Could Sporadic Creutzfeldt-Jakob Disease Be Underdiagnosed in China? Experience From Four Cases
Could Sporadic Creutzfeldt-Jakob Disease Be Underdiagnosed in China? Experience From Four Cases
Yi-Liu Zhang1 , Xiao-Mei Wu1 , Yang Chen1 , Wen-Ping Gu2 * and Wei Lu1 * 1 Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, China, 2 Department of Neurology, Xiangya Hospital, Central South University, Changsha, China
Background: Creutzfeldt-Jakob Disease (CJD) is a rapidly progressive neurodegenerative disease caused by the misfolded version of the cellular prion protein. Here we report four cases of sporadic CJD (sCJD) and describe the diagnostic methods available in order avoid missed or delayed recognition of CJD in China.
Case presentation: We report four patients diagnosed with sCJD between March 2018 and December 2019 at Xiangya Hospital and the Second Xiangya Hospital of Central South University. All patients were admitted to the hospital because of a progressive cognitive decline. Although their routine tests and biochemical indicators in the cerebrospinal fluid (CSF), as well as computed tomography (CT) imaging, did not reveal any apparent abnormalities, the presence of “cortical ribboning” was incidentally found on diffusion-weighted imaging (DWI). The patients were subsequently diagnosed with CJD based on positive testing for 14-3-3 protein in their CSF, and the presence of periodic sharp and slow wave complexes (PSWCs) on their electroencephalograms (EEG). Additionally, two of patients was confirmed pathological examination of cerebral biopsies demonstrating neuronal loss, gliosis, and spongiform changes.
Conclusions: CJD is a rare disease and is easily misdiagnosed by clinician in China due to a lack of recognition and awareness of CJD. Based on our experience described in this report, enhanced vigilance for CJD is required for patients with rapidly progressive dementia in China and other developing countries. DWI, EEG and detection of 14-3-3 protein in CSF should be performed in order to achieve a timely diagnosis of CJD.
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CONCLUSION
CJD is a rare disease, but the limited availability of more advance diagnostic methods combined with a lack of awareness about CJD in China and other developing countries may be leading to a missed diagnoses, misdiagnoses and low diagnostic rate. New, less invasive and more accessible diagnostic techniques as an alternative to brain biopsy, including EEG, DWI, RT-QuIC, 14-3-3 protein, and other biomarkers, could improve the accuracy of diagnosing prion diseases. We diagnosed four cases within a <2-year period, which alerts us to CJD being a public health concern that requires greater attention. In China, awareness should be generated for patients with rapidly progressive dementia in clinical practice, and patients should be examined by methods such as DWI, testing for 14-3-3 proteins in the CSF, RT-QuIC, and so on, in order to ensure a correct diagnosis.
Keywords: Creutzfeldt-Jakob disease, prion disease, neuroinfectious disease, PrPSC, rapidly progressive dementia
FRIDAY, OCTOBER 30, 2020
Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease
Eur Neurol 2020;83:65–72
Sporadic Creutzfeldt-Jakob Disease: A Retrospective Analysis of 104 Cases
Chang Qia, b Jia-Tang Zhanga, b Wei Zhaoc Xiao-Wei Xingb Sheng-Yuan Yua, b a Medical School of Chinese PLA, Beijing, China; bDepartment of Neurology, Chinese PLA General Hospital, Beijing, China; c Shijingshan Teaching Hospital of Capital Medical University, Beijing Shijingshan Hospital, Beijing, China Received: November 3, 2019 Accepted: March 5, 2020 Published online: April 28, 2020 Dr. Jia-Tang Zhang Department of Neurology, Chinese PLA General Hospital Medical School of Chinese PLA No. 28, Fuxing Road, Beijing 100853 (China) zjt1128@aliyun.com © 2020 The Author(s) Published by S. Karger AG, Basel karger@karger.com www.karger.com/ene DOI: 10.1159/000507189
Abstract
Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is an extremely rare fatal and infectious neurodegenerative brain disorder characterized by rapidly progressive dementia, cerebellar ataxia, and visual disturbances. This article summarizes the retrospective analysis of 104 sCJD patients in the First Medical Center of Chinese PLA General Hospital from 2003 to 2019.
Methods: A retrospective analysis of the medical records of the 104 patients diagnosed with sCJD was performed from the aspects of demographic data, clinical manifestations, laboratory examinations, cerebrospinal fluid analysis, electroencephalograms (EEGs), diffusionweighted imaging (DWI) scans, positron emission tomography (PET) scans, and prion protein gene mutations.
Results: In the 104 sCJD patients, pathological evidence of a spongiform change was found in 11 patients, while the remaining 93 patients were probable sCJD. The 104 patients included 57 males and 47 females, with the age of onset ranging from 29 to 82 (mean: 58, median: 60) years. The time from disease onset to death ranged from 1 to 36 months. Most of the patients died 7–12 months after the onset of sCJD. In most patients, rapidly progressive dementia appeared as the initial symptom, followed by cerebellar ataxia, visual disturbances, and neurobehavioral disorders. Most patients’ DWI images showed symmetric or asymmetric hyperintensity in the cortex. In terms of EEGs, 38.2% of the patients had periodic sharp wave complexes. The sensitivity of 14-3-3 protein detection was 34.1%. The brain PET scans of 50 patients with sCJD presented 96% sensitivity for the diagnosis of sCJD.
Conclusions: This study indicated that sCJD occurred at an early age in patients in China. The sensitivity of 14-3-3 protein detection was significantly low, but brain PET was highly sensitive in the diagnosis of sCJD.
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. In conclusion, this study retrospectively analyzed 104 patients diagnosed with sCJD pathologically and clinically. The characteristics of sCJD were summarized from the perspectives of demographic data and auxiliary examinations, and the results were compared with those from sCJD patients in other reports. This study may represent the characteristics of sCJD patients in China or even in Asia. In addition, PET-CT and PET-MRI examinations were important for the early diagnosis of sCJD due to their high sensitivity.
© 2020 The Author(s) Published by S. Karger AG, Basel
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017
This article was published in the following Dove Press journal: Clinical Epidemiology
Yu Sun 1,2 Chih-Ching Liu3, * Ling-Yun Fan4, * Chung-Te Huang5 Ta-Fu Chen2 Chien-Jung Lu1,2 Wan-Yuo Guo 6,7 Yang-Chyuan Chang2,8 Ming-Jang Chiu 2,9
Department of Neurology, En Chu Kong Hospital, New Taipei City, Taiwan; 2 Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan; 3 Department of Healthcare Administration, College of Medical and Health Science, Asia University, Taichung, Taiwan; 4 Queensland Brain Institute, University of Queensland, St. Lucia, Brisbane, QLD, Australia; 5 Center for Research, Diagnostics and Vaccine Development, Taiwan Centers for Disease Control, Taipei, Taiwan; 6 Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan; 7 School of Medicine, National Yang-Ming University, Taipei, Taiwan; 8 Department of Neurology, Min-Sheng General Hospital, Taoyuan, Taiwan; 9 Graduate Institute of Brain and Mind Sciences, College of Medicine, National Taiwan University, Taipei, Taiwan; 10Graduate Institute of Psychology, College of Science, National Taiwan University, Taipei, Taiwan; 11Graduate Institute of Biomedical Electronics and Bioinformatics, National Taiwan University, Taipei, Taiwan
*These authors contributed equally to this work
Correspondence: Ming-Jang Chiu Tel +886-2-23123456 ext 65339 Fax +886-2-23418395 Email mjchiu@ntu.edu.tw
Introduction: Epidemiologic studies of Creutzfeldt-Jakob disease (CJD) have been undertaken worldwide since the new variant CJD outbreak in 1996 in the United Kingdom. A nationwide report system, the Creutzfeldt-Jakob Disease Surveillance Unit (CJDSU), directed by the Centers for Disease Control of Taiwan, was established in 1997 to identify human prion diseases.
Methods: From 1998 to 2017, 647 cases were referred to the committee for confirmation. The report to CJDSU included a structured questionnaire recording the clinical, demographic data, and potential iatrogenic exposure, and the results of the clinical and laboratory examination, including tests of blood and cerebrospinal fluid, electroencephalography, and brain magnetic resonance imaging.
Results: In total, 356 cases (women, n=178) were ascertained to be human prion diseases, and 97.4% (n=347) were sporadic CJD, including three definite, 314 probable, and 30 possible cases; one probable variant CJD and 8 cases of the genetic form human prion diseases. The age- and gender-specific average annual incidence were also significantly higher in the second decade (0.95/1,000,000) than in the first decade (0.63/1,000,000), with an incidence rate ratio of 1.51. The incidences increased with increasing age, reaching a peak at the age of 70–79 years. The 10-year survival curve for sCJD patients showed that the 1-, 5-, and 10-year cumulative survival rate were 52%, 5%, and 1%, respectively. PRNP polymorphisms in 170 patients showed that 98.8% were M129M and 97.6% E219E.
Discussion: The significant increase in incidence after 2008 suggests the increase in the awareness of this rare disease among physicians. The longer disease duration in patients with sCJD in Taiwan than in other countries indicates that the comprehensive support of the health care system, as well as the end-of-life care culture in Taiwan, may prolong survival time in patients with such a progressive and fatal disease.
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Discussion
Our previous epidemiological research of CJD from 1998 to 2007 has been reported 10 years ago.3 In current analysis, we rechecked the information of all the ascertained cases of the first decade in the registry and found that 9 cases before 2005 should be excluded because they could not meet the diagnostic criteria with some missing data. Moreover, 2 cases that were identified in 2008 actually had their disease onset in 2007 by tracing their history again.
The overall incidence rate of CJD from 1998 to 2017 in Taiwan was 0.79 per million persons. The incidence significantly increased after 2008 to approximately 1 case per million persons per year, comparable to the worldwide incidence, typically reported to be approximately 1–2 per million person-years based on surveillance from 2005 onward.2,20 The increase in incidence for the second decade could be explained by the enhanced awareness of clinical physicians, the improved diagnostic tests, and the increase in the aging population in Taiwan, as the incidence of CJD cases, peaked in the 70- to 79-year-old age group similar distribution to the USA study. 21 The incidence of CJD onset after 80 years of age was still high, without a sharp decline, which was different from other reports.6,22 Since the start of the CJDSU in 1997, several national or international symposiums focused on human transmissible spongiform encephalopathy have been held by the Taiwan Neurological Society to educate neurologists and general physicians about the updated diagnostic criteria and management of CJD patients. The Taiwan CDC has regularly revised the workbook.23 In 2008, an imported case of vCJD was reported to CJDSU.19 This first acquired case of CJD in Taiwan was a critical public health concern and might have helped raise awareness of the disease among neurologists, leading to an increase in the number of reported cases afterward. In 2009, an updated set of criteria, including MRI findings, were proposed and soon adopted by the CJDSU.17 The pattern of high signal intensity had high sensitivity and specificity for the differential diagnosis, assisting in distinguishing sCJD from other neurological diseases and helping clinicians detect suspected cases early; this, in part, resulted in the increase in the number of referrals in subsequent years.
The polymorphism at codon 129 (M129V) of PRNP is a recognized genetic marker for susceptibility to CJD in Caucasians.24 In Europe, 51% of the general population has methionine/valine (MV) heterozygosity, while 37% has methionine homozygosity (MM).25,26 In East Asia, the MM genotype was found in 94% of Koreans27 and 92% of Japanese individuals.26 Ethnic Han Chinese, who account for over 95% of the Taiwanese population, has a remarkably high frequency (98%) of methionine homozygosity in the general population.28 Methionine homozygosity is high in both healthy individuals and diseased patients, with 98.8% of CJD patients in our study having the MM genotype and less than 2% carrying the MV genotype. Prion susceptibility and protective alleles can exhibit marked geographic differences, with the effects being different in the Asian and Caucasian populations.26
Because of the short disease duration in most cases, the increase in mortality with time parallels the temporal trend in incidence, as the mortality rate was significantly higher in the second decade than in the first decade. There was no difference in the incidence and mortality rates between men and women in our study. Some countries reported a higher incidence in women than men because of the larger number of women among older populations.21 The disease duration varied based on CJD types, with a relatively chronic course in some genetic forms. The mean survival time was 56.4 (range, 35.1–67.4) months in four GSS patients (P102L), 15.4 (9.0, 21.7) months in two E196A patients, and 11.9 months in an R148H patient, while a substantially shorter disease duration of 2.4 months was noted in a patient with a 72-base pair insertion. Because of the small number of gCJD patients in our study, it is challenging to compare Taiwan with other countries regarding these patients’ survival time.
Among the patients with sCJD, women and those with younger ages at onset had longer disease durations. This finding is comparable with the results from a collaborative multi-national CJD surveillance program (EUROCJD) conducted by the European Union and allied countries. The precise mechanisms underlying the effects of age and gender effects on survival time are not known. Agerelated variations in care or resistance to terminal infections have been proposed as possible explanations.29 Whether gender-specific factors influence the disease duration needs to be studied. In this national cohort, we found some long-term survivors of sCJD, with 3-, 5- and 10-year survival rates of 13%, 5%, and 1%, respectively. The median survival time was 13.5 months (mean: 19.1 months), and 48% died within one year of onset. Our data were similar to those in the report from Japan, in which the mean survival time of sCJD patients was 15.7 (range: 1–126) months, and 46.0% of all patients with prion disease died within one year. 29 However, reports from other counties revealed a much shorter survival time. The study by the EUROCJD involving 2,451 sCJD patients showed that the median survival time was five months (range: 1–81), and 85.8% died within one year of onset.11 The median duration in a study involving 150 definite or probable sCJD cases in Argentina was 4.6 (range: 1–70) months,30 while a Swedish study involving 123 patients with prion disease found that 74.6% of patients died within one year. 31 A study from China also showed a short survival time, with a median duration of 7.1 months (range: 1.0–23.3), and 78.5% of patients died within one year of onset.10
within one year of onset.10 The survival times of patients with sCJD in Taiwan and Japan were relatively longer than those reported in most of the other countries in the world. Japanese researchers explained the prolonged survival as the effects of their robust public medical insurance system and a culture allowing patients with end-stage neurological disease to receive intensive life-sustaining treatments such as tube feeding and intravenous high-calorie infusion.9,32 Taiwan adopted a single-payer National Health Insurance (NHI) system in 1995 that also provides comprehensive healthcare support.33 Patients with human prion disease do not need to pay any co-payment for outpatient or inpatient care. In addition, Taiwanese also share this end-of-life care culture.
There are several limitations to the current study. First, the percentage of definite cases of human spongiform encephalopathies was relatively small. Because of the traditional ethical values among the general population in Taiwanese society, the very low autopsy rate has long been acknowledged as an unfortunate and unavoidable reality. In recent years, using the real-time quaking-induced conversion assay to detect the pathological prion protein in the CSF or other tissues has been developed and has high diagnostic accuracy. 34 However, this technique was not used by the CJDSU in Taiwan during our study period. Without obtaining tissue specimens or testing for the pathological prion protein, we are not only unable to make a definite diagnosis but also are unable to assess the various molecular subtypes of sCJD, which is useful for phenotypic classification. Second, underreporting and underestimation of CJD are inevitable. Because of the older age at onset, patients with CJD may be misdiagnosed with other diseases with the rapid progression of neurological symptoms such as stroke,35 encephalitides,36 degenerative dementia,37 and epilepsy. 38 A retrospective archival survey published in 1995 showed that only approximately 60% of prion disease patients with pathologically spongiform encephalopathy were diagnosed clinically while alive. In recent decades, diagnostic accuracy has been improved by the use of CSF biomarkers and brain MRI.17 Not infrequently, the committee recommended that the primary care physician follow-up during the clinical course and perform MRI/EEG in case of uncertainty. They were obliged to report back at the next relevant meeting.
Furthermore, due to our NHI system’s comprehensive coverage, patients with spongiform encephalopathy received complete financial support covering all necessary MRI or EEG follow-up. There is nearly a consensus in clinical practice among neurologists in Taiwan that for those patients with rapid cognitive decline, in addition to CSF studies, MRI and EEG examination, tests to exclude autoimmune encephalitis or paraneoplastic encephalopathy should usually be performed. Third, the finding of 2% (8/356) of gCJD among all CJD forms was far lower than the corresponding figures elsewhere in the world, which range from 10–14%.25,39 Although the incidence of gCJD varies considerably among countries, we speculate that the lower proportion of gCJD in our study is likely because the PRNP gene sequencing test was not routinely performed before 2009, only 170 of the 356 CJD cases tested. Approximately 60% of genetic CJD cases were reported in patients with no family history, suggesting that they could have been misclassified in the absence of the PRNP genetic analysis.39
In conclusion, this study reports the 20-year epidemiologic features of CJD in Taiwan. The second decade’s incidence rate was comparable with the corresponding figures in most other countries in the world. The significant increase in incidence after 2008 suggests the increase in awareness of this rare disease among clinical physicians and the increase in Taiwan’s aging population. The longer disease duration in patients with sCJD in Taiwan than in Western countries indicates that the healthcare system and end-of-life care culture in Taiwan may prolong survival time in patients with such a rapidly progressive and fatal disease.
Keywords: human prion diseases, spongiform encephalopathy, incidence, mortality, disease duration
Sunday, November 1, 2020
Taiwan Incidence of and Mortality Due to Human TSE Prion Diseases Sees Significant Increase In Incidence After 2008
Taiwan Incidence of and Mortality Due to Human TSE Prion Diseases Sees Significant Increase In Incidence After 2008
Sunday, November 1, 2020
Taiwan Incidence of and Mortality Due to Human TSE Prion Diseases Sees Significant Increase In Incidence After 2008
SATURDAY, SEPTEMBER 26, 2020
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
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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.
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Sunday, December 27, 2020
First autopsy proven case of VPSPr: Variably protease‐sensitive prionopathy in Japan
Tuesday, December 15, 2020
Risk of Transmissibility From Neurodegenerative Disease-Associated Proteins: Experimental Knowns and Unknowns
THURSDAY, JULY 02, 2020
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
SATURDAY, AUGUST 01, 2020
Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons
SATURDAY, FEBRUARY 20, 2021
Abnormal prion protein deposits with high seeding activities in the skeletal muscle, femoral nerve, and scalp of an autopsied case of sporadic Creutzfeldt–Jakob disease
TUESDAY, DECEMBER 01, 2020
Sporadic Creutzfeldt Jakob Disease sCJD and Human TSE Prion Annual Report December 14, 2020
| VOLUME 20, ISSUE 3, P235-246, MARCH 01, 2021
Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease
Peter Hermann, MD Prof Brian Appleby, MD Jean-Philippe Brandel, MD Byron Caughey, PhD Prof Steven Collins, MD Prof Michael D Geschwind, PhD et al. Show all authors
Published:March, 2021DOI: https://doi.org/10.1016/S1474-4422(20)30477-4
Summary
Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrPSc). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. Supportive biomarkers, such as high CSF total tau, could aid the diagnostic process. However, discordant studies have led to controversies about the clinical value of some established surrogate biomarkers. Development and clinical application of disease-specific protein aggregation and amplification assays, such as real-time quaking induced conversion (RT-QuIC), have constituted major breakthroughs for the confident pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Updated criteria for the diagnosis of sporadic Creutzfeldt-Jakob disease, including application of RT-QuIC, should improve early clinical confirmation, surveillance, assessment of PrPSc seeding activity in different tissues, and trial monitoring. Moreover, emerging blood-based, prognostic, and potentially pre-symptomatic biomarker candidates are under investigation.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
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.
Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
APHIS-2021-0004-0002
APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission
June 17, 2019
APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission
Greetings APHIS et al,
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.
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.
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.
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.
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.
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.
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. ...
Comment from Terry Singeltary Posted by the Animal and Plant Health Inspection Service on Jun 19, 2019
WEDNESDAY, JANUARY 1, 2020 USDA OIE BSE TSE PRION FDA PART 589 BSE TSE PRION aka MAD COW FEED BAN Failure 2020 UPDATE
Terry S. Singeltary Sr.
posted by Terry S. Singeltary Sr. at
4:31 PM
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