></div></div></div><span style="background-color: white; color: #1d2228; font-family: arial; font-size: 16px;">Terry S. Singeltary Sr.</span>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-16172799747749583532022-11-14T14:49:00.007-06:002022-11-14T14:49:44.317-06:00Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease<p><span style="background-color: white; font-family: arial; font-size: 16px;">RESEARCH PAPER</span></p><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div><div>Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease</div><div><br /></div><div>Krista L. Harrison a,b,c, Sarah B. Garrettb, Joni Gilissenc,d, Michael J. Terranovae , Alissa Bernstein Sidemanb,c,f, Christine S. Ritchiea,c,g, and Michael D. Geschwindc,e a Division of Geriatrics, University of California, San Francisco, USA; b Philip R. University of California, San Francisco, USA; c Global Brain Health Institute, University of California, San Francisco, California, USA; d End-of-Life Care Research Group, Department of Family Medicine & Chronic Care, Vrije Universiteit Brussel (Vub), Belgium; e Memory and Aging Center, Department of Neurology, Weill Institute for Neurosciences, University of California San Francisco, San Francisco, CA, USA; f Department of Humanities and Social Sciences, University of California San Francisco, San Francisco, California, USA; g The Mongan Institute and the Division of Palliative Care and Geriatric Medicine, Massachusetts General Hospital, Boston, USA</div><div><br /></div><div>ARTICLE HISTORY</div><div><br /></div><div>Received 17 December 2021</div><div><br /></div><div>Accepted 10 February 2022</div><div><br /></div><div>ABSTRACT</div><div><br /></div><div>We aimed to identify targets for neuropalliative care interventions in sporadic Creutzfeldt-Jakob disease by examining characteristics of patients and sources of distress and support among former caregivers. We identified caregivers of decedents with sporadic Creutzfeldt-Jakob disease from the University of California San Francisco Rapidly Progressive Dementia research database. We purposively recruited 12 caregivers for in-depth interviews and extracted associated patient data. We analysed interviews using the constant comparison method and chart data using descriptive statistics. Patients had a median age of 70 (range: 60–86) years and disease duration of 14.5 months (range 4–41 months). Caregivers were interviewed a median of 22 (range 11–39) months after patient death and had a median age of 59 (range 45–73) years. Three major sources of distress included (1) the unique nature of sporadic Creutzfeldt-Jakob disease; (2) clinical care issues such as difficult diagnostic process, lack of expertise in sporadic Creutzfeldt-Jakob disease, gaps in clinical systems, and difficulties with end-of-life care; and (3) caregiving issues, including escalating responsibilities, intensifying stress, declining caregiver well-being, and care needs surpassing resources. Two sources of support were (1) clinical care, including guidance from providers about what to expect and supportive relationships; and (2) caregiving supports, including connection to persons with experience managing Creutzfeldt-Jakob disease, instrumental support, and social/emotional support. The challenges and supports described by caregivers align with neuropalliative approaches and can be used to develop interventions to address needs of persons with sporadic Creutzfeldt-Jakob disease and their caregivers.</div><div><br /></div><div>snip...</div><div><br /></div><div>Sources of support and amelioration</div><div><br /></div><div>We identified 2 major categories of support – clinical care and caregiving.</div><div><br /></div><div>Clinical care</div><div><br /></div><div>Despite challenges, many respondents also experienced clinicians and clinical care as sources of support (Table 5). Caregivers appreciated clinician guidance about diagnosis, prognosis, or referrals. Anticipatory guidance was particularly helpful: to understand the patient’s disease trajectory, ‘[the doctor] told me to focus on the rate of change of various phases, . . . [if] it’s accelerating or he’s moving to another function loss that is your sign that he’s moving forward. But if he stabilizes somehow . . . that may not be sign that’s [he’s] ready to go. That really helped me’ (c4). Guidance about when to seek additional help and the expected disease course, including how to recognize signs of imminent death, helped caregivers feel prepared, take breaks, and feel less guilt.</div><div><br /></div><div>Caregivers valued clinicians’ expertise in sCJD, particularly early in the disease journey, when detailed explanations of the disease were helpful. Respondents appreciated expert management of medications and advice about behavioural adaptations to manage sCJD symptoms. Confirming the CJD as sporadic (not genetic) was a source of relief for nearly all.</div><div><br /></div><div>Sensitive and supportive relationships with clinicians and researchers stood out, such as when clinicians responded quickly and thoroughly: ‘I could call her or text her anytime and she would be answering questions for me’ (c3). Clinicians and others with prion expertise helped caregivers prioritize self-care: ‘[Dr. III] himself . . .. advised me strongly to back off from [caregiving], that I would potentially cause harm to myself that could be damaging. So I really appreciated that advice’ (c4). All caregivers reported engaging hospice, sometimes before the diagnosis of CJD. Though some caregivers expressed frustrations with hospice clinicians or the care model (especially lack of continuity), most found hospice helpful for providing hands-on ADL support, breaks, and comfort. They benefited from fast implementation and hospice staff’s expertise in recognizing imminent death and appreciated when hospice made effort to learn about sCJD and inquire about respondents’ knowledge as sCJD caregivers.</div><div><br /></div><div>Caregiving</div><div><br /></div><div>Finally, respondents identified sources that facilitated being a caregiver (Table 6). They emphasized the benefit of reading or hearing stories from, or connecting with other sCJD caregivers, via YouTube, Facebook, and the CJD Foundation: ‘all you want to do is talk to people that have been through it. Because you don’t know what to expect’ (c7).</div><div><br /></div><div>Caregivers benefited from instrumental support (e.g. paid caregivers or facilities relieved caregiving burden): ‘I did all the heavy lifting at our house . . . When we transferred to the care facility, I felt lighter’ (c7). Friends or family also provided ADL, legal or financial help, such as documenting preferences or decision-makers while the patient was still able to make decisions. Many narratives indicated that socioeconomic resources were essential, such as being able to pay for caregiving, having access to state-funded care, and/or having jobs that permitted reduced schedules and lengthy leaves of absence.</div><div><br /></div><div>Social and emotional support was beneficial. Much of this came from friends and family, e.g., keeping the patient company or reminding (and helping) the caregiver to take a break: ‘There wasn’t anything to do except support her and everybody was ready, willing and able to sign up’ (c5). Some caregivers benefitted resilience-bolstering activities, such as religious practice or exercise. Others found comfort in maintaining close connection to the person with sCJD; one caregiver did ‘spa days’ for his wife after she was bedbound (c12).</div><div><br /></div><div>Caregiver recommendations or wishes</div><div><br /></div><div>Caregivers also identified items that they thought would have been helpful to them or future sCJD caregivers. Though hypothetical, these insights may be useful for intervention development.</div><div><br /></div><div>Regarding clinical care, some caregivers felt earlier accurate diagnosis and familiarity with sCJD among clinicians, hospice staff, and funeral home directors would have made the experience less difficult. Some recommended that clinics provide resources about expected symptoms and prognosis, what to take care of (e.g., advance care planning), resources (e.g., CJD Foundation, support groups, local hospice organizations), and contact information for expert advice about sCJD management to give facilities, hospices, and funeral homes.</div><div><br /></div><div>Regarding caregiving, some respondents thought they would have benefited from more self-care and time connecting with the patient. When asked for recommendations for future sCJD caregivers, respondents echoed these themes: spend more meaningful time with the patient, have more patience with themselves and the patient, connect with other sCJD caregivers, and engage hospice care.</div><div><br /></div><div>snip...</div><div><br /></div><div>Discussion</div><div><br /></div><div>This novel study provides an expanded understanding of challenges experienced by caregivers and persons with sCJD and identifies opportunities for improvement. Challenges primarily related to clinical care and caregiving and were exacerbated by the unique nature of sCJD. To our knowledge, this is the first in-depth description of palliative care needs of persons with sCJD. Ford et al. (2018) previously studied caregivers’ struggles to manage symptoms of patients with sCJD and found the most problematic to be mobility and coordination, mood and behaviour, personal care and continence, eating and swallowing, communication, and cognition and memory [20 – 23, 7]. Caregivers in our study voiced similar challenges with symptoms, and described broader sources of distress and challenges. Caregivers framed changes in patient function within the larger context of major losses and changes to relationships, life plans, and family roles. We additionally asked about supports to identify factors that ameliorated caregivers’ difficulties. Supports were often the inverse of challenges, such as sensitive versus insensitive disclosure of diagnosis and prognosis.</div><div><br /></div><div>Data on sources of distress and support in sCJD facilitates the development of neuropalliative tools and interventions. Table 7 demonstrates how palliative care approaches might be integrated into neurology practice for sCJD and slower-progressing dementia syndromes. For example, neurology trainees can be taught to use serious illness communication strategies [24] for sensitively disclosing a diagnosis of sCJD and asking if patients and caregivers want prognostic information or anticipatory guidance at this time [25,26]. Findings from this study can facilitate improving sCJD-specific care among hospice and palliative care clinicians. Neuropalliative-infused interventions for improving sCJD care will need to be refined with interdisciplinary multi-stakeholder input and tested for utility and effectiveness.</div><div><br /></div><div>Evidence regarding neuropalliative care needs in sCJD may be applicable to other rare and rapidly progressive diseases with no cure, as well as longer-course neurodegenerative diseases. A recent systematic review of factors influencing the provision of palliative care to persons with advanced dementia report similar problems: difficulty managing symptoms, lack of continuity of care, and lack of clinician skill in palliative care (such as sensitive disclosure of information or providing anticipatory guidance) [27]. A systematic review of integration of palliative care into dementia management highlights the importance of discussing disease trajectory and expectations and challenges from suboptimal symptom and medication management [28]. These challenges appeared in our study as well. We are adapting the analytic approach of this sCJD study to our parallel efforts to identify neuropalliative intervention targets for longer-course dementia syndromes [29–31].</div><div><br /></div><div>Limitations of the study include a relatively small sample at one institution, albeit one that recruits study participants nationally (and even globally) for this rare disease. Demographics of participating caregivers suggest that they are well-resourced. Caregivers with fewer resources may encounter more, or more severe, challenges than documented here. Future research should engage larger, more socioeconomically- and globallydiverse populations, and other RPDs that may raise different caregiving challenges. Nevertheless, these novel findings provide foundational data for further research and intervention development.</div><div><br /></div><div>In summary, this study drew on palliative care frameworks and mixed methods to yield a comprehensive description of challenges, supports, and opportunities to improve care for people with sCJD and their caregivers. Though sCJD is rare and rapidly progressing, the themes uncovered provide a framework for ongoing efforts to improve neuropalliative care for dementia care more broadly.</div><div><br /></div></div>snip...see full text;</div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><a fg_scanned="1" href="http://https//europepmc.org/backend/ptpmcrender.fcgi?accid=PMC8896185&blobtype=pdf" style="color: #196ad4;">https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC8896185&blobtype=pdf</a><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><br /></div><div data-setdir="false" dir="ltr" style="background-color: white; font-family: arial; font-size: 16px;"><div dir="ltr"><div dir="ltr" style="font-size: 13.3333px;"><div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">TUESDAY, APRIL 05, 2022<br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;">2022 American Academy of Neurology Emerging Sciences Abstract Website Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014</div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><a fg_scanned="1" href="https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html" rel="nofollow" style="color: blue;" target="_blank">https://creutzfeldt-jakob-disease.blogspot.com/2022/04/incidence-of-creutzfeldt-jakob-disease_5.html</a></div></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><br /></div><div dir="ltr" style="font-family: Helvetica, Arial, sans-serif;"><div dir="ltr" style="color: #1d2228; 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letter-spacing: inherit; text-align: justify;">SUNDAY, OCTOBER 16, 2022 </div><div style="color: black; font-size: 13.3333px; letter-spacing: inherit; text-align: justify;"><br /></div><div style="color: black; font-size: 13.3333px; letter-spacing: inherit; text-align: justify;">USDA Transmissible Spongiform Encephalopathy TSE Prion Action Plan National Program 103 Animal Health 2022-2027 </div><div style="color: black; font-size: 13.3333px; letter-spacing: inherit; text-align: justify;"><br /></div><div style="color: black; font-size: 13.3333px; letter-spacing: inherit; text-align: justify;"><a fg_scanned="1" href="https://transmissiblespongiformencephalopathy.blogspot.com/2022/10/usda-transmissible-spongiform.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://transmissiblespongiformencephalopathy.blogspot.com/2022/10/usda-transmissible-spongiform.html</a></div></div><br /></div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;"><div><div style="color: black; font-size: 13.3333px;">MONDAY, SEPTEMBER 19, 2022 </div><div style="color: black; font-size: 13.3333px;"><br /></div><div style="color: black; font-size: 13.3333px;">589.2001 BSE TSE regulations which prohibits the use of high-risk cattle material in feed for all animal species 2022<br /></div><div style="color: black; font-size: 13.3333px;"><br /></div><div style="color: black; font-size: 13.3333px;"><a fg_scanned="1" href="https://animalhealthreportpriontse.blogspot.com/2022/09/5892001-bse-tse-regulations-which.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://animalhealthreportpriontse.blogspot.com/2022/09/5892001-bse-tse-regulations-which.html</a><br /></div></div><div style="color: black; font-size: 13.3333px;"><br /></div><div dir="ltr" style="color: black; font-size: 13.3333px;"><pre style="white-space: pre-wrap;"><span style="font-family: Arial, Helvetica, sans-serif;">SUNDAY, OCTOBER 30, 2022 </span></pre><pre style="white-space: pre-wrap;"><span style="font-family: Arial, Helvetica, sans-serif;">Why is USDA "only" testing 25,000 samples a year?</span>
</pre><pre style="white-space: pre-wrap;"><span style="font-family: Arial, Helvetica, sans-serif;"><a fg_scanned="1" href="https://bovineprp.blogspot.com/2022/10/why-is-usda-only-testing-25000-samples.html" rel="nofollow noopener noreferrer" style="color: #338fe9; outline-style: solid; outline-width: 0px !important;" target="_blank">https://bovineprp.blogspot.com/2022/10/why-is-usda-only-testing-25000-samples.html</a></span></pre><pre style="white-space: pre-wrap;"><br /></pre></div></div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;">Monday, November 14, 2022 </div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;">Prion Diseases in Dromedary Camels (CPD) 2022 Review </div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;"><br /></div><div dir="ltr" style="color: #1d2228; font-family: arial; font-size: 16px;"><a fg_scanned="1" href="https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html" rel="nofollow noopener noreferrer" style="color: #196ad4;" target="_blank">https://camelusprp.blogspot.com/2022/11/prion-diseases-in-dromedary-camels-cpd.html</a></div></div><br /></div><br /></div><div dir="ltr">Terry S. Singeltary Sr.</div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.comtag:blogger.com,1999:blog-37789475.post-80929445472554826232022-10-06T16:11:00.003-05:002022-10-06T16:11:59.023-05:00Incidences Trends of Creutzfeldt-Jakob Disease in Israel<p> </p><p><br /></p><div style="background-color: white; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><span style="color: #26282a;">Incidences Trends of Creutzfeldt-Jakob Disease in Israel</span><br /></div><div class="yiv6104023021ydp85ef68c3yiv9636725932yqt4572921697" id="yiv6104023021ydp85ef68c3yiv9636725932yqt24167" style="background-color: white; font-family: arial; font-size: 13.3333px;"><div class="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yahoo_quoted" id="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yahoo_quoted_4796865753"><div id="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yiv6818234977"><div class="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yiv6818234977ydp7e066505yahoo-style-wrap"><div id="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yiv6818234977ydp7e066505yiv1244668415"><div class="yiv6104023021ydp85ef68c3yiv9636725932ydp3b952000yiv6818234977ydp7e066505yiv1244668415ydpa631f08fyahoo-style-wrap"><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><div><br clear="none" /></div><div>Yacov Balasha, Meir Walkerb, Esther Kahanac,d, Hadeel Nabale, Emilia Anise, Hanna Rosenmannf, Ron Miloc,d, and Amos D. Korczynb</div><div>aDepartment of Neurology, Kaplan Medical Center, Rehovot, Israel; bSackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel; cDepartment of Neurology, Barzilai University Medical Center, Ashkelon, Israel; dFaculty of Health Sciences, Ben Gurion University of the Negev, Beer-Sheva, Israel; eDivision of Epidemiology, Ministry of Health, Jerusalem, Israel; fDepartment of Neurology, the Agnes Ginges Center for Human Neurogenetics, Hadassah Hebrew University Medical Center, Jerusalem, Israel</div><div><br clear="none" /></div><div>Aims: Sporadic Creutzfeldt-Jakob disease (s-CJD) is a rare, fatal neurodegenerative disorder. Familial cases of Creutzfeldt-Jakob disease (f-CJD) due to mutations in the PRNP gene are even rarer around the world; however, in Israel there is an unusual focus of f-CJD patients carrying the E200K mutation. The number of E200K mutation carriers in Israel is increasing, which raised the suspicion of CJD transmission from person to person. If such transmission does occur, the incidence of s-CJD is expected to increase.</div><div><br clear="none" /></div><div>Materials and Methods: Using data from the national CJD registry and official statistics on the Israeli popula- tion, we studied incidence rates of f-CJD and s-CJD for the period from 1985 to 2018 applying the SEER (Surveillance Epidemiology and End Results) statistical packet elaborated in the US National Cancer Institute Results: In total, we identified 621 CJD patients (405 f-CJD and 216 s-CJD) cases. In the cohort of f-CJD patients the mean age-adjusted annual incidence rate over the above-mentioned period was 1.88 ± 0.09 (95% CI: 1.7–2.08) per 1,000,000. In the cohort of s-CJD patents the mean age-adjusted incidence rate over the same period was 0.93 ± 0.06 (95% CI: 0.81– 1.06) per 1,000,000 people. No significant time trends were found according to the permutation test of join- point regression in either of them.</div><div><br clear="none" /></div><div>When both cohorts were combined, the mean annual age-adjusted incidence of CJD in Israel was 2.81 ± 0.11 (95% CI: 2.58–3.04) per 1,000,000. From 1985 to 2018, there was a borderline increase in inci- dence of 0.8% per year, (95% CI: 0–1.6, p = 0.37), which is non significant.</div><div><br clear="none" /></div><div>Conclusions: Israel has a great predominance of f-CJD compared to s-CJD. The mean incidence of s-CJD in Israel is similar to most countries. Between 1985 and 2018, the annual age adjusted incidence rates for both forms of CJD have remained stable. There is no evidence for transmission of the disease.</div></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><a href="https://www.tandfonline.com/doi/pdf/10.1080/19336896.2022.2091286?needAccess=true" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">https://www.tandfonline.com/doi/pdf/10.1080/19336896.2022.2091286?needAccess=true</a><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><div>Biobank of genetic CJD in Israel</div><div><br clear="none" /></div><div>Alice Anane, and Victor Novack</div><div><br clear="none" /></div><div>CJD Foundation Israel, and Soroka University Medical Center, Israel</div><div><br /></div><div>Aims: A sharing longitudinal biobank of samples and values of genetic families with E200K mutation and patients.</div><div><br clear="none" /></div><div>Material and Methods: The world prevalence of the disease is 1:500,000–1,000,000 a year. About 85% are sporadic cases, and about 15% are genetic. In Israel, there is the highest prevalence in the world of the genetic form (gCJD), estimated by about 1:200,000 a year, due to a common mutation that induces the risk, at the Libyan-Tunisian community at Israel, which is characterized by many members at each family. The estimation is thousands of families with the genetic risk, concentrate in rather small area of the state of Israel.</div><div><br clear="none" /></div><div>My name is Alice Anane, a daughter of a father, who died from this disease at a young age (49). After discovering I and my siblings are carriers, I founded the association in 2008 and I am very active worldwide to advance research for this hor- rible and devastating disease. We unite a community of the genetic families. Our registry counts more than 400 members (representatives of families) and it grows constantly.</div><div><br clear="none" /></div><div>Our partner – Prof. Victor Novack, Clinical Epidemiologist. MD, Ph.D. Head of The Research Authority, Supervising Negev Bio Bank (NBB), a high- quality biorepository of biological samples that operates with MIDGAM, a national Israeli infrastructure for biomedical research and includes five Israeli medical centers, which located in different areas and works as biobanking sites. NBB will be in charge of managing and maintaining the samples of CJD from all the sites in its facilities. We invite researchers to apply and initiate new research on our human samples, sharing values of results with us, for conducting AI of Big Data research.</div><div><br clear="none" /></div><div>Results: Longitude studies for biomarkers, early diag- nosis and therapeuties for gCJD.</div><div><br clear="none" /></div><div>Conclusions: Conducting and sharing data on the same specific samples for longitude studies can bring a revelation for science and for us at the shortest time in the most efficient way. </div><div><br clear="none" /></div></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><a href="https://m.youtube.com/watch?v=8UQTpoBYvdU%26t%3D10s" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">https://m.youtube.com/watch?v=8UQTpoBYvdU%26t%3D10s</a><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><a href="https://www.tandfonline.com/doi/pdf/10.1080/19336896.2022.2091286?needAccess=true" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">https://www.tandfonline.com/doi/pdf/10.1080/19336896.2022.2091286?needAccess=true</a><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><div style="color: black;"><span style="background-color: #fff3db; color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif; font-size: small;">Cathala et al. (9) reported that at least three ancestors of a family with 14 cases of CJD were shepherds. Most of the family had lived in the same farming region for generations. Mayer et al. (14) noted that sheep raising is traditional in the region where a Slovakian CJD cluster occurred. One patient, a 57-year-old female raised sheep for 12 years prior to onset of CJD and admitting eating raw sheep brain (Orolin D. et al., personal communication, 1978). Lo Russo et al. (15) reported that the places of origin of seven out of eight patients with CJD coincided with the distribution of sheep raised in Central and Southern Italy. Libya has one of the highest rates of consumption of sheep and mutton per capita in the world (16), and Libyan immigrants to Israel were found to have more than 30 times the rate of CJD as immigrants to Israel from other countries (17). Thus, some available data suggest a link between CJD and sheep exposure. However, more direct data relating sheep contact and consumption in CJD patients and controls are needed.</span><br style="color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif;" /><br style="color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif;" /><span style="background-color: #fff3db; color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif; font-size: small;">Brown stated that the agent of scrapie has not been detected in sheep muscle. Since mainly muscle is consumed when sheep are eaten, he did not think that sheep meat was a reasonable source of the scrapie agent. However, Pattison and Millson (18) did detect the scrapie virus in the muscle of experimentally infected goats. Very few transmission and virus recovery experiments were done with sheep muscle compared with the number done with other tissues. It should be realized that sheep meat prepared for consumption also contains blood vessels, nervous and lymphatic tissues. Lamb and mutton chops may even contain spinal cord. It has been well established that lymphatic tissues of affected sheep contain scrapie virus from an early age. Even after invading the central nervous system, scrapie agent continues to exist in other tissues (19).</span><br style="color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif;" /></div><div style="color: black;"><span style="background-color: #fff3db; color: #29303b; font-family: Georgia, Times, "Times New Roman", sans-serif; font-size: small;"><br /></span></div><div style="color: black;"><a href="http://scrapie-usa.blogspot.com/2011/02/epidemiologic-critique-of-creutzfeldt.html" rel="nofollow noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">http://scrapie-usa.blogspot.com/2011/02/epidemiologic-critique-of-creutzfeldt.html</a></div><div style="color: black;"><div style="font-family: arial; font-size: 13.3333px;"><div><br /></div><div>Sunday, October 27, 2013 </div><div><br /></div><div>A Kiss of a Prion: New Implications for Oral Transmissibility </div><div><br /></div><div><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2013/10/a-kiss-of-prion-new-implications-for.html" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2013/10/a-kiss-of-prion-new-implications-for.html</a><br /></div><div><br /></div><div><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2013/10/a-kiss-of-prion-new-implications-for.html" rel="nofollow noopener noreferrer" style="color: #196ad4; cursor: pointer;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2013/10/a-kiss-of-prion-new-implications-for.html</a><br /></div><div><br /></div><div><div>***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent.</div><div><br /></div><div>snip...see full text here;</div><div><br /></div><div><a href="http://web.archive.org/web/20050425210551/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20050425210551/http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf</a></div></div></div><div style="font-family: arial; font-size: 10pt;"><br /></div><div style="font-family: arial; font-size: 10pt;">snip...see full text;</div><div style="font-family: arial; font-size: 10pt;"><br /></div><div style="font-family: arial; font-size: 13.3333px;"><a href="https://familialcjdtseprion.blogspot.com/" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">https://familialcjdtseprion.blogspot.com/</a><br /></div><div style="font-family: arial; font-size: 13.3333px;"><br /></div><div style="font-family: arial; font-size: 13.3333px;"><a href="https://betaamyloidcjd.blogspot.com/" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">https://betaamyloidcjd.blogspot.com/</a></div><div style="font-family: arial; font-size: 13.3333px;"><br /></div><div style="font-family: arial; font-size: 13.3333px;"><div style="font-size: 10pt;">vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what if ???</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Greetings Friends, Neighbors, and Colleagues,</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a href="https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a href="http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html</a></div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Saturday, February 2, 2019 </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">CWD GSS TSE PRION SPINAL CORD, Confucius Ponders, What if?</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">snip... </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"> ***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <*** </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">REVIEW </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Thursday, March 8, 2018 </div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;">Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein</div><div style="font-size: 10pt;"><br /></div><div style="font-size: 10pt;"><a href="https://familialcjdtseprion.blogspot.com/2018/03/familial-human-prion-diseases.html" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">https://familialcjdtseprion.blogspot.com/2018/03/familial-human-prion-diseases.html</a></div><div><div><br /></div><div>MONDAY, NOVEMBER 26, 2018 </div><div><br /></div><div>Sporadic Creutzfeldt-Jakob Disease in a Woman Married Into a Gerstmann-Sträussler-Scheinker Family: An Investigation of Prions Transmission via Microchimerism </div><div><br /></div><div><a href="http://creutzfeldt-jakob-disease.blogspot.com/2018/11/sporadic-creutzfeldt-jakob-disease-in.html" rel="noopener noreferrer" style="color: blue; cursor: pointer;" target="_blank">http://creutzfeldt-jakob-disease.blogspot.com/2018/11/sporadic-creutzfeldt-jakob-disease-in.html</a></div></div></div></div></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;">“The number of E200K mutation carriers in Israel is increasing, which raised the suspicion of CJD transmission from person to person. If such transmission does occur, the incidence of s-CJD is expected to increase.”</div><div style="color: #26282a; font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><br clear="none" /></div><div><h1 class="yiv6104023021ydpc8c3c7baarticle-metadata-title" id="yiv6104023021ydpc8c3c7baarticle--current--title" itemprop="name" style="border: 0px; font-family: -webkit-standard; font-size: 26px; line-height: 36px; margin: 0px; padding: 0px; vertical-align: baseline;">Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD).</h1><div class="yiv6104023021ydpc8c3c7bametadata--authors" style="border: 0px; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><div class="yiv6104023021ydpc8c3c7bametadata--author-list" style="border: 0px; font-family: -webkit-standard; font-size: 14px; line-height: 24px; margin: 20px 0px 0px; padding: 0px; vertical-align: baseline;"><span class="yiv6104023021ydpc8c3c7bametadata--author" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><div class="yiv6104023021ydpc8c3c7bametadata--author-block" style="border: 0px; display: inline-block; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><a class="yiv6104023021ydpc8c3c7bametadata--author-link" href="https://europepmc.org/search?query=AUTH%3A%22R%20Gabizon%22" id="yiv6104023021ydpc8c3c7baarticle--author--name-R-Gabizon" rel="nofollow noopener noreferrer" style="border: 0px; color: #615389; cursor: pointer; margin: 0px; overflow-wrap: break-word; padding: 0px; vertical-align: baseline;" target="_blank"><span class="yiv6104023021ydpc8c3c7bametadata--author-name" itemprop="name" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">Gabizon R</span><span class="yiv6104023021ydpc8c3c7basemi-bold" style="border: 0px; font-size: 0.8em; font-weight: 600; line-height: 1; margin: 0px; padding: 0px; vertical-align: 45%;">1</span></a><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">, </span></div></span></span> <span class="yiv6104023021ydpc8c3c7bametadata--author" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><div class="yiv6104023021ydpc8c3c7bametadata--author-block" style="border: 0px; display: inline-block; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><a class="yiv6104023021ydpc8c3c7bametadata--author-link" href="https://europepmc.org/search?query=AUTH%3A%22H%20Rosenmann%22" id="yiv6104023021ydpc8c3c7baarticle--author--name-H-Rosenmann" rel="nofollow noopener noreferrer" style="border: 0px; color: #615389; cursor: pointer; margin: 0px; overflow-wrap: break-word; padding: 0px; vertical-align: baseline;" target="_blank">Rosenmann H</a><span style="border: 0px; 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vertical-align: baseline;">, </span></div></span></span> <span class="yiv6104023021ydpc8c3c7bametadata--author" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><div class="yiv6104023021ydpc8c3c7bametadata--author-block" style="border: 0px; display: inline-block; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><a class="yiv6104023021ydpc8c3c7bametadata--author-link" href="https://europepmc.org/search?query=AUTH%3A%22I%20Kahana%22" id="yiv6104023021ydpc8c3c7baarticle--author--name-I-Kahana" rel="nofollow noopener noreferrer" style="border: 0px; color: #615389; cursor: pointer; margin: 0px; overflow-wrap: break-word; padding: 0px; vertical-align: baseline;" target="_blank">Kahana I</a><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">, </span></div></span></span> <span class="yiv6104023021ydpc8c3c7bametadata--author" style="border: 0px; 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vertical-align: baseline;"><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><div class="yiv6104023021ydpc8c3c7bametadata--author-block" style="border: 0px; display: inline-block; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><a class="yiv6104023021ydpc8c3c7bametadata--author-link" href="https://europepmc.org/search?query=AUTH%3A%22Y%20Shugart%22" id="yiv6104023021ydpc8c3c7baarticle--author--name-Y-Shugart" rel="nofollow noopener noreferrer" style="border: 0px; color: #615389; cursor: pointer; margin: 0px; overflow-wrap: break-word; padding: 0px; vertical-align: baseline;" target="_blank">Shugart Y</a><span style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">, </span></div></span></span> <span class="yiv6104023021ydpc8c3c7bametadata--author" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;"><span style="border: 0px; 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display: inline-block; line-height: 21px; margin: 0px; padding: 0px; vertical-align: baseline;"><a class="yiv6104023021ydpc8c3c7bametadata--author-link" href="https://europepmc.org/search?query=AUTH%3A%22S%20B%20Prusiner%22" id="yiv6104023021ydpc8c3c7baarticle--author--name-S-B-Prusiner" rel="nofollow noopener noreferrer" style="border: 0px; color: #615389; cursor: pointer; margin: 0px; overflow-wrap: break-word; padding: 0px; vertical-align: baseline;" target="_blank">Prusiner SB</a></div></span></span></div><h2 class="yiv6104023021ydpc8c3c7bametadata--dropdown-title" style="border: 0px; font-family: -webkit-standard; font-size: 22px; line-height: 32px; margin: 12px 0px 4px; padding: 0px; vertical-align: baseline;"><span class="yiv6104023021ydpc8c3c7baaction yiv6104023021ydpc8c3c7basmall yiv6104023021ydpc8c3c7basemi-bold" id="yiv6104023021ydpc8c3c7baarticle--expand--authInformation" style="border: 0px; color: #20699c; cursor: pointer; font-size: 16px; line-height: 20px; margin: 0px; outline: none; padding: 0px; text-decoration: inherit; vertical-align: baseline;">Author information<span class="yiv6104023021ydpc8c3c7baaction-icon yiv6104023021ydpc8c3c7baicon-right" style="border: 0px; color: #5797c5; display: inline; margin: 0px; padding: 0px 0px 0px 8px; text-decoration: inherit; vertical-align: baseline; white-space: nowrap;"><span class="yiv6104023021ydpc8c3c7bafas yiv6104023021ydpc8c3c7bafa-caret-right" style="border: 0px; display: inline-block; line-height: 1; margin: 0px; padding: 0px; vertical-align: baseline;"></span></span></span></h2><p class="yiv6104023021ydpc8c3c7bametadata--citation" style="border: 0px; font-family: -webkit-standard; font-size: 14px; line-height: 24px; margin: 12px 0px; max-width: 100%; padding: 0px; vertical-align: baseline;"><span class="yiv6104023021ydpc8c3c7bametadata--source-title" id="yiv6104023021ydpc8c3c7baarticle--publisher--name" itemprop="isPartOf" itemtype="http://schema.org/Periodical" style="border: 0px; font-weight: 600; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">American Journal of Human Genetics</span>, <span itemprop="datepublished" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">01 Oct 1993</span>, 53(4):<span itemprop="pagination" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">828-835</span><br /><span class="yiv6104023021ydpc8c3c7bametadata--pmid" style="border: 0px; margin: 0px 12px 0px 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">PMID: 8105682 </span><span class="yiv6104023021ydpc8c3c7bametadata--pmcid" id="yiv6104023021ydpc8c3c7baarticle--pmcid" style="border: 0px; margin: 0px; padding: 0px; text-decoration: inherit; vertical-align: baseline;">PMCID: PMC1682379</span></p><p class="yiv6104023021ydpc8c3c7bametadata--citation" style="border: 0px; font-family: -webkit-standard; font-size: 14px; line-height: 24px; margin: 12px 0px; max-width: 100%; padding: 0px; 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box-sizing: inherit; color: #212121; cursor: pointer; font-size: 1.6rem; text-decoration-line: none; transition: color 0.3s ease 0s;"></a></li></ul></div></div></div><span class="period" style="box-sizing: inherit; color: #0071bc;">. </span><span class="cit" style="box-sizing: inherit; display: inline-block;">1991;10(5-6):228-31.</span></div> <span class="citation-doi" style="box-sizing: inherit; color: #5b616b; display: inline-block; line-height: 1.5;">doi: 10.1159/000110276.</span></div><h1 class="heading-title" style="box-sizing: inherit; clear: both; font-family: Merriweather, Georgia, Cambria, "Times New Roman", Times, serif; font-size: 2.6rem; line-height: 1.4; margin: 0px 0px 1.6rem; overflow-wrap: break-word;">Genetic and environmental factors determining the development of Creutzfeldt-Jakob disease in Libyan Jews</h1><div class="inline-authors" style="box-sizing: inherit; color: #5b616b; line-height: 1.5; margin-bottom: 1.2rem;"><div class="authors" style="box-sizing: inherit;"><div class="authors-list" style="box-sizing: inherit; display: inline; line-height: 1.5;"><span class="authors-list-item " style="box-sizing: inherit; display: inline-block;"><a class="full-name" data-ga-action="author_link" data-ga-category="search" data-ga-label="J Chapman" href="https://pubmed.ncbi.nlm.nih.gov/?term=Chapman+J&cauthor_id=1798423" ref="linksrc=author_name_link" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline; text-decoration-line: none;">J Chapman</a><span class="affiliation-links" style="box-sizing: inherit; font-size: 12px; line-height: 0; position: relative; top: -0.5em; vertical-align: baseline;"><span class="author-sup-separator" style="box-sizing: inherit;"> </span><a class="affiliation-link" href="https://pubmed.ncbi.nlm.nih.gov/1798423/#affiliation-1" ref="linksrc=author_aff" style="background-color: #f1f1f1; border-radius: 2px; box-sizing: inherit; color: #323a45; cursor: pointer; display: inline-block; font-size: inherit; line-height: 1; margin-right: 0px; padding: 0.1rem 0.3rem; text-decoration-line: none; transition: color 0.3s ease 0s;" title="Sackler School of Medicine, Tel-Aviv University, Ramat Aviv, Israel.">1</a></span><span class="comma" style="box-sizing: inherit;">, </span></span><span class="authors-list-item " style="box-sizing: inherit; display: inline-block;"><a class="full-name" data-ga-action="author_link" data-ga-category="search" data-ga-label="A D Korczyn" href="https://pubmed.ncbi.nlm.nih.gov/?term=Korczyn+AD&cauthor_id=1798423" ref="linksrc=author_name_link" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline; text-decoration-line: none;">A D Korczyn</a></span></div></div></div><div class="short-article-details" style="box-sizing: inherit; margin-bottom: 0.3rem;">Affiliations <button aria-controls="expanded-authors" aria-expanded="false" class="more-details" data-alt-text="Collapse" data-fetch-url="/1798423/long-authors/" data-id-prefix="" data-pinger-ignore="" id="toggle-authors" style="-webkit-font-smoothing: antialiased; appearance: none; background-color: white; border-color: initial; border-radius: 3px; border-style: initial; border-width: 0px; box-sizing: inherit; color: #323a45; cursor: pointer; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 1.6rem; font-stretch: inherit; font-style: inherit; font-variant: inherit; font-weight: normal; height: 2.4rem; line-height: 2.4rem; margin: auto 0px 0px; outline-offset: 0px; overflow: visible; padding: 0px 1rem 0px 2.5rem; position: relative; text-transform: lowercase; transition: color 0.3s ease 0s, background-color 0.3s ease 0s; width: auto;"><span class="title" style="box-sizing: inherit;">expand</span></button></div><ul class="identifiers" id="full-view-identifiers" style="box-sizing: inherit; line-height: 1.5; list-style: none; margin: 0px; padding: 0px;"><li style="box-sizing: inherit; display: inline-block; line-height: 1.5; margin: 0px 1rem 0px 0px; padding: 0px;"><span class="identifier pubmed" style="box-sizing: inherit;"><span class="id-label" style="box-sizing: inherit;">PMID: </span><span class="current-id" style="box-sizing: inherit;" title="PubMed ID">1798423</span></span></li> <li style="box-sizing: inherit; display: inline-block; line-height: 1.5; margin: 0px 1rem 0px 0px; padding: 0px;"><span class="identifier doi" style="box-sizing: inherit;"><span class="id-label" style="box-sizing: inherit;">DOI: </span><a class="id-link" data-ga-action="DOI" data-ga-category="full_text" href="https://doi.org/10.1159/000110276" ref="linksrc=article_id_link&article_id=10.1159/000110276&id_type=DOI" rel="noopener" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; font-size: inherit; text-decoration-line: none;" target="_blank">10.1159/000110276</a></span></li></ul></div></header><div class="abstract" id="abstract" style="box-sizing: inherit; margin: 3.6rem 0px 0px; position: relative; word-break: break-word;"><h2 class="title" style="box-sizing: inherit; clear: both; color: #212121; font-family: Merriweather, Georgia, Cambria, "Times New Roman", Times, serif; font-size: 2rem; line-height: 2.4rem; margin-bottom: 1.2rem; margin-top: 3.6rem;">Abstract</h2><div class="abstract-content selected" id="enc-abstract" style="box-sizing: inherit; clear: left;"><p style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;">The cluster of Creutzfeldt-Jakob disease (CJD) among Jews of Libyan origin is one of the largest in the world. A number of hypotheses have been proposed to account for this cluster, the most prevalent but unsubstantiated hypothesis being that a transmissible agent was ingested in the form of scrapie-infected sheep brains. It has, however, been shown that a modified host protein encoded by the gene specifying the scrapie amyloid precursor is critically involved in the pathogenesis of transmissible spongiform encephalopathies such as CJD, Gerstmann-Strüssler-Scheinker syndrome and Kuru. A mutation at codon 200 in the open reading frame of this gene has recently been linked to a cluster of CJD patients in Slovakia. We examined the prevalence of this mutation among CJD patients of Libyan descent in Israel. All patients were found to have the same codon 200 mutation. These findings implicate this mutation in the high prevalence of CJD among Libyan Jews and Sephardic Jews from other Mediterranean countries.</p><p style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><a href="https://pubmed.ncbi.nlm.nih.gov/1798423/" style="color: #0096ef; cursor: pointer; text-decoration-line: none;">https://pubmed.ncbi.nlm.nih.gov/1798423/</a></p><p style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><a href="https://www.jstor.org/stable/3520745" style="color: #0096ef; cursor: pointer; text-decoration-line: none;">https://www.jstor.org/stable/3520745</a><br /></p><p style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; font-family: Arial, Helvetica, sans-serif;">UNCONVENTIONAL VIRUSES AND THE ORIGIN </span><span style="background-color: transparent; font-family: Arial, Helvetica, sans-serif;">AND DISAPPEARANCE OF KURU</span></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Nobel Lecture, December 13, 1976</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">by D. CARLETON GAJDUSEK</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">National Institutes of Health, Bethesda, Maryland, U.S.A. </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">snip... </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">In a study in Israel, an overall prevalence in Jews of Libyan origin is 30 times as high as in Jews of European origin (40). The custom of eating the eyeballs and brains of sheep in the Jewish households of North African and Middle Eastern origin, as opposed to Jewish households of European origin, has understandably given rise to the conjecture that scrapie-infected sheep tissue might be the source of such CJD infection (37). </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP... </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">The passage of sheep scrapie into other sheep and into goats, at least by the route of feeding of material contaminated with placenta and embryonic membrane (53), and into mink from feeding carcasses of scrapied sheep, are established paths of scrapie transmission. In view of the experimental transmission of scrapie to monkeys, there is serious cause for wonder whether kitchen and butchery accidents involving the contamination of skin and eyes may not be a possible source of CJD in man (36a, 37). </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP... </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Scrapie has been transmitted in our laboratory to five species of monkeys (Tables 9 and 10) (23, 31, 32), and such transmission has occurred using infected brain from naturally infected sheep and from experimentally infected goats and mice (Figures 22a, b, c). The disease produced is clinically and pathologically indistinguishable from experimental CJD in these species. .........</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Figure 22. Scrapie has been transmitted to three species of New World monkeys and two species of Old World monkeys (Tables 9, 10). 22a. Transmission of scrapie from the brain of a scrapie-infected Suffolk ewe (C506) in Illinois to a cynomolgus monkey, and from the 4th mouse passage of this strain of scrapie virus to two squirrel monkeys. Incubation period in the cynomolgus was 73 months and in the squirrel monkeys 31 and 33 months. A chimpanzee and a rhesus monkey inoculated 109 months ago with this sheep brain remain well, as does a spider monkey inoculated 70 months ago with brain from the 4th passage of the C506 strain of scrapie in mice.</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP...</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">22b. Primary transmission of goat-adapted scrapie (Compton, England strain) to the squirrel monkey and to mice and the transmission of mouse-adapted scrapie to two species of Old World and three species of New World monkeys. Numbers in parentheses are the number of months elapsed since inoculation, during which the animal remained asymptomatic.</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP... </span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">22c. Transmission of mouse-adapted sheep scrapie (U. S. strain 434-3-897) to a squirrel monkey 38 months following intracerebral inoculation with a suspension of scrapie-infected mouse brain containing 10a7.3 infectious units of virus per ml. This animal showed signs of ataxia, tremors and incoordination, and the disease was confirmed histologically. See (b) for an explanation of symbols.</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP...</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Figure 23. Transmissible mink encephalopathy (TME), a rare disease of American ranch mink, is possibly a form of scrapie. The clinical picture and histopathological lesions attendant in the brain, resemble that of scrapie, and scrapie sheep carcasses were fed to mink on ranches on which TME appeared. The disease is transmissible to sheep, goats, certain rodents and New and Old World monkeys. Illustrative data on the primary transmissions of transmissible mink encephalopathy to one species of New World monkey and two species of Old World monkeys, and serial passage of the virus in squirrel, rhesus and stumptailed monkeys are presented in this Figure. Incubation periods are shown in months that elapsed between inoculation and onset of clinical disease. (Figure includes information from our laboratory and from R. F. Marsh, R. J. Eckroade, and R. P. Hanson.)</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SNIP... end</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">SOURCE;</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">UNCONVENTIONAL VIRUSES AND THE ORIGIN AND DISAPPEARANCE OF KURU</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Nobel Lecture, December 13, 1976</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">by D. CARLETON GAJDUSEK</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">National Institutes of Health, Bethesda, Maryland, U.S.A.</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Nature. 1972 Mar 10;236(5341):73-4.</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).</span><br /></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">Gibbs CJ Jr, Gajdusek DC. </span><span style="background-color: transparent; color: #212121; font-family: Arial, Helvetica, sans-serif; font-size: 16px;">https://www.jstor.org/stable/1744284</span></p><p style="box-sizing: inherit; line-height: 1.5; margin: 1.2rem 0px; padding: 0px;"><span style="background-color: transparent; color: #212121; font-family: Merriweather, Georgia, Cambria, "Times New Roman", Times, serif; font-size: 2rem;">Similar articles</span><br /></p></div></div><div class="similar-articles" id="similar" style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; margin: 3.6rem 0px 0px; padding: 0px 0px 0.4rem; position: relative; word-break: break-word;"><ul class="articles-list" id="similar-articles-list" style="box-sizing: inherit; line-height: 1.5; list-style: none; margin: 1.2rem 0px; padding: 0px;"><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px 0px 1.2rem; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; display: inline;"><a class="docsum-title" data-ga-action="1761106" data-ga-category="similar_article" data-ga-label="" href="https://pubmed.ncbi.nlm.nih.gov/1761106/" ref="article_id=1761106&linksrc=similar_articles_link&ordinalpos=1" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.8rem; line-height: 2.4rem; margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">Creutzfeldt-Jakob disease among Libyan Jews.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Korczyn AD.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">Eur J Epidemiol. 1991 Sep;7(5):490-3. doi: 10.1007/BF00143127.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">1761106</span></span> <span class="publication-type spaced-citation-item citation-part" style="box-sizing: inherit; display: inline-block; padding-left: 2.4rem;">Review.</span></div></div></li><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px 0px 1.2rem; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; display: inline;"><a class="docsum-title" data-ga-action="1685643" data-ga-category="similar_article" data-ga-label="" href="https://pubmed.ncbi.nlm.nih.gov/1685643/" ref="article_id=1685643&linksrc=similar_articles_link&ordinalpos=2" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.8rem; line-height: 2.4rem; margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">A mutation in the prion protein gene in Creutzfeldt-Jakob disease in Jewish patients of Libyan, Greek, and Tunisian origin.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Korczyn AD, Chapman J, Goldfarb LG, Brown P, Gajdusek DC.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">Ann N Y Acad Sci. 1991;640:171-6. doi: 10.1111/j.1749-6632.1991.tb00211.x.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">1685643</span></span></div></div></li><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px 0px 1.2rem; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; 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margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan Jews.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Meiner Z, Gabizon R, Prusiner SB.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">Medicine (Baltimore). 1997 Jul;76(4):227-37. doi: 10.1097/00005792-199707000-00001.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">9279329</span></span> <span class="publication-type spaced-citation-item citation-part" style="box-sizing: inherit; display: inline-block; padding-left: 2.4rem;">Review.</span></div></div></li></ul><div class="actions-bar" style="box-sizing: inherit; margin-top: 1.8rem;"><a class="usa-button show-all-linked-articles" data-ga-action="show_all" data-ga-category="similar_article" data-href="/?linkname=pubmed_pubmed&from_uid=1798423" style="-webkit-font-smoothing: initial; appearance: none; background-color: transparent; border-radius: 0px; border: 0px; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.6rem; line-height: 1; margin: 0px; padding: 0px; text-align: center; width: auto;">See all similar articles</a></div></div><div class="citedby-articles" id="citedby" style="box-sizing: inherit; margin: 3.6rem 0px 0px; position: relative; word-break: break-word;"><h2 class="title" style="box-sizing: inherit; clear: both; color: #212121; font-family: Merriweather, Georgia, Cambria, "Times New Roman", Times, serif; font-size: 2rem; line-height: 2.4rem; margin-bottom: 1.2rem; margin-top: 3.6rem;">Cited by</h2><ul class="articles-list" id="citedby-articles-list" style="box-sizing: inherit; color: #212121; font-family: BlinkMacSystemFont, -apple-system, "Segoe UI", Roboto, Oxygen, Ubuntu, Cantarell, "Fira Sans", "Droid Sans", "Helvetica Neue", sans-serif; font-size: 16px; line-height: 1.5; list-style: none; margin: 1.2rem 0px; padding: 0px;"><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px 0px 1.2rem; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; display: inline;"><a class="docsum-title" data-ga-action="20827556" data-ga-category="cited_by" data-ga-label="" href="https://pubmed.ncbi.nlm.nih.gov/20827556/" ref="article_id=20827556&linksrc=citedby_articles_link&ordinalpos=1" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.8rem; line-height: 2.4rem; margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">Tau and 14-3-3 of genetic and sporadic Creutzfeldt-Jakob disease patients in Israel.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Meiner Z, Kahana E, Baitcher F, Korczyn AD, Chapman J, Cohen OS, Milo R, Aharon-Perez J, Abramsky O, Gabizon R, Rosenmann H.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">J Neurol. 2011 Feb;258(2):255-62. doi: 10.1007/s00415-010-5738-6. Epub 2010 Sep 9.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">20827556</span></span></div></div></li><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px 0px 1.2rem; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; display: inline;"><a class="docsum-title" data-ga-action="8105028" data-ga-category="cited_by" data-ga-label="" href="https://pubmed.ncbi.nlm.nih.gov/8105028/" ref="article_id=8105028&linksrc=citedby_articles_link&ordinalpos=2" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.8rem; line-height: 2.4rem; margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon 200 mutation.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Chapman J, Brown P, Goldfarb LG, Arlazoroff A, Gajdusek DC, Korczyn AD.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">J Neurol Neurosurg Psychiatry. 1993 Oct;56(10):1109-12. doi: 10.1136/jnnp.56.10.1109.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">8105028</span></span> <span class="free-resources spaced-citation-item citation-part" style="box-sizing: inherit; color: #c05600; display: inline-block; font-weight: 600; padding-left: 2.4rem;">Free PMC article.</span></div></div></li><li class="full-docsum" style="box-sizing: inherit; line-height: 2.4rem; margin: 0px; padding: 0px; position: relative;"><div class="docsum-content" style="box-sizing: inherit; display: inline;"><a class="docsum-title" data-ga-action="1761106" data-ga-category="cited_by" data-ga-label="" href="https://pubmed.ncbi.nlm.nih.gov/1761106/" ref="article_id=1761106&linksrc=citedby_articles_link&ordinalpos=3" style="background-color: transparent; box-sizing: inherit; color: #0071bc; cursor: pointer; display: inline-block; font-size: 1.8rem; line-height: 2.4rem; margin: 0px; outline-offset: 0px; overflow-wrap: break-word; text-decoration-line: none;">Creutzfeldt-Jakob disease among Libyan Jews.</a><div class="docsum-citation full-citation" style="box-sizing: inherit; color: #4d8055; font-size: 1.4rem; line-height: 2.4rem;"><span class="docsum-authors full-authors" style="box-sizing: inherit; color: #212121; display: block;">Korczyn AD.</span><span class="docsum-journal-citation full-journal-citation" style="box-sizing: inherit; display: block;">Eur J Epidemiol. 1991 Sep;7(5):490-3. doi: 10.1007/BF00143127.</span><span class="citation-part" style="box-sizing: inherit; display: inline-block;">PMID: <span class="docsum-pmid" style="box-sizing: inherit;">1761106</span></span> <span class="publication-type spaced-citation-item citation-part" style="box-sizing: inherit; display: inline-block; padding-left: 2.4rem;">Review.</span></div></div></li></ul><div><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 10pt; letter-spacing: 0px; text-align: justify;"><div style="color: black; font-family: arial;"><span style="color: #222222; font-family: Georgia, serif; font-size: 10pt; letter-spacing: 0px;">***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***</span><br clear="none" /></div></div><div style="text-align: justify;"><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px; letter-spacing: 0px;"><span style="font-size: 10pt; line-height: 1.22em;"><span style="font-family: Georgia, serif;"><br clear="none" /></span></span></div><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px; letter-spacing: 0px;"><span style="font-size: 10pt; line-height: 1.22em;"></span><div style="font-family: arial, helvetica; line-height: 1.22em;"><div style="line-height: 1.22em;">Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.</div></div><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a href="https://www..nature.com/articles/srep11573" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a href="https://www.nature.com/articles/srep11573" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank">https://www.nature.com/articles/srep11573</a></span><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"> </span><span style="font-family: arial, helvetica;"><br clear="none" /></span></div><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px; letter-spacing: 0px;"><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"><br clear="none" /></span></div><div><span style="color: #222222; font-family: Georgia, serif; font-size: 10pt; letter-spacing: 0px; line-height: 1.22em;"></span><div style="color: #222222; font-family: Roboto, sans-serif; font-size: 12px; letter-spacing: 0px; line-height: 1.22em;"><span style="font-size: 10pt; line-height: 1.22em;">O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations </span></div><span style="color: #222222; font-family: monospace; font-size: small; letter-spacing: 0px; line-height: 1.22em; white-space: pre;">
</span><div style="line-height: 1.22em;"><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="color: #222222; font-family: arial, helvetica; font-size: 10pt; letter-spacing: 0px; line-height: 1.22em;"><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"><span style="line-height: 1.22em;">Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***is the third potentially zoonotic PD (with BSE and L-type BSE), </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***thus questioning the origin of human sporadic cases. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">=============== </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***thus questioning the origin of human sporadic cases*** </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">=============== </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">============== </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /></span><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a href="https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a href="https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank">https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf</a></span><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"> </span><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /></span><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a href="http://www.tandfonline..com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></span><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"> </span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="color: #222222; font-family: arial, helvetica; font-size: 10pt; letter-spacing: 0px; line-height: 1.22em;"><span style="font-family: Georgia, serif; font-size: 10pt; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">PRION 2016 TOKYO</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Saturday, April 23, 2016</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Taylor & Francis</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Prion 2016 Animal Prion Disease Workshop Abstracts</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">WS-01: Prion diseases in animals and zoonotic potential</span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="line-height: 1.22em;">These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /></span><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a href="http://www.tandfonline..com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a href="http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank">http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20</a></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="color: #222222; font-family: arial, helvetica; font-size: 12pt; letter-spacing: 0px; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="font-size: 10pt; line-height: 1.22em;"><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Georgia, serif; line-height: 1.22em;">Title: Transmission of scrapie prions to primate after an extended silent incubation period) </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Georgia, serif; line-height: 1.22em;">*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Georgia, serif; line-height: 1.22em;">*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /><span style="font-family: Georgia, serif; line-height: 1.22em;">*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. </span><br clear="none" style="line-height: 1.22em;" /><br clear="none" style="line-height: 1.22em;" /></span><span style="font-family: Arial, sans-serif; font-size: 10pt; line-height: 1.22em;"><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank"><span style="font-family: Verdana, sans-serif; line-height: 1.22em;"></span></a><a href="http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160" rel="nofollow noopener noreferrer" shape="rect" style="color: purple; cursor: pointer; line-height: 1.22em;" target="_blank">http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160</a></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="color: #222222; font-family: arial, helvetica; font-size: 12pt; letter-spacing: 0px; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">GAME FARM INDUSTRY WANTS TO COVER UP FINDINGS OF INCREASE RISK TO CJD FROM CERVID</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">BSE INQUIRY</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">CJD9/10022</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">October 1994</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane </span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">BerksWell Coventry CV7 7BZ</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Dear Mr Elmhirst,</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended.. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">The statistical results regarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all. </span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><a href="http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf</a></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasized by the finding that some strains of scrapie produce lesions identical to the once which characterize the human dementias"</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the scrapie problem urgent if the sheep industry is not to suffer grievously.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">snip...</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">76/10.12/4.6</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><a href="http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf</a><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">IN CONFIDENCE</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">SCRAPIE TRANSMISSION TO CHIMPANZEES</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">IN CONFIDENCE</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">reference...</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">RB3.20</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">TRANSMISSION TO CHIMPANZEES</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">1. Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">2. We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, ilp and i/v) :</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">3. I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. Proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">4. In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">6. A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans' susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday' s meeting.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">R. Bradley</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">23 September 1990</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">CVO (+Mr Wells' comments)</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Dr T W A Little</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Dr B J Shreeve</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">90/9.23/1.1.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><a href="http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">IN CONFIDENCE CHIMPANZEES</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">CODE 18-77 Reference RB3.46</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Some further information that may assist in decision making has been gained by discussion with Dr Rosalind Ridley.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">She says that careful study of Gajdusek's work shows no increased susceptibility of chimpanzees over New World Monkeys such as Squirrel Monkeys. She does not think it would tell you anything about the susceptibility to man. Also Gajdusek did not, she believes, challenge chimpanzees with scrapie as severely as we did pigs and we know little of that source of scrapie. Comparisons would be difficult. She also would not expect the Home Office to sanction such experiments here unless there was a very clear and important objective that would be important for human health protection. She doubted such a case could be made. If this is the case she thought it would be unethical to do an experiment abroad because we could not do it in our own country.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Retrospectively she feels they should have put up more marmosets than they did. They all remain healthy. They would normally regard the transmission as negative if no disease resulted in five years.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">We are not being asked for a decision but I think that before we made one we should gain as much knowledge as we can. If we decided to proceed we would have to bear any criticisms for many years if there was an adverse view by scientists or media. This should not be undertaken lightly. There is already some adverse comment here, I gather, on the pig experiment though that will subside.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">The Gibbs' (as' distinct from Schellekers') study is somewhat different. We are merely supplying material for comparative studies in a laboratory with the greatest experience of human SEs in the world and it has been sanctioned by USDA (though we do not know for certain yet if chimpanzees specifically will be used). This would keep it at a lower profile than if we conducted such an experiment in the UK or Europe.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">I consider we must have very powerful and defendable objectives to go beyond Gibbs' proposed experiments and should not initiate others just because an offer has been made.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Scientists have a responsibility to seek other methods of investigative research other than animal experimentation. At present no objective has convinced me we need to do research using Chimpanzees - a species in need of protection. Resisting such proposals would enable us to communicate that information to the scientist and the public should the need arise. A line would have been drawn.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">CVO cc Dr T Dr B W A Little Dr B J Shreeve</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">R Bradley</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">26 September 1990</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">90/9.26/3.2</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica; font-size: 16px;"><a href="http://web.archive.org/web/20090506041605/http://www.bseinquiry.gov.uk/files/yb/1990/09/26003001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20090506041605/http://www.bseinquiry.gov.uk/files/yb/1990/09/26003001.pdf</a><br clear="none" /></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica; font-size: 16px;"><br clear="none" /></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica; font-size: 16px;">this is tse prion political theater here, i.e. what i call TSE PRION POKER...tss</span><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><a href="http://web.archive.org/web/20031028205208/www.bseinquiry.gov.uk/files/yb/1990/08/28002001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20031028205208/www.bseinquiry.gov.uk/files/yb/1990/08/28002001.pdf</a><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><a href="http://web.archive.org/web/20030822170317/www.bseinquiry.gov.uk/files/yb/1990/11/01005001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20030822170317/www.bseinquiry.gov.uk/files/yb/1990/11/01005001.pdf</a><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">snip...</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">PAGE 26</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Transmission Studies</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province! ...page 26. </span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">snip...see;</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">IN CONFIDENCE</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">PERCEPTIONS OF UNCONVENTIONAL SLOW VIRUS DISEASE OF ANIMALS IN THE USA</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">GAH WELLS</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">REPORT OF A VISIT TO THE USA</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;">APRIL-MAY 1989</span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica;"><span style="font-size: 16px;"><br clear="none" /></span></span></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><a href="http://web.archive.org/web/20090506002237/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">http://web.archive.org/web/20090506002237/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf</a><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="font-size: 10pt; line-height: 1.22em; margin: 0in 0in 0.0001pt;"><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;">why do we not want to do TSE transmission studies on chimpanzees $</div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;">5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. </div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;">***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. </div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;">***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.</div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;">snip...</div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><br clear="none" /></div><div class="yiv3874699054aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_aolmail_MsoNormal" style="line-height: 1.22em; margin: 0in 0in 0.0001pt;"><span style="color: #222222; font-family: arial, helvetica; font-size: 16px;"><a href="https://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf" rel="nofollow noopener noreferrer" shape="rect" style="color: blue; cursor: pointer;" target="_blank">https://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf</a></span></div></div></div></div></div></div></div></div><div style="font-family: "Helvetica Neue", Helvetica, Arial, sans-serif; font-size: 16px;"><div style="font-family: arial; font-size: 13.3333px;"><div></div></div><div style="font-family: arial; font-size: 13.3333px;"><br /></div><div style="font-family: arial; font-size: 13.3333px;">Terry S. Singeltary Sr.</div></div></div></div></div></div></div></div></div></div>Terry S. Singeltary Sr.http://www.blogger.com/profile/06986622967539963260noreply@blogger.com