CJD - Against All Odds: Daron Soard

Against All Odds: Daron Soard

Reported by: Tinisha Shade WCIA 3 Sunday, Nov 22, 2009 @11:15pm CST

January 2005 a handsome and healthy Daron Soard enlisted in the army. Later that year he graduated from Ranger School. Daron traveled the world on special missions. He excelled and was climbing the ranks. While on leave in 2006 he met and fell in love the woman who would soon be his wife. He proposed to Brandi a week after they met. One month later the two were married. Life was good. Then in 2007 Daron started getting sick. He spent a month in a hospital in Italy where he was stationed. Test after test. Then, the devastating news: His odds were one in a million but Daron had somehow contracted a rare disease. Creutzfeldt-Jakob disease attacks and eventually destroys the neuromuscular system, brain, and muscles including the heart. Doctors told him he was going to die. Brandi was expecting their first child. She still had about seven months until her due date, and according to doctors, Daron probably wouldn't make it Daron says, “My son was going to be born in the beginning or March, and to see him born, Captain Harris said that would be a miracle.” Thanksgiving came and went along with Christmas, and his first wedding anniversary Then, the day no one thought Daron would see. The birth of his son Dana Michael. “I think that was one of the main things that was pushing me just one more day, just to be able to see my sons face.” Even though Daron's survival has stumped the medical community his condition still takes a toll on his daily life. He's tired lost a lot of muscle mass, and takes several prescriptions everyday to control the pain. He says he knows he's here on borrowed time. Daron says he doesn't know why he's still here, but says he plans to live his life to the fullest, against all odds.

VIDEO

http://illinoishomepage.net/content/video/?cid=118871


Against All Odds: Daron Soard

http://illinoishomepage.net/content/fulltext/?cid=118871


Another sad nightmare from CJD. My heart and condolences goes out to Daron and his family. With what little information we have to date, we can only hope that Daron and his family are treated better than James Alford and his family was. many questions. WERE our soldiers getting the same food as our children in the USDA certified National School Lunch Program from Dead Stock Downer Cows? let's pray that they were NOT. from the video, Daron looks fairly young. young, old, does it really matter. another needless victim of CJD, and of course, with all politics that surround it, we will never know the route and source of this agent of the so called sporadic CJD's. ...TSS


Friday, August 22, 2008

Creutzfeldt Jakob Disease and Veterans and how they are treated at death

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/creutzfeldt-jakob-disease-and-veterans.html



CJD STAFF SGT. JAMES ALFORD 25


http://www.blackfive.net/main/2004/10/a_dying_hero_ss.html



http://www.blackfive.net/main/2004/03/ssg_james_alfor.html



http://creutzfeldt-jakob-disease.blogspot.com/2008/12/james-alford-succumbs-to-cjd-after-long.html



Monday, November 23, 2009

A case of atypical scrapie/Nor98 in a sheep from New Zealand

http://nor-98.blogspot.com/2009/11/case-of-atypical-scrapienor98-in-sheep.html



Wednesday, November 18, 2009

R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission


http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html



Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.


http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html



Thursday, November 12, 2009

BSE FEED RECALL Misbranding of product by partial label removal to hide original source of materials 2009


http://madcowfeed.blogspot.com/2009/11/bse-feed-recall-misbranding-of-product.html



Friday, September 4, 2009

FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009


http://madcowfeed.blogspot.com/2009/09/foia-request-on-feed-recall-product.html



Saturday, August 29, 2009

FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009


http://madcowfeed.blogspot.com/2009/08/foia-request-feed-recall-2009-product.html



2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006


http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html



Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 2009


http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



Thursday, November 05, 2009


Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification


http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html



PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE


http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/



FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008


http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf



Approximately 50.3 million pounds of the beef recalled by HallmarkNVestland went to federal nutrition programs, including the National School Lunch Program, and of those 50.3 million pounds, about 19.6 million pounds had already been consumed at the time the recall was issued. Release No. 0054.08, USDA, Transcript of Technical Briefing - HallmarldWestland Meat Packing Company (Feb. 21, 2008).

9. HSUS members that consume meat products, including beef products, are concerned about eating adulterated meat products and the health risks associated with such adulterated meat. Specifically, they are concerned that downed cattle are at an increased risk for harboring and transmitting BSE prions and other pathogens. The consumption of meat products derived from BSE-infected cattle is believed to cause a human neurological disease known as variant Creutzfeldt-Jakob disease ("vCJD"). The disease is progressive, invariably fatal, and there is no known effective treatment or cure. Downed cattle may also be at higher risk for harboring other foodborne transmissible pathogens, including E. coli 0157:H7, Salmonella, and anthrax. By allowing downed cattle to enter the food supply, USDA's regulatory loophole injures members of The HSUS by placing them at an increased risk of contracting these food-borne illnesses each time they eat beef. 10. Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.


http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf



please see full text here ;

Tuesday, November 17, 2009

SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2



http://downercattle.blogspot.com/2009/11/seac-effect-of-age-on-pathogenesis-of.html





Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1



http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html







TSS

A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom

A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom

Kenji Kamogawa‚Pj‚Qj, Takayuki Toi‚Pj, Kensho Okamoto‚Pjand Bungo Okuda‚Pj

Abstract

A 79-year-old woman was admitted to our hospital, due to acute onset of left hemiparesis and disturbance of consciousness. Although her symptoms improved temporarily, she developed gait disturbance and cognitive deterioration 2 months after the onset. After that, she presented with myoclonus and startle response, followed by akinetic mutism within 8 months after the onset. Serial EEGs revealed no periodic synchronous discharge. Serial diffusion-weighted MRIs showed that high intensity lesions, which initially limited to the right cerebral cortex, gradually spread to the bilateral cerebral cortices and basal ganglia, with relative sparing of central gyri, medial occipital cortices, and hippocampus. Prion protein gene analysis revealed a point mutation (Val¨Ile) at codon 180. The result of this patient suggests that this type of CJD might be associated with an atypical clinical course such as stroke-like episode and selective involvement of cortical and subcortical resions. Key words: Creutzfeldt-Jakob disease, Prion-protein, Codon180, MRI, Stroke-like onset (Nippon Ronen Igakkai Zasshi 2009; 46: 458\461) 1jDepartment of Neurology, Ehime Prefectural Central Hospital 2jDepartment of Geriatric Medicine, Medicine and Bioscience, Graduate School of Medicine, Ehime University


http://www.jstage.jst.go.jp/article/geriatrics/46/5/458/_pdf



Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification


http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html






TSS

Pewsey, died on October 22, more than six years after being diagnosed with Variant CJD

Family say goodbye at Calne funeral of CJD victim


2:36pm Thursday 12th November 2009



By Lewis Cowen »

Tribute was paid yesterday to the bravery of Edward Peduzie, from Calne who died aged 25 after a long battle with Creutzfeldt-Jakob Disease, CJD.

The former painter and decorator, who shared his time between the home of his father in Stokescroft and his mother's house in Pewsey, died on October 22, more than six years after being diagnosed with Variant CJD, the human equivalent of BSE, or mad cow disease, Friends believe the care given by Mr and Mrs Peduzie to their son was the reason he was one of the longest survivors of the incurable degenerative brain disease.

The funeral service took place at St Mary’s Church in Calne yesterday and Mr Peduzie’s coffin was carried in to the sounds of his favourite rap music.

Mr Peduzie’s mother, his father Paulsister Vicky and brother Benjamin led the mourners at the service attended by family and friends.

In his address, the Rev Bob Kenway told the congregation that Mr Peduzie was educated at Pewsey Vale School and qualified as a painter and decorator at Swindon College.

He worked for Calne building firm Wilkins before the disease made it impossible for him to continue with them.

Paul Peduzie told the congregation: “When Ed first became ill, if you asked him if he was all right, he would say, I’m all right.

“Then when he lost the use of his voice and you asked him the same question, he would give you a thumbs-up.

“When he lost the use of his hands, he just smiled. He was the bravest person I ever knew and I am proud to be his dad.”

After the congregation sang Jerusalem, Mr Peduzie’s coffin was carried out to the strains of Swing Low, Sweet Chariot. He had been a keen supporter of Rugby Union.

Although Mr Peduzie had been diagnosed with Variant CJD, doctors do not believe he contracted the disease from contaminated meat.

The family were supported through Mr Peduzie’s illness by the CJD Support Network and mourners were invited to make contributions to the charity.

Maggie Rae, the director of public health in Wiltshire, said CJD was an extremely rare disease.

She said: “I want to reassure people that this rare case poses no infection risk to the public. There have been fewer than 170 cases of Variant CJD in the UK since 1995.”

A new form of CJD, called Variant CJD, was identified in 1996. In May 1995, air cadet Stephen Churchill of Devizes was the first person to die from it. He was 19.

The Health Protection Agency said Variant CJD is strongly linked to people eating meat from cattle infected with BSE. However the possibility of contracting Variant CJD from surgical procedures cannot be ruled out.

The Department of Health says there have been four possible cases of Variant CJD transmission through blood transfusion.

As a result of this, the Government announced that people who had received a blood transfusion since January 1980 would be excluded from donating blood.


http://www.thisiswiltshire.co.uk/news/4736033.Family_say_goodbye_at_Calne_funeral_of_CJD_victim/




Greetings,


Interesting, sad, but interesting.


"Although Mr Peduzie had been diagnosed with Variant CJD, doctors do not believe he contracted the disease from contaminated meat."


Seems they may assume Mr Peduzie did not eat meat. That would be hard to assess fully over a lifetime i.e. cross contamination via processing of foods etc. however, nvCJD is as likely to transmit via Iatrogenic routes as the oral route, in my _opinion_. SO, we are left to ponder once again. That's the saddest part.


Interesting also, is the length of illness to death, from diagnostic date ;


"more than six years after being diagnosed with Variant CJD".


I wonder how long Mr Peduzie was subclinical ???



kind regards,
terry






Tuesday, November 03, 2009

SEVENTEENTH ANNUAL REPORT 2008 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK


snip...


2.3 Variant Creutzfeldt-Jakob Disease Up to 31st December 2008, 167 cases of definite or probable vCJD had been identified in the UK (115 definite, 49 probable who did not undergo post mortem and 3 probable cases still alive). Seventy-three (44%) of the 167 cases were women. The median age at onset of disease was 26 years and the median age at death 28 years (compared with 67 years for the median age at onset and 67 years for the median age at death for sporadic CJD). The youngest case was aged 12 years at onset while the oldest case was aged 74 years. To date, no case of vCJD has been identified in the UK in individuals born after 1989. The age- and sex-specific mortality rates for vCJD over the time period 1 May 1995 to 31 December 2008 are shown in Figure 6. The median duration of illness from the onset of first symptoms to death was 14 months (range 6-40) compared with a median duration of illness for cases of sporadic CJD of 4 months (range 1 to 74) during the period 1990-2008.


In 2008 the NCJDSU was referred for the first time an individual who met the clinical criteria in life for possible vCJD and who was heterozygous (methionine/valine) at codon 129 of the PRNP gene. This individual died in 2009 after a disease of 22 months duration. Consent for a post-mortem was not given. The clinical picture was typical of vCJD seen to date, which is reassuring for surveillance purposes since the clinicopathological phenotype of vCJD in this genotype is unknown. To put this possible vCJD case in perspective, it is useful to examine the final diagnostic outcome of the 116 suspect vCJD cases that were classified as possible vCJD at some point during their diagnostic pathway (that is, they met the criteria for possible vCJD at some point between referral to NCJDSU and death or other final diagnostic outcome). Of the 116 possible vCJD cases, 94 (81%) had a final classification of definite or probable vCJD, 10 (9%) had a final diagnosis of definite sCJD, 5 (4%) had alternative diagnoses to CJD (Alzheimer’s disease, Wilson’s disease, viral encephalitis, syphilis, SSPE), one was diagnosed with genetic CJD, one improved clinically and for one individual the diagnosis remains unclear, but clinically was suggestive of vCJD. Four cases (3%) have resulted in a final classification of possible vCJD, 3 were methionine homozygotes at codon 129 and the recent case heterozygous (methionine/valine) at PRNP codon 129. On the basis of our knowledge of the natural history of other human prion diseases, clinical cases of vCJD in PRNP codon 129 genotypes other than methionine homozygotes could be anticipated.



snip...see full text ;


http://www.cjd.ed.ac.uk/report17.pdf




http://creutzfeldt-jakob-disease.blogspot.com/2009/11/seventeenth-annual-report-2008.html





Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification



Abstract

Six subtypes of sporadic Creutzfeldt–Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrPSc types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrPSc types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in MV or VV subjects. The deposition of either type 1 or 2, when concurrent, was not random and always characterized by the coexistence of phenotypic features previously described in the pure subtypes. PrPSc type 1 accumulation and related pathology predominated in MM and MV cases, while the type 2 phenotype prevailed in VVs. Neuropathological examination best identified the mixed types 1 and 2 features in MMs and most MVs, and also uniquely revealed the cooccurrence of pathological variants sharing PrPSc type 2. In contrast, molecular typing best detected the concurrent PrPSc types in VV subjects and MV cases with kuru plaques. The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt–Jakob disease.

snip...






http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html






TSS

Surveillance of Creutzfeldt-Jakob disease in Australia: 2009 update

Communicable Diseases Intelligence Volume 33 No 2 - June 2009

Surveillance of Creutzfeldt-Jakob disease in Australia: 2009 update

This report provides a snapshot of Creutzfeldt-Jakob disease (CJD) in Australia for the period 1 April 2008 to 31 March 2009. In this short report, the Australian National CJD Registry provides undated Australian human transmissible spongiform encephalopathies (TSE) figures and discuss a recently published investigation of geographical TSE clustering in regional New South Wales.

Communicable Diseases Surveillance

Genevieve M Klug, Alison Boyd, Victoria Lewis, Amelia McGlade, Christiane Stehmann, Colin L Masters, Steven J Collins Abstract

In Australia, the occurrence of all human transmissible spongiform encephalopathies (TSEs) is surveyed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR). While prospective surveillance commenced in October 1993, the ANCJDR also retrospectively ascertained cases that occurred between 1970 and 1993. During the surveillance period of 1 April 2008 to 31 March 2009, the ANCJDR received 90 suspect TSE case notifications, which is slightly increased from previous annual surveillance periods. Based on the total number of probable and definite CJD cases, ascertained between 1993 and 2009, the Australian age-adjusted mortality rate is 1.18 deaths per million per year. In this short report, we provide updated Australian human TSE figures and discuss a recently published investigation of geographical TSE clustering in regional New South Wales. Commun Dis Intell 2009;33:188–191.

snip...

Australian National Creutzfeldt-Jakob Disease Registry surveillance update Since 1 October 1993, the ANCJDR has been notified of 1,345 suspected cases of CJD, arising in both the prospective and retrospective ascertainment periods. For the prospective period, the average annual rate of suspect cases notified to the ANCJDR was 3.1 per million per year. Fluctuations in these annual notification rates have been observed (Figure 1) and the reasons for these have been discussed previously.3 More recently, the rate of notifications from 2006 to 2008 has been sustained at a higher level in comparison to the longer term average observed for 1993–2008. The increased number of notifications is most likely underpinned by increased referrals to the ANCJDR of cerebrospinal fluid for 14-3-3 protein testing, particularly for the 2007–2008 period where CSF referrals have increased by 43% in comparison with the previous 6 year average. This sustained increased level of CSF referrals has given the ANCJDR confidence that the introduction of 'fee-for-service' from 1 January 2007 has not detrimentally affected CSF referrals and consequently rates of suspect case notification, as initially speculated.

Figure 1: Annual age-standardised Creutzfeldt-Jakob disease mortality rates and suspect case* notification rates, 1993 to 2008

During the 2008–2009 reporting period to 31 March 2009, 90 suspect cases were added to the Register, which is a 17% increase from the previous surveillance year. Of these 90 suspect case notifications, 20 have been reclassified as definite TSE cases. A further 15 cases that were notified prior to 1 April 2008 have also been confirmed as definite (11) or probable (4) TSE since the last update. The Register has a large group of cases still under investigation (184), with the majority of these still alive as at 31 March 2009. Despite active investigation of all suspect cases, the number of incomplete cases continues to expand, although final outcomes for the large majority of all suspect cases have been obtained through detailed investigation. The number of incomplete cases is of concern to the ANCJDR as for many of these cases, a conclusive outcome may not be achievable. Follow-up can be difficult for cases that have been notified several years previously. In 541 cases, CJD has been excluded while 608 cases have been classified as definite (395) or probable (213) CJD and a further 12 cases fulfil the possible case definition (Table 1). A sustained elevation of the annual CJD incidence and the proportions of autopsy-confirmed cases for the surveillance years of 2005–2008 has been observed (Figure 2). As previously discussed, this relates to the growing number of notifications and pro-active approach of the ANCJDR of seeking autopsy in all clinically suspect cases.3

Table 1: Classification of cases by the Australian National Creutzfeldt-Jakob Disease Registry, 1 January 1970 to 31 March 2009

snip...

Age-standardised mortality rates were calculated using the Australian Bureau of Statistics 2000 estimated resident population for Australia.

The aetiologic proportions of all Australian TSE cases are consistent with previous observations.3 Cases classified as sporadic CJD comprise 90.5% of all Australian cases, while 8.2% of cases are genetic and the remaining 1.3% are iatrogenic. During the 2008–2009 surveillance period, 4 new cases of familial TSE were classified, while no further iatrogenic CJD and no cases of vCJD were identified in Australia.

Based on the 608 definite and probable cases, TSE incidence peaks at 4.9 cases per million per year in the 65–69 year age group, an incidence almost 5 times the reported global incidence. As sporadic cases comprise the majority of cases, the peak incidence in this group closely aligns with overall TSE rates. Since the last surveillance period, the median age at death for sporadic cases has remained unchanged; 66 years (males, 65 years; females, 67 years), the proportion of female sporadic cases has remained consistent at 53% and their median duration of disease similar at 4 months. A slightly shorter disease duration is observed in males (median, 3 months). Genetically determined TSEs have a younger age at death (medians, overall 59 years; males, 51 years; females, 62 years) and longer illness duration (medians, overall 6 months; males, 4 months; females, 7.5 months) when compared to sporadic cases. The sex ratio for familial cases is slightly biased towards females with 58% of the cases being female.

Based on all definite and probable TSE cases, the average, age-adjusted mortality rate in Australia for the 1970–2009 period is 0.88 deaths per million per year. A restriction of the timeframe to the prospective surveillance period of 1993–2009 provides a more reliable representation of the true national figures with a mortality rate of 1.18 deaths per million per year. By individual state and territory, the TSE mortality rates (Table 2) in some jurisdictions have altered since the previous update.3 Notably, the average age-standardised mortality rates during 1993–2009 have increased in Western Australia, the Australian Capital Territory and the Northern Territory, while in Tasmania, the rate has continued to decline and is currently around half the rate observed in Victoria and Western Australia (Table 2). This strongly suggests case under-ascertainment in Tasmania for this specific period. In the remaining states and territories, no significant changes in the longer-term mortality rate average (1993–2009) have occurred since last reported.

Analysis of incidence rates by state and territory over the last decade highlights the strengths and weaknesses of surveillance in the various regions (Table 2). In Victoria, case numbers have remained constant over this period and have resulted in the highest mortality rate in Australia. In contrast, a lower than expected mortality rate in Queensland, South Australia and Tasmania was observed. This decline was concerning as the 10-year timeframe provided us with a recent snapshot of confirmed cases, excluding the diluting influence of the earlier prospective surveillance years. Broadened surveillance and diagnostic responsibilities, changes to privacy legislation around 2000 and less accessible autopsy services in various regions may have contributed to the lower mortality in the specific states.

snip...

Suspect case notification between this and the previous reporting period have remained stable in the Australian Capital Territory, the Northern Territory, South Australia and Western Australia (Figure 3). In contrast, a 70% increase in notifications has been observed in Victoria. While the total number of CSF referrals arising in Victoria has remained unchanged, there has been an increase in the number of clinically suspect TSE cases added to the Register, derived from CSF referrals. Marked declines in notifications in the large populations of New South Wales and Queensland, and to a lesser degree in Tasmania, were observed in 2008. The impact of these lower notifications may be reflected in a reduced number of confirmed CJD cases for this period. A contractual agreement between the ANCJDR and Queensland Health Department was established in May 2008 to evaluate all cases of suspect TSE. The impact of this agreement on TSE incidence in Queensland will be of particular interest.

snip...

Analysis of a potential Creutzfeldt-Jakob disease cluster During 2008, the ANCJDR published findings from an investigation conducted assessing an increased number of sporadic CJD cases within a coastal region of New South Wales during the period 1993–2006.4 Statistical analysis identified a spatially significant cluster in 3 contiguous statistical local areas, consisting of 13 definite and 1 probable CJD case. An epidemiological review of ANCJDR case data for the 14 cases did not reveal a plausible cross-over or point source transmission event to explain the cluster of cases.

One potential hypothesis for the significant finding related to the region's clinicians and their management of potential CJD cases. To investigate this theory, further evaluation was undertaken comparing the regional area with the entire state, emphasising rates of referrals for 14-3-3 CSF testing, rates of case notification to the ANCJDR and suspect CJD post-mortem rates. These observations were chosen to objectively quantify an intensity of surveillance and how this relates to incidence rates.

Our analyses demonstrated that the cluster area maintained a higher level of surveillance and clinical awareness compared with the entire State of New South Wales. The population-based rate of notification of all suspect cases to the ANCJDR was 68% greater in the cluster area than for New South Wales (age-adjusted RRMH: 1.68, 95% CI=1.36–2.10) and similarly, the population-based rate of request for CSF testing was 59% greater than the state referral rate (age-adjusted RRMH: 1.59, 95% CI=1.25–2.02). No difference between the likelihood of a suspect case being confirmed as probable or definite CJD (all types or sporadic only) was observed, suggesting that once CJD was questioned as a diagnosis in a clinical setting, the likelihood of a case being assessed for CJD classification was no different in the circumscribed area to the entire state. In contrast, a difference did exist in the proportion of cases that were assessed by neuropathological examination (biopsy or autopsy), with the cluster area having an almost two and half times greater neuropathological examination rate in suspect cases compared with New South Wales (age-adjusted RRMH, 2.34, 95% CI=1.56–3.51). Simply stated, approximately double the intensity of surveillance translated to a doubling of the CJD incidence rate.

One of the distinguishing features of the 14 cluster cases provided another key piece of supporting evidence for enhanced surveillance. The cohort displayed a significantly older age at death when compared with sporadic CJD cases from New South Wales and Australia overall. Analysis of autopsy data in Austria, where autopsy of all suspect CJD cases is mandatory, suggests global under-ascertainment of older age CJD cases.5 Hence, the ability to detect older and less typical cases in this cluster region suggests clinicians manifested a greater than usual suspicion of CJD and atypical presentations.

These findings have provided us with a hypothesis that intensity of surveillance for rare disorders can be quantified and this can positively correlate with higher incidence. It further suggests that the true incidence of CJD in Australia may be almost twice the currently observed average rate of 1.18 cases per million per year. A further exploration of this hypothesis is needed within and between individual nations and may give us an improved understanding of methodologies for optimal surveillance for rare conditions such as CJD.


http://www.health.gov.au/internet/main/publishing.nsf/Content/cda-cdi3302d.htm



http://www.health.gov.au/internet/main/publishing.nsf/Content/cda-cdi3302-pdf-cnt.htm/$FILE/cdi3302d.pdf



http://www.health.gov.au/internet/main/publishing.nsf/Content/health-pubhlth-strateg-phi-index.htm



Surveillance of Creutzfeldt-Jakob disease in Australia: 2008


http://www.health.gov.au/internet/main/publishing.nsf/Content/cda-cdi3202d.htm



http://www.health.gov.au/internet/main/publishing.nsf/Content/cda-cdi3202-pdf-cnt.htm/$FILE/cdi3202d.pdf



Do Reports of sCJD Clusters Matter? January 13, 2004 JP Morgan North American Equity Research

There have been seven cases of human sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 2.1 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. · There is no proven link between sCJD and BSE, and hence it is considered a different disease from vCJD (which has been linked to BSE). However, the existence of clusters raises the question of "contamination" or "infection", and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. · Clusters are not spontaneous, they normally have a source. Moreover, some cases of sCJD may have been improperly diagnosed as Alzheimer's.· We continue to believe that as long as no further cases of BSE-positive cows are found in North America and the industry has respected the 1997 ban on animal feed for live cattle, beef consumption in the US will not suffer. · Moreover, due to political pressure we expect key overseas markets (Japan, South Korea, and Mexico) to open up to US beef in the next six months - the recent 20% drop in cattle prices can be attributed mainly to these import bans. · However, two concerns linger and should be kept in mind by investors, 1) Has the 1997 ban on animal feed for live cattle been honored by the beef industry? 2) Can clusters of cases of sporadic CJD (or sCJD) really be a variant of CJD and indeed be linked to BSE? In this note we focus on the issue of sCJD clusters, and the potential impact that the growing debate on clusters could have on beef consumption in the US. United States Foods

Pablo E. Zuanic(1-212) 622-6744pablo.zuanic@jpmorgan.comChristopher
M. Bledsoe(1-212) 622-6386christopher.m.bledsoe@jpmorgan.comDaniel
Ogbonna(212) 622-6382daniel.c.ogbonna@jpmorgan.com

State of Our Views Regarding BSE in the US We continue to believe that as long as no further cases of BSE-positive cows are found in North America and the industry has respected the 1997 ban on animal feed for live cattle, beef consumption in the US will not suffer. Moreover, due to political pressure we expect key overseas markets (Japan, South Korea, and Mexico) to open up to US beef in the next six months - the recent 20% drop in cattle prices can be attributed mainly to these import bans.

· However, two concerns linger and should be kept in mind by investors, 1) Has the 1997 ban on animal feed for live cattle been honored by the beef industry? The government's General Accounting Office says it has not; 2) Can clusters of cases of sporadic CJD (or sCJD as it is commonly known) really be a variant of CJD and indeed be linked to BSE (vCJD is the scientific term for the disease linked to mad cow)?

· In this note we focus on the issue of sCJD clusters.

· Do sCJD Clusters Matter?

· The media focus (and as a result, the consumer at large) since December 23, thus far, has been on the potential of new BSE-positive cows being found, and on the various initiatives the authorities are taking to prevent an outbreak of BSE. However, the apparent existence of sCJD clusters in the US has received little publicity. If sporadic (or spontaneous) CJD is really spontaneous, it should not be found in population clusters. The fact that it indeed has been found in clusters raises concerns.

· Understanding the "Difference" Between sCJD and vCJD

· Prior to 1996 there was only one known type of CJD, and it was called "sporadic" or "spontaneous" because it was unclear where it came from, or how it was generated. In 1996 scientists in England "discovered" a "variant" of CJD (vCJD), which they indicated could be linked to the animal form of the disease (BSE or Mad Cow). Experts kept vCJD separate from sCJD because unlike the new vCJD the original sCJD could not be directly linked to BSE. However, not enough is known to be fully certain that sporadic CJD is truly spontaneous and has no external catalyst. The other notable difference between vCJD and sCJD is the incubation period. Whereas sCJD has an average incubation period of 40 years and is exceedingly rare in young people, vCJD can affect people of all ages and has a much shorter incubation period of just two to five years. An even more relevant difference is that sCJD is found in 1 out of 1 million people per annum, or 5,000 cases per year on a global basis, while only 180 human cases of vCJD (the type of CJD linked to BSE) have ever been reported.

· Existence of Clusters of sCJD May Imply They Are Really Cases of vCJD There have been seven sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 1.2 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. The existence of clusters raises the question of "contamination" or "infection", and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. Clusters are not spontaneous, they normally have a source.

· A cluster consists of two statistical improbabilities: 1) multiple cases occurring in a relatively limited geographic area, and 2) multiple cases occurring within the same time period. The most recent cluster was found in Cherry Hill, New Jersey. The others have been found in Lehigh, Pennsylvania (1986-90), Allentown, Pennsylvania (1989-92), Tampa, Florida (1996-97), Oregon (2001-02), and Nassau County, New York (1999-2000). Given that sCJD occurs randomly in one out of one million cases, it is a statistical rarity to find an sCJD cluster - let alone six. The following tables highlight known clusters in the US.

· Table 1: Clustered sCJD Deaths

· Local sCJD Deaths

· Time Span State Local Area Pop. (MM) Period (mo.) Total Ann'lized 1986-1990 PA Lehigh Valley 0.5 48 18 4.5 1989-1992 PA Allentown 2.5 36 15 5.0 1996-1997 FL Tampa 2.2 18 13 8.7 1996-1999 TX Denton .01 38 4 1.3 1999-2000 NY Nassau County 1.3 12 7 7.0 2001-2002 OR Entire State 3.4 24 14 7.0 2000-2003* NJ Cherry Hill Area 1.7 36 12 4.0 Source: JPMorgan.

The second table, below, shows what portion of the state's total expected sCJD cases (as based on a one per million occurrence) were found in the local cluster, comparing the local cluster's portion of cases with the local area's portion of the state's total population. The greater the factor between the former and the latter suggests a higher statistical improbability that the cluster is spontaneous (sCJD).

· Table 2: Clustered sCJD Deaths vs. Expected State Cases

· Annual Statewide Local Area (% of:)

· Time Span State Local Area sCJD Deaths* exp. state cases state pop. 1986-1990 PA Lehigh Valley 11.9 37.8% 4.5% 1989-1992 PA Allentown 12.0 41.7% 20.8% 1996-1997 FL Tampa 14.1 61.5% 15.7% 1996-1999 TX Denton 20.9 6.1% .02% 1999-2000 NY Nassau County 18.1 38.7% 7.4% 2001-2002 OR Entire State 3.4 205.9% 100.0% 2000-2003* NJ Cherry Hill Area 8.0 50.0% 21.6% * *State cases are extrapolated based on state population and the 1 per million national average. Source: JPMorgan.

The CDC Is Currently Investigating the New Jersey Cluster The US Center for Disease Control (CDC) has opened an investigation into a cluster of deaths in an area surrounding Cherry Hill, New Jersey. Specifically, after dismissing the case when it was first brought to their attention earlier in 2003, the agency has since reversed course and on December 31, 2003 sought out information from Janet Skarbek. Skarbek, a Cinnaminson, New Jersey CPA believes she has uncovered a common link between seven deaths in the local area and a restaurant at the now-closed Garden State Race Track. All of the deaths had first been identified as the randomly occurring (one out of one million) cases of sporadic Creutzfeldt-Jakob Disease (sCJD), and six of the deaths occurred between 2000 and 2003.

Science Expanding its Knowledge of CJDs

Have Cases of sCJD Been Overlooked?

Dr. Omar Bagasra believes that a 29 year old that died of presumably sCJD in the New Jersey cluster may have died from a new, mutated form of CJD since sCJD has a typical incubation period of 40 years and is limited to elderly patients in almost all cases. Moreover, he suspects that the link between the seven local deaths (clustered geographically and chronologically) indicates that the new form of the disease is caused by some external catalyst, unlike the randomly occurring sporadic CJD (sCJD). He adds, though, that there may actually have been other unreported CJD-related deaths in the area since the disease is often misdiagnosed as Alzheimer's.

Diagnoses of Alzheimer's Might Have Been Cases of CJD

Lawrence Schonberger, the CDC epidemiologist who contacted Janet Skarbek on December 31, is quoted separately as saying that sCJD is underreported on death certificates, and that about 14 percent of cases are missed. In fact, due to similarities between sCJD and Alzheimer's disease, a 1998 Yale study found that as many as 13 percent of Alzheimer's deaths are actually sCJD, but conservative estimates place this number closer to 1 percent. If we extrapolate this finding to the 50,000 Alzheimer's deaths each year in the US, the number of actual sCJD deaths per year is somewhere between 500 and 6500. But, for us this raises additional questions, since at a rate of one per million, the US should not experience much more than 300 sCJD deaths in a single year. Furthermore, Alzheimer cases have grown 50-fold in the last 25 years from 857 cases in 1979 to 50,000 cases in today (albeit part of the increase could very well be attributed to improvements in reporting).

Can sCJD Be Caused by External Agents?

A recent study out of Imperial College in London has led some to believe that the same prions that cause the BSE-related vCJD may also cause a disease that manifests itself in a way that more closely resembles sporadic CJD. John Collinge, the scientist that conducted the experiment, is basing this assertion on findings in the study's mice, which were injected with BSE prions. As expected, some of the mice developed symptoms from vCJD, but unexpectedly, others suffered from symptoms that more closely resembled sCJD. If true, the implications are significant, as it will force scientists to consider whether cases of sporadic CJD may actually have been caused by consumption of contaminated beef.

Does All This Matter?

For now we await the results of the CDC investigation of the New Jersey cluster. Previous investigations have found clusters to be just coincidences. While this may be the case, we believe that the media may start focusing more on the issue of clusters, and that the debate could raise consumers' concerns about beef. But even in the worst case scenario that these clusters were indeed linked to BSE, one could still make the argument that these cases were generated before the 1997 ban on animal feed for cattle was imposed, and that hence chances of contamination since then are unlikely. Still, the question lingers, has the 1997 ban been respected? Will consumers concerns increase as the discussion of CJD clusters hits the national media? Bottom Line: If no new cases of BSE-positive cows are found and the issue of CJD clusters is disregarded by consumers, then the effect on beef consumption will be negligible. On the other hand, new cases of infected cows and/or a wider debate of CJD clusters could indeed have an effect on beef sales. ...END


Subject: J.P. MORGAN INVESTORS WARNED ABOUT POTENTIAL SPORADIC CJD AND BSE RELATION From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy Date: Mon, 19 Jan 2004 08:33:38 -0600 Content-Type: text/plain

https://lists.aegee.org/cgi-bin/wa?A2=ind0401&L=BSE-L&T=0&F=&S=&P=25337&X=14C6D9515C8B05410B



BSE (Mad Cow) Update: Do Reports of sCJD Clusters Matter?

snip... see full text ;

http://cjdtexas.blogspot.com/



Thursday, November 05, 2009

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html



Friday, October 23, 2009

Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008

http://cjdtexas.blogspot.com/2009/10/creutzfeldt-jakob-disease-surveillance.html



TSS

CJD GERMANY UDPATE 2009

----- Original Message -----
From: Terry S. Singeltary Sr.
To: TERRY SINGELTARY
Sent: Tuesday, October 27, 2009 12:36 PM
Subject: CJD GERMANY


CJK in Deutschland


Stand 06.10.2009


Jahr sicher wahrscheinlich möglich GSS FFI genetische


CJD


Iatrogen vCJK Inzidenz


1993 24 8 4 1 0 0 0 - 0,7
1994 45 27 26 0 2 4 1 - 0,9
1995 64 23 15 1 2 2 0 - 1,1
1996 55 34 22 1 5 5 1 - 1,1
1997 73 34 31 1 1 6 1 - 1,3
1998 63 54 11 1 3 7 0 - 1,4
1999 68 35 5 0 1 9 1 - 1,3
2000 53 55 4 2 1 7 1 - 1,3
2001 69 55 11 0 3 8 0 - 1,5
2002 52 46 6 0 2 7 0 - 1,2
2003 52 63 8 1 1 3 3 - 1,4
2004 80 60 5 1 4 4 0 - 1,7
2005 62 82 9 0 5 9 2 - 1,8
2006 61 80 8 0 4 5 1 - 1,7
2007 48 83 14 0 3 1 0 - 1,6
2008 57 78 9 0 3 0 0 - 1,6
2009 16 70 8 1 4 1 0 - 1,4*



http://www.cjd-goettingen.de/CJK_in_D.pdf




suspected CJD cases <50 years


http://www.cjd-goettingen.de/Tabelleu50-e.html



http://www.cjd-goettingen.de/Grafiku50.pdf




vCJD cases worldwide


http://www.cjd-goettingen.de/vcjd-e.html




see links ;


http://www.cjd-goettingen.de/Links-e.html




Thursday, November 05, 2009



Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification


http://creutzfeldt-jakob-disease.blogspot.com/2009/11/incidence-and-spectrum-of-sporadic.html




Friday, October 23, 2009



Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008


http://cjdtexas.blogspot.com/2009/10/creutzfeldt-jakob-disease-surveillance.html






TSS

Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

Piero Parchi Æ Rosaria Strammiello Æ Silvio Notari Æ Armin Giese Æ Jan P. M. Langeveld Æ Anna Ladogana Æ Inga Zerr Æ Federico Roncaroli Æ Patrich Cras Æ Bernardino Ghetti Æ Maurizio Pocchiari Æ Hans Kretzschmar Æ Sabina Capellari

Received: 30 June 2009 / Revised: 16 August 2009 / Accepted: 17 August 2009 / Published online: 29 August 2009


The Author(s) 2009. This article is published with open access at Springerlink.com

Abstract

Six subtypes of sporadic Creutzfeldt–Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrPSc types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrPSc types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in MV or VV subjects. The deposition of either type 1 or 2, when concurrent, was not random and always characterized by the coexistence of phenotypic features previously described in the pure subtypes. PrPSc type 1 accumulation and related pathology predominated in MM and MV cases, while the type 2 phenotype prevailed in VVs. Neuropathological examination best identified the mixed types 1 and 2 features in MMs and most MVs, and also uniquely revealed the cooccurrence of pathological variants sharing PrPSc type 2. In contrast, molecular typing best detected the concurrent PrPSc types in VV subjects and MV cases with kuru plaques. The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt–Jakob disease.


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Discussion


Previous studies have addressed the issue of PrPSc types 1 and 2 co-occurrence in sCJD. Most of them raised the question of the influence of the number of cases and brain areas analyzed and emphasized the possibility that the cooccurrence of PrPSc types 1 and 2 is underestimated [13, 18, 20, 30, 37, 40, 43]. On the other hand, the use of a novel, potentially very sensitive approach, later shown to have pitfalls related to the detection of unspecific bands generated by partially digested PrPSc fragments [25], likely led other investigators to overestimate the incidence of the concurrent PrPSc types [35, 45]. Thus, the overall results on the phenomenon of the coexistence of molecular and clinico-pathological sCJD subtypes are at present inconclusive with respect to incidence, effect on disease phenotype and criteria for disease classification. To contribute to the full understanding of these issues, in the present study, we combined a systematic analysis of several brain regions in a large series of case including all codon 129 genotypes and the rarest phenotypes with the use of a refined methodology for the detection of the PrPSc type concurrence, which provides good sensitivity combined with high specificity [25].

After screening about 4,200 samples from a largely consecutive series of 200 cases, we estimated that PrPSc types 1 and 2 coexist in about 35% of sCJD cases, which is overall consistent with figures from some of the previous studies [13, 37, 43] in which the number of cases and areas analyzed were significantly lower. This finding supports the idea that PrPSc types co-occurrence involves a relevant but limited group of sCJD subjects and indicates that the incidence of the phenomenon had not been significantly underestimated.

As far as the characteristics of the CJD population with mixed phenotypes are concerned, our data show that the PrPSc types 1 and 2 co-occur more frequently in the MM than in the MV and VV genotypes. More specifically, the large majority of sCJD cases with concurrent PrPSc types combines features of the MM and MM 2C sCJD subtypes, in variable proportions. Most commonly, in such cases, the MM1 phenotype is predominant over the MM 2C phenotype, but the opposite situation also rarely occurs. The latter results significantly differ from those obtained in most previous studies. Indeed, Head et al. [13] mainly found a focal type 1 co-occurrence in MM and MV subjects with dominant type 2, Schoch et al. [40] detected the mixed protein types mostly in MV2 cases showing the type 1 only focally in subcortical areas, and Uro-Coste et al. [43] mainly detected a random co-occurrence of type 1 in MV or VV cases with dominant type 2. Given that only our study was based on a large series of consecutive cases, we attribute such heterogeneity of previous results to case selection biases, although methodological differences may also have contributed [43].

Since subjects with mixed PrPSc types represent a significant proportion of the sCJD population, show distinctive phenotypic features, and potentially represent a distinct subtype in terms of biological relevance, it is important that they are properly identified and are added as new subtypes in the current sCJD classification (Table 6). Despite the emphasis on molecular features of current sCJD classification, it has become increasingly clear that PrPSc typing alone, when limited to a single or even a few brain samples, fails to provide an accurate classification in a significant proportion of cases. This is mainly related to the focal nature of the ‘‘mixed features’’ in many sCJD cases with PrPSc types 1 and 2 concurrence. Indeed, we would have misclassified the disease subtype in about 27.5% (using 3F4) of cases with MM or MV genotype, if we had analyzed PrPSc only in the frontal cortex, the area more commonly used for typing worldwide. For the same reason, discrepancies may arise when PrPSc-typing and PrP immunohistochemistry are performed from individual samples taken from opposite hemispheres or even adjacent cortical gyri. However, our study shows that the regional deposition of either type 1 or type 2 when concurrent is not random and that a relatively limited number of critical brain structures must be assessed to reach an accurate classification. Furthermore, our results further underline the importance of applying both molecular and neuropathological assessment for sCJD subtype classification. In this regard, the lack of detection of PrPSc type 2 in a minority of MM subjects, despite the presence of a mixed synaptic and perivacuolar pattern of PrP deposition, indicates that when type 2 is very focal or limited in amount, histopathologic examination is more sensitive in identifying such cases than PrPSc typing, at least when only the 3F4 antibody is used. Given the very strong correlation in MM subjects between PrPSc type 2 detection and the large ‘‘grape-like’’ vacuoles and the perivacuolar pattern of PrP deposition on histopathologic examination, which is in line with results previously obtained in other studies [18, 37], we propose that these cases are classified as MM 1?2C or MV 1?2C even without the final proof of type 2 detection by western blot. Alternatively, PrPSc typing using the antibody 1E4 was in our hand as sensitive as the histopathologic examination in the detection of cases with very focal type 2. In the light of the present results, the most important regions to be assessed pathologically include the cerebral cortex from each of the 4 lobes, the striatum, hippocampus, thalamus and cerebellum. The cerebellum, in particular, is critical for the recognition of the synaptic pattern of PrP deposition as marker of PrPSc type 1 concurrence in the cases with dominant type 2.

Taken together, our data indicate that a protocol including the neuropathologic assessment of the eight brain regions mentioned above and PrPSc typing in four critical regions such as the temporal, parietal and occipital neocortices, and medial thalamus is strongly recommended for a reliable sCJD group classification addressing the issue of mixed phenotypes. Indeed, by applying this protocol instead of examining all 21 brain regions, we would have reached the same classification of cases in the present series.

We also wish to underline the importance of identifying correctly the sCJD cases with mixed features for transmission purposes. Indeed, the question of whether the concurrence of PrPSc types 1 and 2 in CJD reflects a coinfection by two prion strains related to specific undiscovered human genotypes, or determined by epigenetic factors remains unanswered and will largely rely on transmission studies in which the careful selection of samples will be of critical importance. Concerning this critical question, we find intriguing that the large, confluent vacuoles and the perivacuolar pattern of PrPSc deposition, we originally linked to sCJD MM 2C are also found in a subgroup of MV 2K subjects in addition to MM/MV 1?2C. In addition, we have described here the same morphological features in one case of fatal insomnia (i.e. the MM2-thalamic subtype or MM 2T) which adds to two previously reported cases [19, 30, 31]. Thus, it seems that large confluent vacuoles and the perivacuolar pattern of PrPSc deposition may be found in sCJD associated with all phenotypes linked to MM or MV at codon 129. Although this observation remains difficult to interpret at present, it appears relevant for our future understanding of the molecular basis and the extent of strain variation in sCJD. In any case, our observation strongly suggests that the phenomenon of mixed phenotypes in sCJD goes beyond PrPSc types 1 and 2 coexistence and also involves subtypes which shares the same PrPSc type. This, in turn, further underlines the importance of combining histopathological assessment and biochemical PrPSc typing for sCJD subtype characterization.

The present data also show that the association of two PrP27-30 fragments, which does not represent a bona-fide type 1 and 2 concurrence, may also be a feature of some sCJD cases. Thus, the PrP27-30 profile in VV2 cases in the cerebellum, thalamus and midbrain is sometime characterized by a doublet comprising a 18.5 kDa in addition to the typical 19 kDa band, while the western blot profile of PrP27-30 in the MV 2K cases appears almost invariably characterized by the association of two PrPSc core fragments including a classic 19 kDa type 2 band and a slower migrating band of about 20 kDa. Although these profiles truly represent concurrent PrPSc fragments, and the 20 and 18.5 kDa fragments likely reflect specific PK cleavage sites, the 20 and 18.5 kDa bands are distinguished from the type 1 and type 2 fragments because, at least to date, they were never detected independently from types 1 and 2, and are not markers of specific clinico-pathological phenotypes. Knowledge of these regional variations is nonetheless important to avoid misinterpreting a PrPSc profile as novel when only one brain region is analyzed [21].

Finally, the results obtained from the analyses of lesion profiles and clinical features in the subgroups of sCJD cases with mixed features deserve further comment. By showing that the relative ‘‘load’’ of each of the two PrPSc types significantly correlates with disease duration, the relative frequency of certain symptoms, and the ratio between cortical and cerebellar pathology, our study provides further strong evidence for the PrPSc type being a major biological determinant in human prion disease. In conclusion, the present data add to our knowledge of the prevalence and phenotypic spectrum of the sCJD variants with mixed molecular and pathological features, provide an updated molecular classification of the disease subtypes and will serve for future epidemiologic and transmission studies aimed at disclosing the etiology and extent of strain variation in sCJD.

Acknowledgments We wish to thank Barbara Polischi and Sabrina Boninsegna for her technical assistance. We also thank all the physicians who provided clinical data and helped in the collection of tissues and all family members who consented to the use of tissue for research. This study was funded in the frame of the bilateral Italy (ISS)–USA (NIH, Office for Rare Diseases) agreement on joint research on rare diseases, by the European Commission (FOOD-CT- 2004-506579), the Italian Ministry of University, Research and Technology (FIRB-2003-RBNE03FMCJ_006), the Federal Ministry of Health (ZV2-1369-340): grant PHS P30 AG010133, and the Gino Galletti Foundation.


Keywords Prion protein
Brain mapping
Molecular typing
Neurodegeneration
Classification


P. Parchi
R. Strammiello
S. Notari
S. Capellari Dipartimento di Scienze Neurologiche, Universita` di Bologna, Bologna, Italy A. Giese
H. Kretzschmar Institut fu¨r Neuropathologie, Ludwig-Maximilians-Universita¨t Mu¨nchen, Munich, Germany J. P. M. Langeveld Central Veterinary Institute of Wageningen UR, Lelystad, The Netherlands A. Ladogana
M. Pocchiari Department of Cell Biology and Neurosciences, Istituto Superiore di Sanita`, Rome, Italy I. Zerr Department of Neurology, National Reference Center for TSE Surveillance, Georg-August University, Go¨ttingen, Germany F. Roncaroli Division of Neuroscience and Mental Health, Department of Clinical Neuroscience, Imperial College, London, UK P. Cras Born-Bunge Institute (BBI), University of Antwerp (UA), Antwerp, Belgium B. Ghetti Department of Pathology, Indiana University, Indianapolis, IN, USA P. Parchi (&) Department of Neurological Sciences, Universtity of Bologna, Via Foscolo 7, 40123 Bologna, Italy e-mail: piero.parchi@unibo.it


http://www.springerlink.com/content/21552482u6761291/fulltext.pdf




MANY, MANY THANKS TO Parchi et al for this study, AND for the public access to full text. ...TSS



Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P
.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

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COMMENT

snip...see full text ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

August 10, 2009

Greetings,

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.

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.

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. ...

please see history, and the ever evolving TSE science to date ;

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



WHO WILL WATCH THE CHILDREN for CJD over the next 5 decades ?

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/



Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



Sunday, September 6, 2009

MAD COW USA 1997 SECRET VIDEO

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html



U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN see video

http://maddeer.org/video/embedded/prusinerclip.html



CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008

PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS

BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START

http://madcowfeed.blogspot.com/2008/06/texas-firm-recalls-cattle-heads-that.html



2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html




Sent: Tuesday, November 03, 2009 9:07 PM

Subject: [BSE-L] re-FOIA REQUEST ON FEED RECALL PRODUCT contaminated with prohibited material Recall # V-258-2009 and Recall # V-256-2009


http://madcowfeed.blogspot.com/2009/11/re-foia-request-on-feed-recall-product.html





TSS

SEVENTEENTH ANNUAL REPORT 2008 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK

SEVENTEENTH ANNUAL REPORT 2008 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK


The National CJD Surveillance Unit Western General Hospital, Edinburgh, EH4 2XU www.cjd.ed.ac.uk Infectious Disease Epidemiology Unit London School of Hygiene and Tropical Medicine Keppel Street, London, WC1E 7HT



SUMMARY

The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology of human transmissible spongiform encephalopathies (TSEs). In September 2001 the National Care Team was formed, which currently comprises two care coordinators and a secretary. It is based within the NCJDSU and was formed in response to concerns regarding the care of CJD patients. The information provided in this Seventeenth Annual Report continues to indicate that the number of sporadic cases remains relatively stable (the data for 2008 may still be incomplete). Detailed clinical and epidemiological information has been obtained for the great majority of patients. Referrals, having been fewer between 2004 and 2007, increased in 2008, back towards pre-2004 levels. 2008 has seen the highest mortality rate from sporadic CJD in the UK (1.43 per million per year) since 1985; a rate which is comparable with other European countries. Although the post mortem rate for patients with suspected CJD has declined, in line with general autopsy rates in the UK, it remains high (around 60%). The number of brain specimens examined for sporadic CJD in the neuropathology laboratory rose from 23 in 2007 to 28 in 2008 (32 in 2006). In 1990-2008 average annual mortality rates from sporadic CJD in England, Wales, Scotland and Northern Ireland were, respectively, 0.94, 1.08, 0.96 and 0.58/million/year. The differences between these rates are not statistically significant (p=0.4). The mortality rates from sporadic CJD in the UK are comparable to those observed in most other European countries and elsewhere in the world, including countries that are free of BSE. The highest and lowest mortality rates from sporadic CJD were observed in the South West (SMR=122) and Northern Ireland (SMR=74) respectively. The variation in the observed mortality rates between the different regions within the UK is not statistically significant (p>0.1). Up to 31 December 2008, there were 164 deaths from definite or probable variant CJD (vCJD) in the UK. Of these, 115 were confirmed by neuropathology. A further 3 probable cases were alive on 31st December 2008. The clinical, neuropathological and epidemiological features of the cases of vCJD are remarkably uniform and consistent with previous descriptions. Risk factors for the development of vCJD include age, residence in the UK and methionine homozygosity at codon 129 of the prion protein gene - all 147 clinically affected cases of vCJD with available genetic analysis have been methionine homozygotes. In 2008 the NCJDSU was referred the first case who met the clinical criteria in life for possible vCJD and was heterozygous (methionine/valine) at codon 129 of the PRNP gene (no postmortem was undertaken). The clinical picture was typical of vCJD seen to date, which is reassuring for surveillance purposes. For further information on this case, see Section 2.3, page 14. Although a single case with only a ‘possible’ classification, this may have implications for the presentation of further clinical cases in codon 129 heterozygotes in the future and for the estimation of prevalence of sub-clinical infection in the population. Section 1 T Seventeenth Annual Report 2008 4 Analysis of vCJD diagnoses and deaths from January 1994 to December 2008 indicates that a peak has passed. While this is an encouraging finding, the incidence of vCJD may increase again, particularly if different genetic subgroups with longer incubation periods exist. The identification of an individual of the PRNP-129 MV genotype as a possible case of vCJD and, in a separate case, disease-related prion protein in the spleen of a clinically unaffected blood recipient (reported in 2004) is consistent with such a hypothesis. These cases, along with the report of the prevalence of abnormal prion protein in the large study of appendix and tonsil tissues, suggests the possibility of a greater number of prec inical or subclinical cases in the population than might be indicated by the present numbers of confirmed clinical cases. The incidence of vCJD is higher in the north of Britain than in the south and the only statistically significant geographic cluster of vCJD cases in the UK remains that seen in Leicestershire (5 cases occurring between 1996 and 1999. The NCJDSU continues to collaborate with the Health Protection Agency Centre for Infections and Health Protection Scotland, in relation to a range of activities, including testing of pathological specimens from the National Anonymous Tonsil Archive study through to input into the development and implementation of public health policy, for example, in relation to the follow up of those identified as at increased risk of CJD. This year, the neuropathology laboratory identified a UK adult haemophiliac patient with PrPres in a restricted distribution in the spleen. This patient had been entered into a joint study with the UK Haemophilia Centre Doctors’ Organisation and NCJDSU. The patient had no neurological signs or symptoms, and no neuropathological evidence of vCJD. This case raises the possibility of transmission of vCJD infectivity via plasma products, and is the subject of ongoing investigations. A case of protease-sensitive prionopathy was identified on neuropathological and biochemical grounds, the first case of this disorder identified in the UK since its description by Gambetti et al in the USA in 2008. The activities of the NCJDSU are strengthened by collaboration with other surveillance projects, including the Transfusion Medicine Epidemiology Review and the study of Progressive Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in these projects is greatly appreciated; the effectiveness of this collaboration allowed the identification in 2003 of a case of vCJD associated with blood transfusion and the identification in 2004 of disease-related PrP in the spleen of a recipient of blood donated by someone incubating vCJD. In 2008, for the first time, the Unit prepared a Scientific Report, which is available on the Unit’s website (www.cjd.ed.ac.uk). The aim of the Scientific Report is to inform interested parties of details of the current and planned scientific research being undertaken by staff at the NCJDSU, in the context of the Unit’s previous research and its on-going core background surveillance. The Scientific Report complements the Annual Report, which provides a description of the clinicopathological epidemiology of CJD in the previous 12 months, reflecting the Unit’s core surveillance work. The NCJDSU Business Plan provides financial, structural and organisational information. The success of the National CJD Surveillance Unit continues to depend on the extraordinary level of cooperation from the neurology and neuropathology communities and other medical and paramedical staff throughout the UK. Ongoing support is provided by the Infectious Disease Epidemiology Unit, London School of Hygiene and Tropical Medicine. We are also particularly grateful to the relatives of patients for their collaboration.

see full text ;


http://www.cjd.ed.ac.uk/report17.pdf



Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P
.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

snip...

COMMENT

snip...see full text ;


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

August 10, 2009

Greetings,

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.

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.

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. ...

please see history, and the ever evolving TSE science to date ;

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009


http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



WHO WILL WATCH THE CHILDREN for CJD over the next 5 decades ?

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE


http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/



Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009


http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



Sunday, September 6, 2009

MAD COW USA 1997 SECRET VIDEO


http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html



U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom


http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN see video


http://maddeer.org/video/embedded/prusinerclip.html



CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008

PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS

BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START


http://madcowfeed.blogspot.com/2008/06/texas-firm-recalls-cattle-heads-that.html



2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006


http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html



TSS