SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008
ITEM 2 – APPROVAL OF MINUTES FROM SEAC 99 (SEAC 100/1) 6. The minutes of SEAC 99 were agreed as a correct record with the following amendment: 4 © SEAC 2008 • Paragraph 7, second bullet point, change “…Institute of Animal Health (IAH).” to “…Roslin Institute (RI).” 7. The committee was updated about transmission studies using isolates from a case of Creutzfeldt-Jakob Disease (CJD)1 which had been discussed at SEAC 99. Two lines of transgenic mice expressing the human prion protein gene homozygous for methionine (MM) or valine (VV) at codon 129 and two lines of conventional mice had been inoculated with isolates from the case. Transmission has been more efficient to the humanised mice compared with the conventional mice. The molecular characteristics of the abnormal prion protein (PrPSc) altered on transmission suggesting the patient may have been infected with an unstable TSE strain. Further work was required to characterise the TSE strain. However, it would be difficult to confirm whether this was related to BSE infection unless additional similar cases arose which could be investigated. 8.
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35. Members asked about the spread of chronic wasting disease (CWD) in the USA. Mr Burke replied that CWD was continuing to spread in the cervid population in the USA.
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One sheep in Cyprus, two in the UK and four from France are under investigation for possible BSE infection. Two further BSE cases were identified with unusual transmission properties in a Defra research project (SE1849) and 5 Countries where cases of BSE in cattle were reported in 2007: UK, Austria, Canada, Czech Republic, France, Germany, Hungary, Ireland, Italy, Japan, Poland, Portugal, Slovenia, Slovakia, Spain and The Netherlands. 6 Countries where atypical scrapie in sheep has been reported since 2002: Belgium, Denmark, the Falkland Islands, Finland, France, Germany, Greece, Hungary, Ireland, Italy, The Netherlands, Norway, Portugal, Slovenia, Spain, Sweden, UK and USA. 13 © SEAC 2008 are also subject to further characterisation. BSE has been confirmed in one French goat slaughtered in 2002. A UK goat which was originally diagnosed as a case of scrapie in 1990 is under investigation for possible BSE infection. 37.
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40. Members agreed that the statement should not focus entirely on the risk from BSE. A paragraph should be included to describe the risk that a new zoonotic TSE strain might emerge as a result of the relaxations to TSE controls. The possibility that the transmissibility to humans of such a new strain could be much greater than that of BSE should be acknowledged.
http://www.seac.gov.uk/minutes/final100.pdf
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007
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ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.
A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.
There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.
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http://www.seac.gov.uk/minutes/99.pdf
>>There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. <<<
10 people killed by new CJD-like disease
Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]
Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist
10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.
No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.
Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
snip... see full text ;
http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
Communicated by: Terry S. Singeltary Sr. <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:flounder9@verizon.net>
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Rule out of variant CJD as the cause of death of a Virginia resident
Recently, the National Prion Disease Pathology Surveillance Center (NPDPSC) ruled out the presence of variant CJD (vCJD) as the cause of death of a young Virginia woman who died earlier this year. Although this suspected case received international media attention, NPDPSC determined that the cause of death was not due to vCJD.
As of June 2008, the total number of vCJD cases identified in residents of the United States is three; all of which were epidemiologically linked to likely exposures to cattle products contaminated with bovine spongiform encephalopathy (BSE, commonly known as "mad cow disease")while residing in the United Kingdom (2 cases) or Saudi Arabia (1 case).
The NPDPSC was established by the Centers for Disease Control and Prevention in collaboration with the American Association of Neuropathologists for the purpose of enhancing national surveillance of human prion diseases such as classic CJD and vCJD. US physicians are encouraged to utilize the diagnostic services of the NPDPSC to confirm all clinically suspected and diagnosed cases of CJD and vCJD. For additional information about the NPDPSC and how to submit diagnostic specimens, consult www.CJDSurveillance.com.
http://www.cdc.gov/ncidod/dvrd/vcjd/other/News_06122008.htm
DOES anyone know if a final diagnosis was ever made ???
IT would seem that IF it was important enough for the USDA et al to first announce this at a news conference about beef trade with Korea i.e. not nvCJD, they could at least announce whether or not it was some form of sporadic CJD or any other TSE. IF they cannot because of the infamous 'confidentiality' claus, then that should have applied to announcing the negative on the nvCJD announcement as well, ya think ???
please see full text ;
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html
Wednesday, July 9, 2008
[Docket No. FDA-2008-N-0369] Ruminant Feed Ban Support Project; ``Response to RFA-FDA-08-008''
http://madcowfeed.blogspot.com/2008/07/docket-no-fda-2008-n-0369-ruminant-feed.html
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.
In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf
EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA)
Summary of the Scientific Report
The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.
The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.
A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90's when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.
EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.
http://www.efsa.europa.eu/en/science/tse_assessments/gbr_assessments/573.html
http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/573.Par.0004.File.dat/sr03_biohaz02_usa_report_v2_en1.pdf
Tuesday, June 3, 2008
SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
Tuesday, June 3, 2008
SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
http://nor-98.blogspot.com/
In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.
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see full report here ;
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/yearly_report.pps
Thursday, April 24, 2008
RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]
http://foiamadsheepmadrivervalley.blogspot.com/2008/04/re-foia-of-declaration-of-extraordinary.html
Chronic Wasting Disease
8. Human susceptibility to CWD
Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle [2]. Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades [51]. Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,
8
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al. [68] showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. [96], although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains [79]. To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days [41]. In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk [84]. However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indi rect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission [49]. Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans [72], and therefore may not indicate that human prion conversion would occur by CWD.
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11. Disease control challenges posed by CWD
Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control [91]. Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease [50]. An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection [55]. Modelling studies have provided further
10
support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions [75]. Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.
12. Conclusion
CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.
Acknowledgements
I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.
snip...see full text 19 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v08092.pdf
http://chronic-wasting-disease.blogspot.com/
Wednesday, June 18, 2008 CHRONIC WASTING DISEASE FOUND IN 24 MORE DEER IN ALBERTA
http://chronic-wasting-disease.blogspot.com/2008/06/chronic-wasting-disease-found-in-24.html
Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008
1: Vet Res. 2008 Apr 3;39(4):41
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
PLEASE NOTE THE PARTIAL AND VOLUNTARY MAD COW FEED BAN OF AUGUST 4, 1997 nothing more than ink on paper ... TSS
Wednesday, April 23, 2008
FDA Strengthens Safeguards for Consumers of Beef Issues Regulation on Animal Feeds with Added Safeguards Against BSE
http://madcowfeed.blogspot.com/
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html
----- Original Message -----
From: "Terry S. Singeltary Sr." mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:flounder9@verizon.net To: "Bovine Spongiform Encephalopathy" mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:BSE-L@aegee.org Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:heggem.daniel@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:sibert.christopher@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:denne.jane@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:hazen.susan@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:mcrosby@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:erobinson@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:enegin@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:cjdvoice@yahoogroups.com; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:madcow@lists.iatp.org
Sent: Monday, April 28, 2008 9:48 PM
Subject: Interference at the EPA Science and Politics at the U.S. Environmental Protection Agency
Reports and Research
Interference at the EPA
Science and Politics at the U.S. Environmental Protection Agency
The U.S. Environmental Protection Agency (EPA) has the simple yet profound charge "to protect human health and the environment." EPA scientists apply their expertise to protect the public from air and water pollution, clean up hazardous waste, and study emerging threats such as global warming. Because each year brings new and potentially toxic chemicals into our homes and workplaces, because air pollution still threatens our public health, and because environmental challenges are becoming more complex and global, a strong and capable EPA is more important than ever.
Yet challenges from industry lobbyists and some political leaders to the agency's decisions have too often led to the suppression and distortion of the scientific findings underlying those decisions—to the detriment of both science and the health of our nation. While every regulatory agency must balance scientific findings with other considerations, policy makers need access to the highest-quality scientific information to make fully informed decisions.
Concern over this problem led the Union of Concerned Scientists (UCS) to investigate political interference in science at the EPA. The investigation combines dozens of interviews with current and former EPA staff, analysis of government documents, more than 1,600 responses to a survey sent to current EPA scientists, and written comments from EPA scientists.
The results of these investigations show an agency under siege from political pressures. On numerous issues—ranging from mercury pollution to groundwater contamination to climate change—political appointees have edited scientific documents, manipulated scientific assessments, and generally sought to undermine the science behind dozens of EPA regulations. ...
snip...please see full text ;
http://sciencebushwhacked.blogspot.com/
Wednesday, April 30, 2008
Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings
http://cjdmadcowbaseoct2007.blogspot.com/2008/04/consumption-of-beef-tongue-human-bse.html
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
http://cjdmadcowbaseoct2007.blogspot.com/2008/04/srm-mad-cow-recall-406-thousand-pounds.html
2006 was a banner year too for mad cow protein. those were just one of many ;
Specified Risk Materials
http://madcowspontaneousnot.blogspot.com/2008/02/specified-risk-materials-srm.html
Thu Dec 6, 2007 11:38
FDA IN CRISIS MODE, AMERICAN LIVES AT RISK
http://www.cidrap.umn.edu/cidrap/content/fs/food-disease/news/dec0407fda.html
FDA SCIENCE AND MISSION AT RISK
http://www.fda.gov/ohrms/dockets/ac/07/briefing/2007-4329b_02_01_FDA%20Report%20on%20Science%20and%20Technology.pdf
THE only fool is one who fools himself, and GW and his administration and their junk science will fool humans for just so long, then the incubation will catch up. none of this was about science, it was all about commodities and futures and the exporting of beef. nothing else mattered, literally, just ask old stanley prusiner the nobel prize winner for the PRION, what did old stan say ;
STANLEY PRUSINER NOBEL PEACE PRIZE WINNER ON THE PRION
US AG SEC AND LAYCRAFT
“nothing matters, except beef from Canada under 30 months bone in beef product, that’s ALL THAT MATTERS!”
US SENATOR AND STAN THE MAN SLAM USDA ”DAMNING TESTIMONY”
Senator Michael Machado from California
”USDA does not know what’s going on”. ”USDA is protecting the industry”. ” SHOULD the state of California step in”
Stanley Prusiner
”nobody has ever ask us to comment”
”they don’t want us to comment”
”they never ask”
i tried to see Venemon, after Canadian cow was discovered with BSE. went to see lyle. after talking with him…
absolute ignorance…
then thought i should see Venemon…
it was clear his entire policy was to get cattle boneless beef prods across the border…
nothing else mattered…
his aids confirmed this…
5 times i tried to see Venemon, never worked…
eventually met with carl rove the political…
he is the one that arranged meeting with Venemon…
just trying to give you a sense of the distance…
threat to health public safety…
was never contacted…
yes i believe that prions are bad to eat and you can die from them…END
Dr. Stan bashing Ann Veneman - 3 minutes - Damning testimony
http://maddeer.org/video/embedded/08snip.ram
File Name: USDA DON'T ASK DON'T TELL POLICY 02snip.rpm
DAMNING testimony of consumer consumption of Washington mad cow in California
http://www.maddeer.org/video/embedded/02snip.rm
In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.
In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125
Audit Report
Animal and Plant Health Inspection Service
Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II
and
Food Safety and Inspection Service
Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
Report No. 50601-10-KC January 2006
Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain
Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.
USDA/OIG-A/50601-10-KC Page 27
observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.
snip...
http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
2 January 2000
British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.
http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Saturday, March 22, 2008
10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures
http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html
re-Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease
http://betaamyloidcjd.blogspot.com/2008/04/re-association-between-deposition-of.html
full text ;
Friday, June 20, 2008
USDA TO KOREA AND THE WORLD, EAT THAT AND LIKE IT
http://usdavskorea.blogspot.com/2008/06/usda-to-korea-and-world-eat-that-and.html
Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0006 Public Submission Title Comment from Terry S Singletary Sr Views Add Comments How To Comment
snip...
MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???
go figure....
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&d=APHIS-2006-0041-0006
ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)
THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted blood from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone.
These are the facts as i have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. ...
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
snip... 48 pages...
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
PEACE
Terry S. Singeltary Sr. P.O. Box 42 Baycliff, Texas USA 77518
Sunday, July 13, 2008
Wednesday, June 4, 2008
SEAC 2008 ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
SEAC 2008
ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
The Spongiform Encephalopathy Advisory Committee held its 100th meeting in London on 25th April 2008, and discussed the following:
CURRENT ISSUES
SEAC was informed about:
• Two recently identified cases of variant Creutzfeldt-Jakob Disease (vCJD) in Spain. • Proposals for the future regulation of ‘high street’ dentistry outlined in a consultation issued recently by the Department of Health (DH)1. SEAC agreed to respond to the consultation, welcoming the proposals. • The detection of material of unknown animal origin in a batch of wheat feed distributed for use in livestock feed2.
ASSESSMENT OF THE PREVALENCE OF SUBCLINICAL vCJD SEAC was updated about the progress of the National Anonymous Tonsil Archive (NATA) and of discussions around a proposed post mortem tissue archive. These would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease). Approximately 55 000 NATA samples had been screened by the end of March 2008. Although none was positive for abnormal prion protein (PrPvCJD), some testing remains to be done on some samples. SEAC expressed disappointment that it is currently
1 DH (2008) The future regulation of health and adult social care in England: A consultation on the framework for the registration of health and adult social care providers.
http://www.dh.gov.uk/en/Consultations/Liveconsultations/DH_083625
2
http://www.food.gov.uk/news/newsarchive/2008/apr/feedcontam
2 © SEAC 2008
proving difficult to establish a post mortem tissue archive through collection of tissues from Coroners’ autopsies. In light of the current difficulties in establishing a post mortem tissue archive, DH asked for advice about how existing data from NATA might be combined with a completed survey of appendix samples3, which had found PrPvCJD in three out of about 11 000 samples. SEAC considered that the data from these studies are not, at the present time, discrepant. However, given the uncertainties about the tissue distribution of PrPvCJD during vCJD incubation, it would be hard to see how these data could be combined.
SEAC considered what further work might be done to obtain better estimates for the prevalence of subclinical vCJD including additional appendix studies and a post mortem tissue archive. SEAC strongly recommended that every avenue be pursued to establish such an archive.
The committee agreed to produce a statement.
UPDATE ON ANIMAL TSEs
SEAC was updated on transmissible spongiform encephalopathies (TSEs) in animals in the UK and elsewhere.
In the UK, the Bovine Spongiform Encephalopathy (BSE) epidemic in cattle peaked in 1992, with over 37 000 confirmed cases but has since declined with 67 cases confirmed in 2007. By the end of 2007, there had been 178 BSE cases confirmed in the UK in cattle born after the introduction of the reinforced feed ban in 1996. Two unusual (H-type) BSE cases have been found in the UK. Relatively low numbers of BSE cases have also been found in the other European Union (EU) countries and elsewhere.
No TSEs have been found in a relatively small EU survey of deer. There is no evidence for the presence of BSE from surveillance of UK sheep. Classical scrapie case numbers continue to decline in the UK with 31 cases confirmed in 2007. Small numbers of
3 Hilton et al. (2004) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol. 203, 733-739.
3 © SEAC 2008
atypical scrapie cases continue to be found with a total of 194 cases confirmed in the UK since surveillance for atypical scrapie began in 2002. Classical and atypical scrapie continue to be found in EU and other countries.
CONSIDERATION OF OPTIONS FOR RELAXATION OF THE TOTAL FEED BAN
At SEAC 99, Rural Affairs Departments and the Food Standards Agency (FSA) asked SEAC to assess the possible consequences of various options for relaxing the total feed ban. These included the introduction of tolerance levels for certain types of processed animal protein (PAP) in feed, the inclusion of fish meal in young ruminant diets and the feeding of non-ruminant PAP to nonruminants of a different species. Following that meeting, a draft statement was prepared based on the discussions.
SEAC discussed the draft statement and agreed modifications. Once the statement is finalised, it will be published on the SEAC website.
PROPOSALS TO REDUCE TESTING OF CATTLE SLAUGHTERED FOR FOOD – IMPACT ON RISK TO HUMAN HEALTH
FSA asked SEAC to consider an analysis of the human health risk of a range of options for altering the BSE surveillance programme by increasing the minimum age at which healthy slaughtered and fallen stock cattle must be tested for BSE. A BSE risk model constructed by the Veterinary Laboratories Agency, that had been previously reviewed and accepted by SEAC, was used for the analysis.
SEAC noted that the increased risks calculated by the model arising as a result of raising the age at which cattle are tested for BSE are very small. These calculations are subject to uncertainties, particularly in relation to assumptions made about infectivity in tissues and the BSE epidemic. The committee asked for further information about how well outputs from the model fit actual surveillance data. SEAC suggested that future modelling could examine the effect of changing controls on effectiveness of one control. SEAC noted that BSE testing of cattle provides
4 © SEAC 2008
important data on the incidence of the disease and confers some public health protection.
HORIZON SCANNING
SEAC considered issues that might emerge on the TSE science horizon. It noted that significant progress continues to be made to understand TSEs better and to develop effective TSE control policies. SEAC considered areas of TSE science important for the future could include the:
• nature of the TSE carrier state • genetic factors that modulate susceptibility to TSEs • molecular basis of TSE strains and the relationship with host genetics • potential for animal TSEs to transmit to humans • development of ante mortem diagnostic tests • proportionality of TSE controls
http://www.seac.gov.uk/summaries/seac100_summary.pdf
The finalised minutes of the 99th meeting
http://www.seac.gov.uk/minutes/99.pdf
Thursday, January 31, 2008
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION
40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base
13 © SEAC 2007
cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”
41. A member considered that this question ............
http://www.seac.gov.uk/minutes/99.pdf
snip... please see full text, sources, and comments here ;
http://seac992007.blogspot.com/2008/01/spongiform-encephalopathy-advisory.html
FC5.3 Assessing the Risk of vCJD Transmission by Dentistry; Distribution of Infectivity in Oral Tissues of VM Mice after Simulated Oral Feeding of BSE-301V
Sutton, JM1; Kirby, E1; Dickinson, J1; Dennis, M1; Cornwall, M1; Vassey, MJ1; Smith, A2; Marsh, PD3; Walker, JT1; Raven, NDH1 1Health Protection Agency, Centre for Emergency Preparedness and Response,, TSE Research group, UK; 2University of Glasgow, Dental School, UK; 3Health Protection Agency, Centre for Emergency Preparedness and Response,, UK
Background: Ongoing concerns about the prevalence of variant Creutzfeldt Jakob Disease (vCJD) in the UK population has heightened concerns about the risks of iatrogenic transmission of the disease. Although there have been no cases to date of transmission by surgery there have been 4 cases involving blood transfusion. This study aims to assess the potential of transmission of the disease by dental procedures. Whilst the risks are undoubtably low the very large numbers of procedures carried out annually have the potential to amplify the risks considerably and there is very little data in this area to form the basis for accurate risk assessments. Aim(s)/Objective(s): To assess the relative levels of infectivity in oral tissues from a murine model following exposure to BSE-301V through the small intestine. Methods. The study uses a BSE-301V, VM mouse model as a clinically relevant model for assessing iatrogenic vCJD transmission between humans. Infectious mouse brain homogenate was prepared and inoculated into a loop of the duodenum, to prevent direct contamination of the oral tissues. Mice were sacrificed at 3-weekly intervals and at appearance of clinical symptoms. A range of oral tissues, including dental pulp, gingival margin, salivary gland, saliva, lingual tonsil and trigeminal ganglia, together with brain and spleen tissues were removed, processed as homogenates and reinoculated intracranially (ic.) into indicator mice. Results: The primary challenge proved to be a very efficient route of infection with a 100% attack rate and a mean incubation to clinical disease of 157 ± 17 days (compared to 120 days for the same titre inoculum ic.). Infectivity was observed in all oral and control tissues with varying time-courses and titres estimated from incubation period. Discussion: The results throw new light on the potential routes of dissemination and spread of infectivity from the small intestine to the oral cavity and its implications for possible iatrogenic transmission of vCJD via dental, endoscopic or other forms of surgery. Conclusion: The data generated from the study provides support for ongoing risk assessments to look at the potential for vCJD transmission via dental procedures alongside other elements of studies looking at effectiveness of decontamination and re-use of dental instruments.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Monday, December 31, 2007
Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html
Subject: CJD: update for dental staff Date: November 12, 2006 at 3:25 pm PST
1: Dent Update. 2006 Oct;33(8):454-6, 458-60.
CJD: update for dental staff.
Scully C, Smith AJ, Bagg J. Eastman Dental Institute, University of London.
It is almost a decade since the recognition of the emergence of a new infectious disease termed variant Creutzfeldt-Jakob disease (vCJD) caused by prions (PrPTSE), abnormal variants of a normal human cell surface protein (PrP).This disease has a number of similarities to other forms of CJD--lethal disorders characterized by a prolonged incubation period, and progressive mental deterioration. In relation to oral tissues, PrPTSE have been found in neural, gingival, pulpal, lingual, lymphoreticular and salivary gland tissue in animal models. In both sporadic and variant CJD, PrPTSE is detectable in the trigeminal ganglion and, in vCJD, in lymphoreticular tissues, but infectivity has not been tested in other human oral tissues. CLINICAL RELEVANCE: PrPTSE is much more resistant to the common methods of inactivation than conventional pathogens, and it adheres avidly to steel whilst retaining its infectivity. Particular attention must be paid to cleaning and sterilizing re-usable dental instruments. Single-use devices, such as endodontic files and matrix bands, must never be re-used. Advice on the reprocessing of dental instruments used on known CJD patients must be obtained from local infection control teams. Research into effective methods of prion inactivation appears promising, although further work on the applicability to general dental practice is required.
PMID: 17087448 [PubMed - in process]
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17087448&query_hl=1&itool=pubmed_docsum
Subject: PrPSc in salivary glands of scrapie-affected sheep Date: February 15, 2007 at 9:33 am PST
J. Virol. doi:10.1128/JVI.02148-06 Copyright (c) 2007, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.
PrPSc in salivary glands of scrapie-affected sheep
Marta Vascellari*, Romolo Nonno, Franco Mutinelli, Michela Bigolaro, Michele Angelo Di Bari, Erica Melchiotti, Stefano Marcon, Claudia D'Agostino, Gabriele Vaccari, Michela Conte, Luigi De Grossi, Francesca Rosone, Francesco Giordani, and Umberto Agrimi Istituto Zooprofilattico Sperimentale delle Venezie, Histopathology Department, Viale dell'Università 10, 35020 Legnaro (PD), Italy; Istituto Superiore di Sanità, Department of Food Safety and Animal Health, Viale Regina Elena 299, 00161 Roma, Italy; Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Strada Terme, 01100 Viterbo, Italy
* To whom correspondence should be addressed. Email: mvascellari@izsvenezie.it .
Abstract
The salivary glands of scrapie-affected sheep and healthy controls were investigated for the presence of the pathological prion protein (PrPSc). PrPSc was detected in major (parotid and mandibular) and minor (buccal, labial and palatine) salivary glands of naturally and experimentally infected sheep. By western blot, PrPSc concentration in glands was estimated as 0.02-0.005% of brain. Immunohistochemistry revealed intracellular deposition of PrPSc in ductal and acinar epithelium and occasional labeling into the lumen of salivary ducts. The presence of PrPSc in salivary glands highlights the possible role of saliva in the horizontal transmission of scrapie.
http://jvi.asm.org/cgi/content/abstract/JVI.02148-06v1?papetoc
CJD TEXAS
http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html
This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle
*** and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
The Italian cases (11 and 15 years of age) originally named bovine amyloidotic spongiform encephalopathy (BASE) were characterized by an unglycosylated protein band with a lower molecular mass (thus named L cases) and the predominance of the monoglycosylated band. In addition, immunohistochemical detection of PrPres in these cases found greater deposits in the cerebral cortex and thalamus versus the brain stem. The French cases found a higher molecular mass associated with the unglycosylated protein band and were called H cases (see figure 1). *** The different "strains" are now called atypical BSE. ...
full text, skroll down to page 6 ;
http://www.usaha.org/committees/reports/2006/report-fe-2006.pdf
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE (the last two cases of mad cow disease in the USA were in Alabama, and Texas, both of which were atypical BSE).
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
WHY were they planning to destroy all CJD tissue samples donated ???
Washington Times - Washington,DC,USA NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent
Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.
Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.
Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.
However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.
"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE). ...snip...end...tss
http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm
NIH says it will preserve CJD brains By STEVE MITCHELL
WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.
snip...
May 10, 2005
The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199
Dear Senator Cornyn:
Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.
I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) ...snip...end...tss
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html
NIH says it will preserve CJD brains
Published: May 31, 2005 at 5:26 PM
By STEVE MITCHELL WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.
An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.
That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.
"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.
Cornyn had inquired about the status of the collection in April.
Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.
Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.
Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.
"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.
The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.
CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.
Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.
Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.
"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.
"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.
"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.
"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.
Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.
Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.
--
Steve Mitchell is UPI's Medical Correspondent. E-mail: sciencemail@upi.com
© 2005 United Press International. All Rights Reserved. This material may not be reproduced, redistributed, or manipulated in any form.
http://www.upi.com/NewsTrack/Science/2005/05/31/nih_says_it_will_preserve_cjd_brains/6771/print_view/
In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.
In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
snip...
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
http://nor-98.blogspot.com/
Tuesday, June 3, 2008
SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008
1: Vet Res. 2008 Apr 3;39(4):41
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Journal of American Medical Association
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
http://jama.ama-assn.org/
2 January 2000 British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey b y intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
www.cjdsupport.net
Newsletter issue 17 april 2008
the importance of gene types
We have known for many years that a common variation in a gene, known as the prion protein gene, is very important in determining the risk of developing prion diseases and how long it takes for the disease to develop when someone becomes infected. There are three genetic types in the UK population known as MM, VV and MV. So far, vCJD has only affected people with MM genetic type. Around 40% of healthy people in the UK are MM, about 50% are MV and around 10% are VV. It is likely that BSE prions will infect people of the VV and MV types also, but they may have much longer incubation periods (the time taken from being infected with prions until the brain disease becomes apparent) and may also develop a pattern of disease which may be different to vCJD. We suspect this again as result of research in laboratory mice wher e those that had the VV and MV genes had a different type of disease and different types or ‘strains’ of prions developed.
snip...
This unusual finding reminds us of the importance of keeping alert to the possibility that BSE prions will cause disease in individuals with different genetic types, who may develop a disease that may resemble sporadic CJD, or vCJD, or have a new pattern of disease.
Prion disease and gene types
Dr Simon Mead and Professor John Collinge, NHS National Prion Clinic
Reference: Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman Simon Mead; Susan Joiner; Melanie Desbruslais; Jonathan A. Beck; Michael O’ Donoghue; Peter Lantos; Jonathan D. F. Wadsworth; John Collinge Arch Neurol. 2007;64(12):1780-1784.
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
The Spongiform Encephalopathy Advisory Committee held its 100th meeting in London on 25th April 2008, and discussed the following:
CURRENT ISSUES
SEAC was informed about:
• Two recently identified cases of variant Creutzfeldt-Jakob Disease (vCJD) in Spain. • Proposals for the future regulation of ‘high street’ dentistry outlined in a consultation issued recently by the Department of Health (DH)1. SEAC agreed to respond to the consultation, welcoming the proposals. • The detection of material of unknown animal origin in a batch of wheat feed distributed for use in livestock feed2.
ASSESSMENT OF THE PREVALENCE OF SUBCLINICAL vCJD SEAC was updated about the progress of the National Anonymous Tonsil Archive (NATA) and of discussions around a proposed post mortem tissue archive. These would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease). Approximately 55 000 NATA samples had been screened by the end of March 2008. Although none was positive for abnormal prion protein (PrPvCJD), some testing remains to be done on some samples. SEAC expressed disappointment that it is currently
1 DH (2008) The future regulation of health and adult social care in England: A consultation on the framework for the registration of health and adult social care providers.
http://www.dh.gov.uk/en/Consultations/Liveconsultations/DH_083625
2
http://www.food.gov.uk/news/newsarchive/2008/apr/feedcontam
2 © SEAC 2008
proving difficult to establish a post mortem tissue archive through collection of tissues from Coroners’ autopsies. In light of the current difficulties in establishing a post mortem tissue archive, DH asked for advice about how existing data from NATA might be combined with a completed survey of appendix samples3, which had found PrPvCJD in three out of about 11 000 samples. SEAC considered that the data from these studies are not, at the present time, discrepant. However, given the uncertainties about the tissue distribution of PrPvCJD during vCJD incubation, it would be hard to see how these data could be combined.
SEAC considered what further work might be done to obtain better estimates for the prevalence of subclinical vCJD including additional appendix studies and a post mortem tissue archive. SEAC strongly recommended that every avenue be pursued to establish such an archive.
The committee agreed to produce a statement.
UPDATE ON ANIMAL TSEs
SEAC was updated on transmissible spongiform encephalopathies (TSEs) in animals in the UK and elsewhere.
In the UK, the Bovine Spongiform Encephalopathy (BSE) epidemic in cattle peaked in 1992, with over 37 000 confirmed cases but has since declined with 67 cases confirmed in 2007. By the end of 2007, there had been 178 BSE cases confirmed in the UK in cattle born after the introduction of the reinforced feed ban in 1996. Two unusual (H-type) BSE cases have been found in the UK. Relatively low numbers of BSE cases have also been found in the other European Union (EU) countries and elsewhere.
No TSEs have been found in a relatively small EU survey of deer. There is no evidence for the presence of BSE from surveillance of UK sheep. Classical scrapie case numbers continue to decline in the UK with 31 cases confirmed in 2007. Small numbers of
3 Hilton et al. (2004) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol. 203, 733-739.
3 © SEAC 2008
atypical scrapie cases continue to be found with a total of 194 cases confirmed in the UK since surveillance for atypical scrapie began in 2002. Classical and atypical scrapie continue to be found in EU and other countries.
CONSIDERATION OF OPTIONS FOR RELAXATION OF THE TOTAL FEED BAN
At SEAC 99, Rural Affairs Departments and the Food Standards Agency (FSA) asked SEAC to assess the possible consequences of various options for relaxing the total feed ban. These included the introduction of tolerance levels for certain types of processed animal protein (PAP) in feed, the inclusion of fish meal in young ruminant diets and the feeding of non-ruminant PAP to nonruminants of a different species. Following that meeting, a draft statement was prepared based on the discussions.
SEAC discussed the draft statement and agreed modifications. Once the statement is finalised, it will be published on the SEAC website.
PROPOSALS TO REDUCE TESTING OF CATTLE SLAUGHTERED FOR FOOD – IMPACT ON RISK TO HUMAN HEALTH
FSA asked SEAC to consider an analysis of the human health risk of a range of options for altering the BSE surveillance programme by increasing the minimum age at which healthy slaughtered and fallen stock cattle must be tested for BSE. A BSE risk model constructed by the Veterinary Laboratories Agency, that had been previously reviewed and accepted by SEAC, was used for the analysis.
SEAC noted that the increased risks calculated by the model arising as a result of raising the age at which cattle are tested for BSE are very small. These calculations are subject to uncertainties, particularly in relation to assumptions made about infectivity in tissues and the BSE epidemic. The committee asked for further information about how well outputs from the model fit actual surveillance data. SEAC suggested that future modelling could examine the effect of changing controls on effectiveness of one control. SEAC noted that BSE testing of cattle provides
4 © SEAC 2008
important data on the incidence of the disease and confers some public health protection.
HORIZON SCANNING
SEAC considered issues that might emerge on the TSE science horizon. It noted that significant progress continues to be made to understand TSEs better and to develop effective TSE control policies. SEAC considered areas of TSE science important for the future could include the:
• nature of the TSE carrier state • genetic factors that modulate susceptibility to TSEs • molecular basis of TSE strains and the relationship with host genetics • potential for animal TSEs to transmit to humans • development of ante mortem diagnostic tests • proportionality of TSE controls
http://www.seac.gov.uk/summaries/seac100_summary.pdf
The finalised minutes of the 99th meeting
http://www.seac.gov.uk/minutes/99.pdf
Thursday, January 31, 2008
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION
40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base
13 © SEAC 2007
cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”
41. A member considered that this question ............
http://www.seac.gov.uk/minutes/99.pdf
snip... please see full text, sources, and comments here ;
http://seac992007.blogspot.com/2008/01/spongiform-encephalopathy-advisory.html
FC5.3 Assessing the Risk of vCJD Transmission by Dentistry; Distribution of Infectivity in Oral Tissues of VM Mice after Simulated Oral Feeding of BSE-301V
Sutton, JM1; Kirby, E1; Dickinson, J1; Dennis, M1; Cornwall, M1; Vassey, MJ1; Smith, A2; Marsh, PD3; Walker, JT1; Raven, NDH1 1Health Protection Agency, Centre for Emergency Preparedness and Response,, TSE Research group, UK; 2University of Glasgow, Dental School, UK; 3Health Protection Agency, Centre for Emergency Preparedness and Response,, UK
Background: Ongoing concerns about the prevalence of variant Creutzfeldt Jakob Disease (vCJD) in the UK population has heightened concerns about the risks of iatrogenic transmission of the disease. Although there have been no cases to date of transmission by surgery there have been 4 cases involving blood transfusion. This study aims to assess the potential of transmission of the disease by dental procedures. Whilst the risks are undoubtably low the very large numbers of procedures carried out annually have the potential to amplify the risks considerably and there is very little data in this area to form the basis for accurate risk assessments. Aim(s)/Objective(s): To assess the relative levels of infectivity in oral tissues from a murine model following exposure to BSE-301V through the small intestine. Methods. The study uses a BSE-301V, VM mouse model as a clinically relevant model for assessing iatrogenic vCJD transmission between humans. Infectious mouse brain homogenate was prepared and inoculated into a loop of the duodenum, to prevent direct contamination of the oral tissues. Mice were sacrificed at 3-weekly intervals and at appearance of clinical symptoms. A range of oral tissues, including dental pulp, gingival margin, salivary gland, saliva, lingual tonsil and trigeminal ganglia, together with brain and spleen tissues were removed, processed as homogenates and reinoculated intracranially (ic.) into indicator mice. Results: The primary challenge proved to be a very efficient route of infection with a 100% attack rate and a mean incubation to clinical disease of 157 ± 17 days (compared to 120 days for the same titre inoculum ic.). Infectivity was observed in all oral and control tissues with varying time-courses and titres estimated from incubation period. Discussion: The results throw new light on the potential routes of dissemination and spread of infectivity from the small intestine to the oral cavity and its implications for possible iatrogenic transmission of vCJD via dental, endoscopic or other forms of surgery. Conclusion: The data generated from the study provides support for ongoing risk assessments to look at the potential for vCJD transmission via dental procedures alongside other elements of studies looking at effectiveness of decontamination and re-use of dental instruments.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Monday, December 31, 2007
Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html
Subject: CJD: update for dental staff Date: November 12, 2006 at 3:25 pm PST
1: Dent Update. 2006 Oct;33(8):454-6, 458-60.
CJD: update for dental staff.
Scully C, Smith AJ, Bagg J. Eastman Dental Institute, University of London.
It is almost a decade since the recognition of the emergence of a new infectious disease termed variant Creutzfeldt-Jakob disease (vCJD) caused by prions (PrPTSE), abnormal variants of a normal human cell surface protein (PrP).This disease has a number of similarities to other forms of CJD--lethal disorders characterized by a prolonged incubation period, and progressive mental deterioration. In relation to oral tissues, PrPTSE have been found in neural, gingival, pulpal, lingual, lymphoreticular and salivary gland tissue in animal models. In both sporadic and variant CJD, PrPTSE is detectable in the trigeminal ganglion and, in vCJD, in lymphoreticular tissues, but infectivity has not been tested in other human oral tissues. CLINICAL RELEVANCE: PrPTSE is much more resistant to the common methods of inactivation than conventional pathogens, and it adheres avidly to steel whilst retaining its infectivity. Particular attention must be paid to cleaning and sterilizing re-usable dental instruments. Single-use devices, such as endodontic files and matrix bands, must never be re-used. Advice on the reprocessing of dental instruments used on known CJD patients must be obtained from local infection control teams. Research into effective methods of prion inactivation appears promising, although further work on the applicability to general dental practice is required.
PMID: 17087448 [PubMed - in process]
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17087448&query_hl=1&itool=pubmed_docsum
Subject: PrPSc in salivary glands of scrapie-affected sheep Date: February 15, 2007 at 9:33 am PST
J. Virol. doi:10.1128/JVI.02148-06 Copyright (c) 2007, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.
PrPSc in salivary glands of scrapie-affected sheep
Marta Vascellari*, Romolo Nonno, Franco Mutinelli, Michela Bigolaro, Michele Angelo Di Bari, Erica Melchiotti, Stefano Marcon, Claudia D'Agostino, Gabriele Vaccari, Michela Conte, Luigi De Grossi, Francesca Rosone, Francesco Giordani, and Umberto Agrimi Istituto Zooprofilattico Sperimentale delle Venezie, Histopathology Department, Viale dell'Università 10, 35020 Legnaro (PD), Italy; Istituto Superiore di Sanità, Department of Food Safety and Animal Health, Viale Regina Elena 299, 00161 Roma, Italy; Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Strada Terme, 01100 Viterbo, Italy
* To whom correspondence should be addressed. Email: mvascellari@izsvenezie.it .
Abstract
The salivary glands of scrapie-affected sheep and healthy controls were investigated for the presence of the pathological prion protein (PrPSc). PrPSc was detected in major (parotid and mandibular) and minor (buccal, labial and palatine) salivary glands of naturally and experimentally infected sheep. By western blot, PrPSc concentration in glands was estimated as 0.02-0.005% of brain. Immunohistochemistry revealed intracellular deposition of PrPSc in ductal and acinar epithelium and occasional labeling into the lumen of salivary ducts. The presence of PrPSc in salivary glands highlights the possible role of saliva in the horizontal transmission of scrapie.
http://jvi.asm.org/cgi/content/abstract/JVI.02148-06v1?papetoc
CJD TEXAS
http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html
This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle
*** and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
The Italian cases (11 and 15 years of age) originally named bovine amyloidotic spongiform encephalopathy (BASE) were characterized by an unglycosylated protein band with a lower molecular mass (thus named L cases) and the predominance of the monoglycosylated band. In addition, immunohistochemical detection of PrPres in these cases found greater deposits in the cerebral cortex and thalamus versus the brain stem. The French cases found a higher molecular mass associated with the unglycosylated protein band and were called H cases (see figure 1). *** The different "strains" are now called atypical BSE. ...
full text, skroll down to page 6 ;
http://www.usaha.org/committees/reports/2006/report-fe-2006.pdf
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE (the last two cases of mad cow disease in the USA were in Alabama, and Texas, both of which were atypical BSE).
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
WHY were they planning to destroy all CJD tissue samples donated ???
Washington Times - Washington,DC,USA NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent
Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.
Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.
Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.
However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.
"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE). ...snip...end...tss
http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm
NIH says it will preserve CJD brains By STEVE MITCHELL
WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.
snip...
May 10, 2005
The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199
Dear Senator Cornyn:
Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.
I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) ...snip...end...tss
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html
NIH says it will preserve CJD brains
Published: May 31, 2005 at 5:26 PM
By STEVE MITCHELL WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.
An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.
That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.
"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.
Cornyn had inquired about the status of the collection in April.
Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.
Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.
Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.
"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.
The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.
CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.
Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.
Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.
"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.
"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.
"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.
"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.
Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.
Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.
--
Steve Mitchell is UPI's Medical Correspondent. E-mail: sciencemail@upi.com
© 2005 United Press International. All Rights Reserved. This material may not be reproduced, redistributed, or manipulated in any form.
http://www.upi.com/NewsTrack/Science/2005/05/31/nih_says_it_will_preserve_cjd_brains/6771/print_view/
In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.
In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
snip...
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
http://nor-98.blogspot.com/
Tuesday, June 3, 2008
SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008
1: Vet Res. 2008 Apr 3;39(4):41
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Journal of American Medical Association
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
http://jama.ama-assn.org/
2 January 2000 British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey b y intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
www.cjdsupport.net
Newsletter issue 17 april 2008
the importance of gene types
We have known for many years that a common variation in a gene, known as the prion protein gene, is very important in determining the risk of developing prion diseases and how long it takes for the disease to develop when someone becomes infected. There are three genetic types in the UK population known as MM, VV and MV. So far, vCJD has only affected people with MM genetic type. Around 40% of healthy people in the UK are MM, about 50% are MV and around 10% are VV. It is likely that BSE prions will infect people of the VV and MV types also, but they may have much longer incubation periods (the time taken from being infected with prions until the brain disease becomes apparent) and may also develop a pattern of disease which may be different to vCJD. We suspect this again as result of research in laboratory mice wher e those that had the VV and MV genes had a different type of disease and different types or ‘strains’ of prions developed.
snip...
This unusual finding reminds us of the importance of keeping alert to the possibility that BSE prions will cause disease in individuals with different genetic types, who may develop a disease that may resemble sporadic CJD, or vCJD, or have a new pattern of disease.
Prion disease and gene types
Dr Simon Mead and Professor John Collinge, NHS National Prion Clinic
Reference: Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman Simon Mead; Susan Joiner; Melanie Desbruslais; Jonathan A. Beck; Michael O’ Donoghue; Peter Lantos; Jonathan D. F. Wadsworth; John Collinge Arch Neurol. 2007;64(12):1780-1784.
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
Thursday, January 31, 2008
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
Draft minutes of the 99th meeting held on 14th December 2007
snip...
• A member explained that a confirmed case of atypical scrapie
had been recently identified in a research project at the
Institute of Animal Health (IAH). The animal had been born in
1997 in New Zealand, imported into the UK in 1998, spending
the first six months at the Arthur Rickwood Sheep Unit
(ARSU) before transfer to the IAH site at Compton. Thus, it is
possible it may have become infected either in New Zealand,
whilst at ARSU, where two other cases have now been
confirmed, or at Compton, perhaps as a result of the
experiments conducted during the research study involving
transfusion of blood between sheep. The case is under
further investigation.
• Members considered a report describing a case of
Creutzfeldt-Jakob Disease (CJD) of prion protein gene codon
129 VV genotype who had died in 2000 at the age of 39 years
old. The case had unusual neuropathological features and
abnormal prion protein (PrPSc) western blot banding pattern1.
One possible interpretation of these data was that this could
represent the first case of vCJD in an individual of VV
genotype. However, members noted that the clinical, cerebral
Magnetic Resonance Imaging and neuropathological features
were within the range previously observed with sporadic CJD
(sCJD). Although there were similarities between the
molecular features of PrPSc in the case and those of cases of
vCJD, they were not identical and only strain typing mouse
bioassays could provide conclusive evidence about the
causative transmissible spongiform encephalopathy (TSE)
agent. The paper stated that transmission studies of this
case, in transgenic mice, were being undertaken but there
was no information about the current status of these
experiments. The Chair asked the Acting Secretary to contact
1 Mead et al. (2007) Creutzfeldt-Jakob disease, prion protein gene codon
129VV, and
a novel PrPSc type in a young British women. Arch. Neurol. 64, 1780-1784.
5
© SEAC 2007
the research group to ask about such transmission
experiments.
• A member informed SEAC that the Department for
Environment, Food and Rural Affairs (Defra) was consulting
on cost and responsibility sharing for animal health and
welfare2 and that this included specific proposals for TSE
controls between government and industry.
snip...
11. Dr Danny Matthews (Veterinary Laboratories Agency [VLA]) noted
that all three of the atypical scrapie cases associated with ARSU
are of the Cheviot breed and two were homozygous and one was
heterozygous for the AFRQ allele. It was possible that the sheep
carrying this allele may be susceptible to atypical scrapie that
arises spontaneously.
2 Defra consultation on sharing costs and responsibility: animal health and
welfare.
http://www.defra.gov.uk/news/latest/2007/animal-1211.htm
6
© SEAC 2007
12. Members asked what measures might be taken to minimise the
spread of atypical scrapie at the site referred to in paragraph 15 of
the report and were informed that manure from the Unit had been
spread on adjacent farmland, which provided a source of straw of
the Unit. It had been suggested this practice might represent a
potential route for recycling of the atypical scrapie agent, and may
be inadvisable.
13. In relation to (ii), members noted there were four hypotheses for
the interpretation of the findings from passage of two sheep TSE
cases in mice: an experimental error had occurred, the features
observed may be a normal consequence of passage of classical
scrapie isolates in the breed and genotype of sheep, a strain
conversion may have occurred or the features observed may
reflect a mixed BSE-classical scrapie infection in sheep.
14. Dr Matthews explained that an internal audit had found no
evidence of experimental error but an independent audit was
planned with Professor Alun Williams as the scientific advisor. Dr
Jim Hope (VLA) explained that the suggestion that the features
observed on strain typing of two sheep TSE cases may be a
normal consequence of passage of classical scrapie isolates from
the particular breed and genotype of sheep, had arisen as one of
the cases was an ARQ/ARQ Swaledale. Sheep of this genotype
and breed rarely succumb to classical scrapie. Only one other
case of classical scrapie in an animal of this genotype and breed
had been strain typed by mouse bioassay with the features on
passage consistent with classical scrapie.
15. The Chair considered that of the four possibilities, the first two
appear to be the least likely, the third possibility may be the most
likely but is unproven and the fourth possibility cannot be excluded.
It is important to note that the features observed on mouse
bioassay are not consistent with features observed when BSE is
passaged in mice. If the findings did reflect a mixed BSE-classical
scrapie infection, the isolates were from historic sheep TSE cases
and the probability of mixed infections was low. Thus, there is no
indication from these data of a current significant risk to human
health from BSE in sheep.
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered.
17. A member suggested that less certainty should be reflected in the
estimate of the probability of mixed infection arising (footnote 8 of
the draft report) as the estimates relied on an assumption that BSE
and classical scrapie would occur independently. In addition, it
was incorrect to state that the most likely prevalence of BSE in
sheep was zero (paragraphs 32 and 39 of the draft report); the
sample gives an estimate of the maximum prevalence that is
consistent with the results of the survey.
snip...
ITEM 5 – SCIENTIFIC BASIS OF CLASSICAL SCRAPIE CONTROLS
(SEAC 99/3)
21. Mr Andrew Gresham (Defra) gave an overview of the background
and policy context of the issue. The European Court of First
Instance had, following an application by the French Government
and, pending a full hearing, suspended clauses in new European
Commission legislation to allow sheep from classical scrapie
affected flocks to enter the human food chain if testing negative for
TSE. The UK intended to support the Commission at the full
hearing but wished to seek SEAC’s advice in relation to possible
links between classical scrapie and human TSEs and the
performance characteristics of discriminatory tests for sheep TSEs.
SEAC had been provided with the opinions of the French Food
Safety Authority (AFSSA), the European Food Safety Authority
(EFSA) and the German TSE advisory committee (KOM AG TSE)
8
© SEAC 2007
that had considered these issues. Advice from SEAC could be
incorporated into a UK submission to the Court.
22. A member noted that the AFSSA opinion reflected concerns that
as a consequence of the release of animals from classical scrapie
affected sheep flocks into the human chain, cases of undiagnosed
BSE may also be inadvertently released into the food chain.
Furthermore, a greater number of classical scrapie infected sheep
may enter the food chain even though it is not possible to exclude
a risk to human health from classical scrapie. Three key
uncertainties had been identified by AFSSA, EFSA and KOM AG
TSE, although there were some differences in emphasis about the
uncertainties in the opinions. The uncertainties related to (i) the
capability of tests to detect TSEs in sheep during the stage when
PrPSc is accumulating in the periphery only, (ii) the ability of the
tests to detect BSE when another TSE is present and (iii) the
evidence suggesting a lack of link between human and animal
TSEs other than BSE. In relation to (iii), observations that classical
scrapie has been an endemic disease in sheep for more than 200
years without any apparent association with human disease, and
that sporadic Creutzfeldt-Jakob Disease (sCJD) exists in countries
such as Australia and New Zealand with no reported cases of
classical scrapie, are incontrovertible. However, it should be noted
that it would be very difficult to demonstrate an epidemiological link
between such relatively rare diseases in animals and humans.
Authors of two epidemiological studies3,4 that had examined risk
factors for sporadic Creutzfeldt-Jakob Disease (sCJD) dismissed a
link between classical scrapie and sCJD. However, these data
could be interpreted differently to suggest a potential link, this
could be a chance association arising from biases inherent in the
design of these retrospective studies. It was therefore important
not to be completely dismissive of a lack of a link as it would be
very difficult to prove an epidemiological link between such rare
diseases.
23. Members noted that, although there is no evidence for a risk to
human health from classical scrapie, a risk could never be ruled
out. However, even if there is a risk, the risk must be very small
indeed as the observed prevalence of sCJD is very low.
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION
40. The Chair explained that the purpose of the question and answer
session was to give members of the public an opportunity to ask
questions related to the work of SEAC. Mr Terry Singeltary
(Texas, USA) had submitted a question prior to the meeting,
asking: “With the Nor-98 now documented in five different states so
far in the USA in 2007, and with the two atypical BSE H-base
13
© SEAC 2007
cases in Texas and Alabama, with both scrapie and chronic
wasting disease (CWD) running rampant in the USA, is there any
concern from SEAC with the rise of sporadic CJD in the USA from
''unknown phenotype'', and what concerns if any, in relations to
blood donations, surgery, optical, and dental treatment, do you
have with these unknown atypical phenotypes in both humans and
animals in the USA? Does it concern SEAC, or is it of no concern
to SEAC? Should it concern USA animal and human health
officials?”
41. A member considered that this question appeared to be primarily
related to possible links between animal and human TSEs in the
USA. There is no evidence that sCJD is increasing in the USA and
no evidence of any direct link between TSEs and CJD in the USA.
Current evidence does not suggest that CWD is a significant risk to
human health. There are unpublished data from a case of human
TSE in the USA that are suggestive of an apparently novel form of
prion disease with distinct molecular characteristics. However, it is
unclear whether the case had been further characterised, if it could
be linked to animal TSEs or if other similar cases had been found
in the USA or elsewhere. In relation to the possible public health
implications of atypical scrapie, H-type BSE and CWD, research
was being conducted to investigate possible links and surveillance
was in place to detect any changes in human prion diseases.
Although possible links between these diseases and human TSEs
are of concern and require research, there is no evidence to
suggest immediate public health action is warranted. The possible
human health risks from classical scrapie had been discussed
earlier in the meeting. Members noted that there are effective
channels of discussion and collaboration on research between
USA and European groups. Members agreed it is important that to
keep a watching brief on new developments on TSEs.
ITEM 9 – UPDATE ON vCJD AND sCJD EPIDEMIOLOGY
42. Dr Richard Knight (NCJDSU) presented an update on the
epidemiology of cases of sCJD and vCJD in the UK and
elsewhere. Between May 1990 and October 2007, 944 cases of
sCJD had been identified in the UK with a mean age at onset of 66
(range 15-94) years and mean age of death of 67 (range 20-95)
years. There is no significant gender difference in sCJD incidence.
There had been a trend towards an increasing number of cases
over time to almost 80 cases per year in 2003; this increased trend
had also been observed in other countries and was considered to
be a result of better surveillance and diagnosis of disease. There
has been a decline in number since 2003, but this may not be of
15
© SEAC 2007
significance. The post mortem rate for sCJD referral is about 60%.
The genotype distribution of sCJD cases was 64% MM, 18% MV
and 18% VV at codon 129 of the prion protein gene.
43. Dr Knight explained that the total number of definite and probable
vCJD cases in the UK up to November 2007 was 166, with four
cases still alive. Three of out of four vCJD cases treated with
pentosan polysulphate (PPS) had appreciably longer survival
times, but it is not proven that this is the result of treatment. No
statistically significant gender difference had been observed in
vCJD cases. The age distribution of vCJD had not altered over the
course of the UK epidemic, with the median age of death of 30
(range 14-75) years. Statistical analysis of the UK incidence of
deaths from vCJD suggested the epidemic had peaked in 2000
with 28 deaths. There are three cases identified with onset in 2006
and four deaths in 2007. Geographical distribution of vCJD cases
in the UK shows higher incidence in the North than South. All 146
vCJD cases tested to date are of the MM genotype.
44. Dr Knight explained that elsewhere in the world up to November
2007, 39 vCJD cases have been reported with 23 in France, four in
the Republic of Ireland (RoI), three in the USA, two in the
Netherlands, two in Portugal and single cases in Italy, Canada,
Japan, Saudi Arabia and Spain. Infection is considered likely to
have occurred in the UK in two RoI cases, two USA cases, one
French case, the Japanese, and Canadian cases. One of the
French cases had a history of possibly significant residence in the
UK. One USA case is thought likely to have been exposed to
infection in Saudi Arabia, rather than the USA.
45. Dr Knight explained that the Transfusion Medicine Epidemiology
Review study had identified four instances of vCJD infection
resulting from receipt of non-leucodepleted red blood cells donated
by individuals who had subsequently developed vCJD. The donors
developed clinical vCJD ranging from 17 months to 3.5 years after
blood donation and this indicates that blood can be infective 3.5
years before the development of clinical disease. Clinical vCJD
was identified in three recipients (all of MM genotype) between 6.5
and 8.3 years after receipt of blood. The fourth recipient, who died
of non-neurological disease, with only lymphoreticular evidence of
vCJD infection was of MV genotype.
46. In response to a question about the neuropathology of the vCJD
case that died after receiving PPS, Dr Knight explained that no
autopsy was undertaken.
16
© SEAC 2007
47. A member asked about the reason for the increase in sCJD
detection in the year up to 2003. Dr Knight replied that it was
probably due to better awareness of the disease and the
availability of better diagnostic methods such as cerebrospinal fluid
testing and magnetic resonance imaging.
48. Mr Mark Noterman (Department of Health [DH]) asked whether the
neuropathological referrals rate had increased after the Chief
Medical Officer’s letter to clinicians earlier in the year to remain
vigilant about cases of neurological disease that could be related to
prion disease. Dr Knight replied that there had been no
subsequent significant increase in referral rate.
snip...
ITEM 11 – RE-ASSESSMENT OF THE POTENTIAL RISK OF vCJD
TRANSMISSION VIA DENTISTRY (SEAC 99/7)
59. Dr Bennett and Dr Peter Grove (DH) presented findings of an
interim assessment examining the risk that vCJD may be
transmitted via dental procedures. As there is a lack of substantial
19
© SEAC 2007
data with which to accurately quantify many of the key parameters
in the risk assessment, plausible ranges for parameters were
established to take account of the often large uncertainties in the
data. The key areas of uncertainty are infectivity in dental and oral
tissues of patients incubating vCJD, the level of protein residues on
dental instruments following decontamination, the efficacy of
autoclaving, the current prevalence of vCJD infection in the
population, and the epidemiology of vCJD. These uncertainties
strongly influence the quantification of the risk.
60. It was explained that many plausible scenarios built up using
ranges for each of these factors suggest that dental transmission
may have no detectable effect on the course of the vCJD epidemic.
However, there are some scenarios which include a combination of
pessimistic assumptions as regards the infectivity of dental/oral
tissues and the effects of instrument decontamination which
suggest that there could be some hundreds of vCJD transmissions
per annum via dentistry, albeit against a background of many
thousand existing subclinical vCJD infections, or where dental
transmission could generate a self-sustaining reservoir of vCJD
infection within the population. Should a large proportion of
secondary transmissions result in subclinical infections, either
never developing into clinical disease or doing so over an extended
time-scale, and such infections are infectious, the likelihood of a
self-sustaining epidemic increases. The proportion of individuals
that may be infected from having consumed BSE contaminated
food or from human to human transmission of vCJD that may enter
such a subclinical carrier state is unknown. Research to address
the key uncertainties is on-going and new data would enable some
of the assumptions underpinning these scenarios to be revised.
61. The committee welcomed the risk assessment, acknowledging it
had been developed in collaboration with a scientific reference
group of independent experts. Studies to address the scientific
uncertainties were considered important, particularly infectivity
studies on human oral and dental tissues from vCJD patients.
62. A member suggested that following secondary transmission, the
agent may adapt to become infectious to all prion protein
genotypes. Dr Grove noted that since the risk assessment
considers four scenarios ranging from one in which no secondary
infection develops into clinical disease to one in which everyone
who is infected develops clinical disease, this possibility is
considered.
20
© SEAC 2007
63. Members noted that there were two obvious precautionary
measures that could be put in place to dramatically reduce the
potential risk of vCJD transmission via dental procedures: making
endodontic files and reamers single use, which was implemented
in April 2007 and improving instrument decontamination using
current technologies. A 0.5 – 1.0 log reduction in infectivity from
improved decontamination practice could remove the risk of a self
sustaining epidemic. It is very important, therefore, that DH
ensures dentists do adopt good practice throughout the profession
and that this is audited. Introduction of consistent decontamination
practices would also reduce the observed variability of instrument
contamination, and thus reduce the risk of local outbreaks of
transmission.
64. Mr Barry Cockcroft (Chief Dental Officer, DH) noted that the effect
of the guidance on making endodontic files and reamers single use
had been included in the risk assessment. There is good evidence
that dentists are adhering to the guidance. A survey by DH
Regional Directors of Public Health could not find any dentists who
are unaware of the Chief Dental Officer’s Professional Letter
advising that files and reamers should be treated as single use
only, and dental instrument suppliers have reported that sales of
files and reamers have increased dramatically since the guidance
was issued. A draft Health Technical Memorandum would be
issued for consultation in early 2008 designed specifically for
dental practitioners and their staff as a comprehensive guide to
best practice. An audit tool will be available to help dentists assess
their own compliance with the guidance and to enable DH to
assess whether new guidance is working in practice. Dental
nurses will now also be regulated and registered with the General
Dental Council.
snip...end...full text ;
http://www.seac.gov.uk/minutes/99.pdf
SEAC stated ;
> There is no evidence that sCJD is increasing in the USA
i respectfully beg to differ. from 26 documented sCJD cases in 1996 rising to 157 documented sCJD cases in 2004, with 152 cases in 2005, to 143 in 2006, this would seem to be a rise in documented sporadic CJD cases from 1996 to me. ...TSS
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
> 1996 / 42 / 32 / 26 / 4 / 0 / 0
> 1997 / 115 / 68 / 57 / 9 / 0 / 0
> 1998 / 93 / 53 / 45 / 7 / 1 / 0
> 1999 / 114 / 69 / 61 / 8 / 0 / 0
> 2000 / 151 / 103 / 89 / 14 / 0 / 0
> 2001 / 208 / 116 / 106 / 9 / 0 / 0
> 2002 / 255 / 143 / 118 / 23 / 2 / 0
> 2003 / 272 / 174 / 132 / 41 / 0 / 0
> 2004 / 334 / 183 / 157 / 21 / 0 / 1*
> 2005 / 352 / 195 / 152 / 37 / 1 / 0
> 2006 / 372 / 186 / 143 / 30 / 0 / 1**
> 2007 / 120 / 68 / 35 / 7 / 0 / 0
A ProMED-mail post
snip...
[2] USA: National Prion Disease Pathology Surveillance Center
Date: June 2007
Source: National Prion Disease Pathology Surveillance Center (USA) [edited]
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
1996 / 42 / 32 / 26 / 4 / 0 / 0
1997 / 115 / 68 / 57 / 9 / 0 / 0
1998 / 93 / 53 / 45 / 7 / 1 / 0
1999 / 114 / 69 / 61 / 8 / 0 / 0
2000 / 151 / 103 / 89 / 14 / 0 / 0
2001 / 208 / 116 / 106 / 9 / 0 / 0
2002 / 255 / 143 / 118 / 23 / 2 / 0
2003 / 272 / 174 / 132 / 41 / 0 / 0
2004 / 334 / 183 / 157 / 21 / 0 / 1*
2005 / 352 / 195 / 152 / 37 / 1 / 0
2006 / 372 / 186 / 143 / 30 / 0 / 1**
2007 / 120 / 68 / 35 / 7 / 0 / 0
TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Communicated by:
Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. - Mod.CP]
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
PLEASE NOTE SEAC COMMENT ;
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered. ...END
PLEASE SEE THE FOLLOWING ;
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease
Magdalini Polymenidou, Katharina Stoeck, Markus
Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi
Interpretation
The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD classifications.
snip...
The above results set the existing CJD classifications into debate and introduce interesting questions about human CJD types. For example, do human prion types exist in a dynamic equilibrium in the brains of affected individuals? Do they coexist in most or even all CJD cases? Is the biochemically identified PrPSc type simply the dominant type, and not the only PrPSc species?
Published online October 31, 2005
http://neurology.thelancet.com
Detection of Type 1 Prion Protein in Variant Creutzfeldt-Jakob Disease
Helen M. Yull,* Diane L. Ritchie,*
Jan P.M. Langeveld,? Fred G. van Zijderveld,?
Moira E. Bruce,? James W. Ironside,* and
Mark W. Head*
From the National CJD Surveillance Unit,* School of Molecular
and Clinical Medicine, University of Edinburgh, Edinburgh,
United Kingdom; Central Institute for Animal Disease Control
(CIDC)-Lelystad, ? Lelystad, The Netherlands; Institute for Animal
Health, Neuropathogenesis Unit, ? Edinburgh, United Kingdom
Molecular typing of the abnormal form of the prion protein (PrPSc) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrPSc molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). Here we show for the first time that the PrPSc that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component. This minority type 1 PrPSc was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective
of brain region examined, and was also present in the variant Creutzfeldt-Jakob disease tonsil.
The quantitative balance between PrPSc types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was also found in bovine spongiform encephalopathy cattle brain, indicating that the agent rather than the host specifies their relative representation. These results indicate that PrPSc molecular typing is based on quantitative rather than qualitative phenomena and point to a complex relationship between prion protein biochemistry, disease phenotype and agent strain.
(Am J Pathol 2006, 168:151-157;
DOI: 10.2353/ajpath.2006.050766)
snip...END
http://ajp.amjpathol.org/
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older
population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers
ever since. What I have found is that we have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15,
2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number
of campaigners who say that some sCJD, like the variant CJD related to BSE,
is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by some
strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in
Fontenay-aux-Roses, south-west of Paris.
Hans Kretschmar of the University of Göttingen, who coordinates CJD
surveillance in Germany, is so concerned by the findings that he now wants
to trawl back through past sCJD cases to see if any might have been caused
by eating infected mutton or lamb. ...
http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html
DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen)
USA: Loch in der Mauer
Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas
verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehörden
sind lax.
Link auf diesen Artikel im Archiv:
http://service.spiegel.de/digas/find?DID=18578755
"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA
regulations. ...
http://service.spiegel.de/digas/servlet/find/DID=18578755
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in
the United States
http://cjdusa.blogspot.com/
Sunday, December 16, 2007
Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006
http://cjdtexas.blogspot.com/
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
NOR-98 ATYPICAL SCRAPIE CASES USA
http://nor-98.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
Draft minutes of the 99th meeting held on 14th December 2007
snip...
• A member explained that a confirmed case of atypical scrapie
had been recently identified in a research project at the
Institute of Animal Health (IAH). The animal had been born in
1997 in New Zealand, imported into the UK in 1998, spending
the first six months at the Arthur Rickwood Sheep Unit
(ARSU) before transfer to the IAH site at Compton. Thus, it is
possible it may have become infected either in New Zealand,
whilst at ARSU, where two other cases have now been
confirmed, or at Compton, perhaps as a result of the
experiments conducted during the research study involving
transfusion of blood between sheep. The case is under
further investigation.
• Members considered a report describing a case of
Creutzfeldt-Jakob Disease (CJD) of prion protein gene codon
129 VV genotype who had died in 2000 at the age of 39 years
old. The case had unusual neuropathological features and
abnormal prion protein (PrPSc) western blot banding pattern1.
One possible interpretation of these data was that this could
represent the first case of vCJD in an individual of VV
genotype. However, members noted that the clinical, cerebral
Magnetic Resonance Imaging and neuropathological features
were within the range previously observed with sporadic CJD
(sCJD). Although there were similarities between the
molecular features of PrPSc in the case and those of cases of
vCJD, they were not identical and only strain typing mouse
bioassays could provide conclusive evidence about the
causative transmissible spongiform encephalopathy (TSE)
agent. The paper stated that transmission studies of this
case, in transgenic mice, were being undertaken but there
was no information about the current status of these
experiments. The Chair asked the Acting Secretary to contact
1 Mead et al. (2007) Creutzfeldt-Jakob disease, prion protein gene codon
129VV, and
a novel PrPSc type in a young British women. Arch. Neurol. 64, 1780-1784.
5
© SEAC 2007
the research group to ask about such transmission
experiments.
• A member informed SEAC that the Department for
Environment, Food and Rural Affairs (Defra) was consulting
on cost and responsibility sharing for animal health and
welfare2 and that this included specific proposals for TSE
controls between government and industry.
snip...
11. Dr Danny Matthews (Veterinary Laboratories Agency [VLA]) noted
that all three of the atypical scrapie cases associated with ARSU
are of the Cheviot breed and two were homozygous and one was
heterozygous for the AFRQ allele. It was possible that the sheep
carrying this allele may be susceptible to atypical scrapie that
arises spontaneously.
2 Defra consultation on sharing costs and responsibility: animal health and
welfare.
http://www.defra.gov.uk/news/latest/2007/animal-1211.htm
6
© SEAC 2007
12. Members asked what measures might be taken to minimise the
spread of atypical scrapie at the site referred to in paragraph 15 of
the report and were informed that manure from the Unit had been
spread on adjacent farmland, which provided a source of straw of
the Unit. It had been suggested this practice might represent a
potential route for recycling of the atypical scrapie agent, and may
be inadvisable.
13. In relation to (ii), members noted there were four hypotheses for
the interpretation of the findings from passage of two sheep TSE
cases in mice: an experimental error had occurred, the features
observed may be a normal consequence of passage of classical
scrapie isolates in the breed and genotype of sheep, a strain
conversion may have occurred or the features observed may
reflect a mixed BSE-classical scrapie infection in sheep.
14. Dr Matthews explained that an internal audit had found no
evidence of experimental error but an independent audit was
planned with Professor Alun Williams as the scientific advisor. Dr
Jim Hope (VLA) explained that the suggestion that the features
observed on strain typing of two sheep TSE cases may be a
normal consequence of passage of classical scrapie isolates from
the particular breed and genotype of sheep, had arisen as one of
the cases was an ARQ/ARQ Swaledale. Sheep of this genotype
and breed rarely succumb to classical scrapie. Only one other
case of classical scrapie in an animal of this genotype and breed
had been strain typed by mouse bioassay with the features on
passage consistent with classical scrapie.
15. The Chair considered that of the four possibilities, the first two
appear to be the least likely, the third possibility may be the most
likely but is unproven and the fourth possibility cannot be excluded.
It is important to note that the features observed on mouse
bioassay are not consistent with features observed when BSE is
passaged in mice. If the findings did reflect a mixed BSE-classical
scrapie infection, the isolates were from historic sheep TSE cases
and the probability of mixed infections was low. Thus, there is no
indication from these data of a current significant risk to human
health from BSE in sheep.
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered.
17. A member suggested that less certainty should be reflected in the
estimate of the probability of mixed infection arising (footnote 8 of
the draft report) as the estimates relied on an assumption that BSE
and classical scrapie would occur independently. In addition, it
was incorrect to state that the most likely prevalence of BSE in
sheep was zero (paragraphs 32 and 39 of the draft report); the
sample gives an estimate of the maximum prevalence that is
consistent with the results of the survey.
snip...
ITEM 5 – SCIENTIFIC BASIS OF CLASSICAL SCRAPIE CONTROLS
(SEAC 99/3)
21. Mr Andrew Gresham (Defra) gave an overview of the background
and policy context of the issue. The European Court of First
Instance had, following an application by the French Government
and, pending a full hearing, suspended clauses in new European
Commission legislation to allow sheep from classical scrapie
affected flocks to enter the human food chain if testing negative for
TSE. The UK intended to support the Commission at the full
hearing but wished to seek SEAC’s advice in relation to possible
links between classical scrapie and human TSEs and the
performance characteristics of discriminatory tests for sheep TSEs.
SEAC had been provided with the opinions of the French Food
Safety Authority (AFSSA), the European Food Safety Authority
(EFSA) and the German TSE advisory committee (KOM AG TSE)
8
© SEAC 2007
that had considered these issues. Advice from SEAC could be
incorporated into a UK submission to the Court.
22. A member noted that the AFSSA opinion reflected concerns that
as a consequence of the release of animals from classical scrapie
affected sheep flocks into the human chain, cases of undiagnosed
BSE may also be inadvertently released into the food chain.
Furthermore, a greater number of classical scrapie infected sheep
may enter the food chain even though it is not possible to exclude
a risk to human health from classical scrapie. Three key
uncertainties had been identified by AFSSA, EFSA and KOM AG
TSE, although there were some differences in emphasis about the
uncertainties in the opinions. The uncertainties related to (i) the
capability of tests to detect TSEs in sheep during the stage when
PrPSc is accumulating in the periphery only, (ii) the ability of the
tests to detect BSE when another TSE is present and (iii) the
evidence suggesting a lack of link between human and animal
TSEs other than BSE. In relation to (iii), observations that classical
scrapie has been an endemic disease in sheep for more than 200
years without any apparent association with human disease, and
that sporadic Creutzfeldt-Jakob Disease (sCJD) exists in countries
such as Australia and New Zealand with no reported cases of
classical scrapie, are incontrovertible. However, it should be noted
that it would be very difficult to demonstrate an epidemiological link
between such relatively rare diseases in animals and humans.
Authors of two epidemiological studies3,4 that had examined risk
factors for sporadic Creutzfeldt-Jakob Disease (sCJD) dismissed a
link between classical scrapie and sCJD. However, these data
could be interpreted differently to suggest a potential link, this
could be a chance association arising from biases inherent in the
design of these retrospective studies. It was therefore important
not to be completely dismissive of a lack of a link as it would be
very difficult to prove an epidemiological link between such rare
diseases.
23. Members noted that, although there is no evidence for a risk to
human health from classical scrapie, a risk could never be ruled
out. However, even if there is a risk, the risk must be very small
indeed as the observed prevalence of sCJD is very low.
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION
40. The Chair explained that the purpose of the question and answer
session was to give members of the public an opportunity to ask
questions related to the work of SEAC. Mr Terry Singeltary
(Texas, USA) had submitted a question prior to the meeting,
asking: “With the Nor-98 now documented in five different states so
far in the USA in 2007, and with the two atypical BSE H-base
13
© SEAC 2007
cases in Texas and Alabama, with both scrapie and chronic
wasting disease (CWD) running rampant in the USA, is there any
concern from SEAC with the rise of sporadic CJD in the USA from
''unknown phenotype'', and what concerns if any, in relations to
blood donations, surgery, optical, and dental treatment, do you
have with these unknown atypical phenotypes in both humans and
animals in the USA? Does it concern SEAC, or is it of no concern
to SEAC? Should it concern USA animal and human health
officials?”
41. A member considered that this question appeared to be primarily
related to possible links between animal and human TSEs in the
USA. There is no evidence that sCJD is increasing in the USA and
no evidence of any direct link between TSEs and CJD in the USA.
Current evidence does not suggest that CWD is a significant risk to
human health. There are unpublished data from a case of human
TSE in the USA that are suggestive of an apparently novel form of
prion disease with distinct molecular characteristics. However, it is
unclear whether the case had been further characterised, if it could
be linked to animal TSEs or if other similar cases had been found
in the USA or elsewhere. In relation to the possible public health
implications of atypical scrapie, H-type BSE and CWD, research
was being conducted to investigate possible links and surveillance
was in place to detect any changes in human prion diseases.
Although possible links between these diseases and human TSEs
are of concern and require research, there is no evidence to
suggest immediate public health action is warranted. The possible
human health risks from classical scrapie had been discussed
earlier in the meeting. Members noted that there are effective
channels of discussion and collaboration on research between
USA and European groups. Members agreed it is important that to
keep a watching brief on new developments on TSEs.
ITEM 9 – UPDATE ON vCJD AND sCJD EPIDEMIOLOGY
42. Dr Richard Knight (NCJDSU) presented an update on the
epidemiology of cases of sCJD and vCJD in the UK and
elsewhere. Between May 1990 and October 2007, 944 cases of
sCJD had been identified in the UK with a mean age at onset of 66
(range 15-94) years and mean age of death of 67 (range 20-95)
years. There is no significant gender difference in sCJD incidence.
There had been a trend towards an increasing number of cases
over time to almost 80 cases per year in 2003; this increased trend
had also been observed in other countries and was considered to
be a result of better surveillance and diagnosis of disease. There
has been a decline in number since 2003, but this may not be of
15
© SEAC 2007
significance. The post mortem rate for sCJD referral is about 60%.
The genotype distribution of sCJD cases was 64% MM, 18% MV
and 18% VV at codon 129 of the prion protein gene.
43. Dr Knight explained that the total number of definite and probable
vCJD cases in the UK up to November 2007 was 166, with four
cases still alive. Three of out of four vCJD cases treated with
pentosan polysulphate (PPS) had appreciably longer survival
times, but it is not proven that this is the result of treatment. No
statistically significant gender difference had been observed in
vCJD cases. The age distribution of vCJD had not altered over the
course of the UK epidemic, with the median age of death of 30
(range 14-75) years. Statistical analysis of the UK incidence of
deaths from vCJD suggested the epidemic had peaked in 2000
with 28 deaths. There are three cases identified with onset in 2006
and four deaths in 2007. Geographical distribution of vCJD cases
in the UK shows higher incidence in the North than South. All 146
vCJD cases tested to date are of the MM genotype.
44. Dr Knight explained that elsewhere in the world up to November
2007, 39 vCJD cases have been reported with 23 in France, four in
the Republic of Ireland (RoI), three in the USA, two in the
Netherlands, two in Portugal and single cases in Italy, Canada,
Japan, Saudi Arabia and Spain. Infection is considered likely to
have occurred in the UK in two RoI cases, two USA cases, one
French case, the Japanese, and Canadian cases. One of the
French cases had a history of possibly significant residence in the
UK. One USA case is thought likely to have been exposed to
infection in Saudi Arabia, rather than the USA.
45. Dr Knight explained that the Transfusion Medicine Epidemiology
Review study had identified four instances of vCJD infection
resulting from receipt of non-leucodepleted red blood cells donated
by individuals who had subsequently developed vCJD. The donors
developed clinical vCJD ranging from 17 months to 3.5 years after
blood donation and this indicates that blood can be infective 3.5
years before the development of clinical disease. Clinical vCJD
was identified in three recipients (all of MM genotype) between 6.5
and 8.3 years after receipt of blood. The fourth recipient, who died
of non-neurological disease, with only lymphoreticular evidence of
vCJD infection was of MV genotype.
46. In response to a question about the neuropathology of the vCJD
case that died after receiving PPS, Dr Knight explained that no
autopsy was undertaken.
16
© SEAC 2007
47. A member asked about the reason for the increase in sCJD
detection in the year up to 2003. Dr Knight replied that it was
probably due to better awareness of the disease and the
availability of better diagnostic methods such as cerebrospinal fluid
testing and magnetic resonance imaging.
48. Mr Mark Noterman (Department of Health [DH]) asked whether the
neuropathological referrals rate had increased after the Chief
Medical Officer’s letter to clinicians earlier in the year to remain
vigilant about cases of neurological disease that could be related to
prion disease. Dr Knight replied that there had been no
subsequent significant increase in referral rate.
snip...
ITEM 11 – RE-ASSESSMENT OF THE POTENTIAL RISK OF vCJD
TRANSMISSION VIA DENTISTRY (SEAC 99/7)
59. Dr Bennett and Dr Peter Grove (DH) presented findings of an
interim assessment examining the risk that vCJD may be
transmitted via dental procedures. As there is a lack of substantial
19
© SEAC 2007
data with which to accurately quantify many of the key parameters
in the risk assessment, plausible ranges for parameters were
established to take account of the often large uncertainties in the
data. The key areas of uncertainty are infectivity in dental and oral
tissues of patients incubating vCJD, the level of protein residues on
dental instruments following decontamination, the efficacy of
autoclaving, the current prevalence of vCJD infection in the
population, and the epidemiology of vCJD. These uncertainties
strongly influence the quantification of the risk.
60. It was explained that many plausible scenarios built up using
ranges for each of these factors suggest that dental transmission
may have no detectable effect on the course of the vCJD epidemic.
However, there are some scenarios which include a combination of
pessimistic assumptions as regards the infectivity of dental/oral
tissues and the effects of instrument decontamination which
suggest that there could be some hundreds of vCJD transmissions
per annum via dentistry, albeit against a background of many
thousand existing subclinical vCJD infections, or where dental
transmission could generate a self-sustaining reservoir of vCJD
infection within the population. Should a large proportion of
secondary transmissions result in subclinical infections, either
never developing into clinical disease or doing so over an extended
time-scale, and such infections are infectious, the likelihood of a
self-sustaining epidemic increases. The proportion of individuals
that may be infected from having consumed BSE contaminated
food or from human to human transmission of vCJD that may enter
such a subclinical carrier state is unknown. Research to address
the key uncertainties is on-going and new data would enable some
of the assumptions underpinning these scenarios to be revised.
61. The committee welcomed the risk assessment, acknowledging it
had been developed in collaboration with a scientific reference
group of independent experts. Studies to address the scientific
uncertainties were considered important, particularly infectivity
studies on human oral and dental tissues from vCJD patients.
62. A member suggested that following secondary transmission, the
agent may adapt to become infectious to all prion protein
genotypes. Dr Grove noted that since the risk assessment
considers four scenarios ranging from one in which no secondary
infection develops into clinical disease to one in which everyone
who is infected develops clinical disease, this possibility is
considered.
20
© SEAC 2007
63. Members noted that there were two obvious precautionary
measures that could be put in place to dramatically reduce the
potential risk of vCJD transmission via dental procedures: making
endodontic files and reamers single use, which was implemented
in April 2007 and improving instrument decontamination using
current technologies. A 0.5 – 1.0 log reduction in infectivity from
improved decontamination practice could remove the risk of a self
sustaining epidemic. It is very important, therefore, that DH
ensures dentists do adopt good practice throughout the profession
and that this is audited. Introduction of consistent decontamination
practices would also reduce the observed variability of instrument
contamination, and thus reduce the risk of local outbreaks of
transmission.
64. Mr Barry Cockcroft (Chief Dental Officer, DH) noted that the effect
of the guidance on making endodontic files and reamers single use
had been included in the risk assessment. There is good evidence
that dentists are adhering to the guidance. A survey by DH
Regional Directors of Public Health could not find any dentists who
are unaware of the Chief Dental Officer’s Professional Letter
advising that files and reamers should be treated as single use
only, and dental instrument suppliers have reported that sales of
files and reamers have increased dramatically since the guidance
was issued. A draft Health Technical Memorandum would be
issued for consultation in early 2008 designed specifically for
dental practitioners and their staff as a comprehensive guide to
best practice. An audit tool will be available to help dentists assess
their own compliance with the guidance and to enable DH to
assess whether new guidance is working in practice. Dental
nurses will now also be regulated and registered with the General
Dental Council.
snip...end...full text ;
http://www.seac.gov.uk/minutes/99.pdf
SEAC stated ;
> There is no evidence that sCJD is increasing in the USA
i respectfully beg to differ. from 26 documented sCJD cases in 1996 rising to 157 documented sCJD cases in 2004, with 152 cases in 2005, to 143 in 2006, this would seem to be a rise in documented sporadic CJD cases from 1996 to me. ...TSS
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
> 1996 / 42 / 32 / 26 / 4 / 0 / 0
> 1997 / 115 / 68 / 57 / 9 / 0 / 0
> 1998 / 93 / 53 / 45 / 7 / 1 / 0
> 1999 / 114 / 69 / 61 / 8 / 0 / 0
> 2000 / 151 / 103 / 89 / 14 / 0 / 0
> 2001 / 208 / 116 / 106 / 9 / 0 / 0
> 2002 / 255 / 143 / 118 / 23 / 2 / 0
> 2003 / 272 / 174 / 132 / 41 / 0 / 0
> 2004 / 334 / 183 / 157 / 21 / 0 / 1*
> 2005 / 352 / 195 / 152 / 37 / 1 / 0
> 2006 / 372 / 186 / 143 / 30 / 0 / 1**
> 2007 / 120 / 68 / 35 / 7 / 0 / 0
A ProMED-mail post
snip...
[2] USA: National Prion Disease Pathology Surveillance Center
Date: June 2007
Source: National Prion Disease Pathology Surveillance Center (USA) [edited]
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
1996 / 42 / 32 / 26 / 4 / 0 / 0
1997 / 115 / 68 / 57 / 9 / 0 / 0
1998 / 93 / 53 / 45 / 7 / 1 / 0
1999 / 114 / 69 / 61 / 8 / 0 / 0
2000 / 151 / 103 / 89 / 14 / 0 / 0
2001 / 208 / 116 / 106 / 9 / 0 / 0
2002 / 255 / 143 / 118 / 23 / 2 / 0
2003 / 272 / 174 / 132 / 41 / 0 / 0
2004 / 334 / 183 / 157 / 21 / 0 / 1*
2005 / 352 / 195 / 152 / 37 / 1 / 0
2006 / 372 / 186 / 143 / 30 / 0 / 1**
2007 / 120 / 68 / 35 / 7 / 0 / 0
TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Communicated by:
Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. - Mod.CP]
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
PLEASE NOTE SEAC COMMENT ;
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered. ...END
PLEASE SEE THE FOLLOWING ;
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease
Magdalini Polymenidou, Katharina Stoeck, Markus
Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi
Interpretation
The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD classifications.
snip...
The above results set the existing CJD classifications into debate and introduce interesting questions about human CJD types. For example, do human prion types exist in a dynamic equilibrium in the brains of affected individuals? Do they coexist in most or even all CJD cases? Is the biochemically identified PrPSc type simply the dominant type, and not the only PrPSc species?
Published online October 31, 2005
http://neurology.thelancet.com
Detection of Type 1 Prion Protein in Variant Creutzfeldt-Jakob Disease
Helen M. Yull,* Diane L. Ritchie,*
Jan P.M. Langeveld,? Fred G. van Zijderveld,?
Moira E. Bruce,? James W. Ironside,* and
Mark W. Head*
From the National CJD Surveillance Unit,* School of Molecular
and Clinical Medicine, University of Edinburgh, Edinburgh,
United Kingdom; Central Institute for Animal Disease Control
(CIDC)-Lelystad, ? Lelystad, The Netherlands; Institute for Animal
Health, Neuropathogenesis Unit, ? Edinburgh, United Kingdom
Molecular typing of the abnormal form of the prion protein (PrPSc) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrPSc molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). Here we show for the first time that the PrPSc that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component. This minority type 1 PrPSc was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective
of brain region examined, and was also present in the variant Creutzfeldt-Jakob disease tonsil.
The quantitative balance between PrPSc types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was also found in bovine spongiform encephalopathy cattle brain, indicating that the agent rather than the host specifies their relative representation. These results indicate that PrPSc molecular typing is based on quantitative rather than qualitative phenomena and point to a complex relationship between prion protein biochemistry, disease phenotype and agent strain.
(Am J Pathol 2006, 168:151-157;
DOI: 10.2353/ajpath.2006.050766)
snip...END
http://ajp.amjpathol.org/
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older
population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers
ever since. What I have found is that we have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15,
2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number
of campaigners who say that some sCJD, like the variant CJD related to BSE,
is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by some
strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in
Fontenay-aux-Roses, south-west of Paris.
Hans Kretschmar of the University of Göttingen, who coordinates CJD
surveillance in Germany, is so concerned by the findings that he now wants
to trawl back through past sCJD cases to see if any might have been caused
by eating infected mutton or lamb. ...
http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html
DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen)
USA: Loch in der Mauer
Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas
verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehörden
sind lax.
Link auf diesen Artikel im Archiv:
http://service.spiegel.de/digas/find?DID=18578755
"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA
regulations. ...
http://service.spiegel.de/digas/servlet/find/DID=18578755
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in
the United States
http://cjdusa.blogspot.com/
Sunday, December 16, 2007
Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006
http://cjdtexas.blogspot.com/
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
NOR-98 ATYPICAL SCRAPIE CASES USA
http://nor-98.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
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