CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA

Massachusetts patient tested for mad cow disease Sunday, July 20, 2008 8:33 PM

2008-07-21T20:22:00.000-07:00

Sunday, July 20, 2008 8:33 PM Massachusetts patient tested for mad cow disease

Massachusetts patient tested for mad cow disease

By STEVE LeBLANC – 34 minutes ago

BOSTON (AP) — Public health officials in Massachusetts are investigating whether a patient in a Cape Cod hospital has the human form of mad cow disease.

Dr. Alfred DeMaria, the state's director of communicable disease control, confirmed Sunday to The Associated Press that tests are being done to see if the patient has Creutzfeldt-Jakob disease and whether it's the variant attributed to mad cow.

There have only been three cases of the human form of mad cow disease reported in the United States in the last several years, and officials say it's extremely unlikely the patient in Cape Cod Hospital has the disease.

Mad cow disease — medically known as bovine spongiform encephalopathy, or BSE — causes spongy holes in the brain.

Eating meat products contaminated with mad cow disease is linked to variant Creutzfeldt-Jakob disease, a rare and fatal human malady.

DeMaria says it will take a few more days before the test results are available. He said there are about a half-dozen cases reported every year in Massachusetts and about 300 nationwide.

A spokesman for Cape Cod Hospital confirmed the facility notified public health officials Thursday of a patient with test results that require reporting. He said hospital officials were told the illness was not contagious and that there was no cause for concern.

snip...end

UPDATE

the old ukbsenvcjd only theory played out to a T. and why not, it's worked this long. ...tss

Test confirms Cape patient has rare brain disease July 21, 2008 02:49 PM By Stephen Smith, Globe Staff

An elderly patient on Cape Cod has tested positive for a rare brain ailment called Creutzfeldt-Jackob disease, state public health officials announced this afternoon.

Each year in Massachusetts, six or seven people are diagnosed with the degenerative disorder, which in most cases leads to rapid death.

The disease, known for decades among neurologists, first came to widespread public attention during the mad cow scare of the 1980s, when cases of the disorder were linked to tainted beef in the United Kingdom. But only three such cases have ever been identified in the United States, and all of those were in patients who had come from Great Britain.

Further tests will be conducted to determine the cause of the Cape patient's illness, but state disease trackers said there is nothing to suggest that the patient's case is associated with mad cow disease. Instead, like virtually all cases in the United States, it is almost certainly not linked to any obvious external cause.

http://www.boston.com/news/local/breaking_news/2008/07/test_confirms_c.html

don't these dummies know by now that the USA does not have any mad cow disease and or any human cjd ramifications from a mad cow, cause the USDA says so... NOT

there has been a decade old, systematic cover-up of corporate homicide just because of trade, futures and commodities. the elderly demented, your grandma and grandpa, mom and dad, sisters and brothers, are all expendable, due to the fact the American joe-cue-public is just to damn lazy to care. the elderly and demented are expendable. but mark my word here and now, it's here, and has been, call it what you like.....

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008

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...end

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

sporadic CJD, the big lie

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

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

Saturday, June 21, 2008 HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

FDA FAILED US

http://fdafailedus.blogspot.com/

SCIENCE BUSHWHACKED

http://sciencebushwhacked.blogspot.com/

Sunday, July 20, 2008 Red Cross told to fix blood collection or face charges 15 years after warnings issued, few changes made to ensure safety

http://vcjdblood.blogspot.com/2008/07/red-cross-told-to-fix-blood-collection.html

TSS

A New Prionopathy update July 10, 2008

2008-07-10T10:58:00.000-07:00

A New Prionopathy

In this issue of Annals of Neurology, Gambetti and colleagues1 describe a new form of prion disease designated proteinase-sensitive prionopathy (PSPr). The discovery of any new form of disease is a milestone. The identification of this novel phenotype of prion disease reflects the value of a rigorous systematic surveillance program and underlines the importance of neuropathological examination and prion protein (PrP) typing in prion disease classification.

PSPr is characterized clinically by a rather nonspecific phenotype involving progressive dementia, with ataxia and Parkinsonism occurring in a proportion of cases. Although it is possible that the relatively short total duration of illness (mean, 20 months) alerted clinicians to the possibility of the diagnosis, it is of note that none of the investigations generally used in the screening for human prion disease (electroencephalogram, cerebrospinal fluid 14-3-3, or magnetic resonance imaging brain scan) provided support for the diagnosis of Creutzfeldt–Jakob Disease (CJD). It was fortunate that these cases were referred by the National Prion Disease Pathology Surveillance Center and one can only speculate that additional cases of this condition were likely not to have been notified to the surveillance system.

PSPr accounted for 3% of all sporadic CJD cases identified by the National Prion Disease Pathology Surveillance Center, and with an overall annual incidence rate in sporadic CJD of about 1 to 1.5 cases/ million,2 this may be a rare form of dementia assuming that a significant proportion of all cases was identified. The authors make the important point that PSPr might be misclassified clinically as a form of non- Alzheimer’s dementia, but all cases had spongiform change on neuropathological examination in the cortex, basal ganglia, and thalamus, with positive PrP immunostaining in a similar distribution. In contrast with other forms of sporadic CJD (and control subjects), treatment with proteinase K almost abolished the immunostaining. It will be interesting to see whether a review of cases of CJD identified by other CJD surveillance systems will identify similar cases. Although final classification as PSPr requires detailed biochemical analysis of the PrP deposited in the brain, the diagnosis of a form of CJD may be made on routine histopathology. Thus, it is likely that cases of PSPr would have been identified as CJD (even if not appropriately subclassified), at least in countries with high postmortem rates for rapidly progressive dementia illnesses.

That PSPr is a novel form of human prion disease is supported by the unusual clinical phenotype, the distinctive neuropathological features, including clusters of PrP-positive granules in the cerebrum, cerebellum, and less frequently the white matter, and the presence of abnormal PrP that is overall more sensitive to proteolytic digestion, less highly aggregated, and yielding protease-resistant core fragments distinct from those found in other forms of CJD. The identification of any new form of prion disease inevitably raises questions about the origin of the condition.

In PSPr, there was a family history of dementia in 8 of 10 cases (a family history was not available in 1 case), but sequencing of the open reading frame of the prion protein gene (PRNP) did not identify any of the mutations associated with genetic forms of human prion disease. In sporadic CJD, a family history of dementia is present in 15% of cases,3 and although this may be related to the occasional misclassification of the cause of death in preceding generations, many of these cases also lack mutations in PRNP, which raises the possibility of genetic influences on human prion diseases outside of PRNP. This possibility is also consistent with studies in laboratory animals.4,5 Further study of the genetics of PSPr and other human prion diseases may provide important insights into the pathogenesis of these conditions. It is also noteworthy that all of the cases were valine homozygous at codon 129 of PRNP. With informed consent, genetic analysis is now performed routinely in many CJD surveillance centers, and the identification of this genotype in a suspect case may now raise suspicions of PSPr, particularly in cases with a relatively indolent onset and a family history of dementia.

The identification of PSPr raises a number of fundamental questions about prion diseases. This class of conditions was formally known as the transmissible spongiform encephalopathies, but there are examples of these conditions that are not transmissible and/or have no spongiform change. An unanswered question is whether PSPr is a transmissible disease, and appropriate laboratory investigations are under way.

The term prion disease has become in many but not all circles the conventional terminology for this group of diseases, but it is now clear that the biochemical spectrum of abnormal forms of the PrP that characterize these diseases is broad and cannot be defined simply as those in which conventional PrP27-30 is readily detectable. In addition to diseases such as some forms of Gerstmann-Straussler-Scheinker Disease (GSS) in which abnormal PrP is present but evidence of transmission lacking,6 there are examples of experimental “spongiform encephalopathies” that are transmissible but that EDITORIAL © 2008 American Neurological Association 677 Published by Wiley-Liss, Inc., through Wiley Subscription Services contain no detectable disease-associated PrP27-30 in brain tissue.7,8 The exact relation between abnormal PrP, transmissibility and disease phenotype therefore remains unknown, and a good working definition of prion disease remains problematic. This has been compounded by the recent description of protease-resistant forms of PrP in normal human brain9 and the apparent generation of infectious PrP from the brains of uninfected animal species by protein misfolding cyclic amplification.10 Perhaps the most appropriate current definition of prion disease is that proposed by the authors of this article: “A condition in which PrP is abnormal and appears to play a central role in pathology. 1” This definition does not necessarily imply transmissibility, and the importance of resolving the role of different forms of PrP in infectivity and the possibility of nontransmissible forms of these diseases have been raised by other authors.11

One component of the definition of PSPr is the presence of a particular biochemical type of PrP in the brains of affected cases. This raises the issue of whether it is appropriate to define a disease or imply a cause solely by the biochemical characteristics of the associated abnormal PrP. This does not apply to PSPr because the disease is defined by additional features, including the clinical phenotype and the histopathological appearances, as well as the PrP type, but the authors themselves note that from PrP biochemistry considered in isolation, it would be tempting to speculate that this disease is “sporadic GSS.” The seminal work in subclassifying sporadic CJD by PrP type and codon 129 genotype12 and in identifying an apparently specific PrP type in variant CJD13 has been extended to animal diseases, some of which are currently largely defined by PrP characteristics (eg, atypical scrapie and some forms of atypical bovine spongiform encephalopathy). In human disease, the use of PrP typing has been a major advance in disease classification, but there are overlaps of the biochemical features of abnormal PrP between diseases with quite distinct causes.14 Similarities in PrP biochemistry between, for example, animal and human disease need not imply a causal relation. As with this careful description of PSPr, the definition of a disease and assumptions about cause depend on an assessment of clinical and pathological phenotype, as well as PrP type. Laboratory transmission studies remain the final arbiter of the causal agent strain present, and epidemiology provides a rigorous test of whether a proposed cause is, in fact, plausible.

Robert Will, FRCP, and Mark Head, PhD National Creutzfeldt-Jakob Disease Surveillance Unit University of Edinburgh Edinburgh, United Kingdom

References

1. Gambetti P, Dong Z, Yuan J, et al. A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 2008. 2. Ladogana A, Puopolo M, Croes EA, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 2005;64:1586 –1591. 3. The EUROCJD Group. Genetic epidemiology of Creutzfeldt- Jakob disease in Europe. Rev Neurol (Paris) 2001;157: 633–637. 4. Stephenson DA, Chotti K, Ebeling C, et al. Quantitative trait loci affecting prion incubation time in mice. Genomics 2000; 69:47–53. 5. Lloyd SE, Onwuazor ON, Beck JA, et al. Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. Proc Natl Acad Sci U S A 2001;98: 6279–6283. 6. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994;35:513–529. 7. Lasmezas CI, Deslys J-P, Robain O, et al. Transmission of the BSE agent to mice in the absence of detectable abnormal prion protein. Science 1997;275:402– 405. 8. Manson JC, Jamieson E, Baybutt H, et al. A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy. EMBO J 1999;18:6855– 6864. 9. Yuan J, Xiao X, McGeehan J, et al. Insoluble aggregates and protease-resistant conformers of prion protein in unifected human brains. J Biol Chem 2006;46:34848 –34858. 10. Castilla J, Nonno R, Fernandez-Borgese N, et al. De novo generation of prions in a cell free system. Prion 2007;16 (Abstract, FC 7.4). 11. Piccardo P, Manson JC, King D, et al. Accumulation of prion protein in the brain that is not associated with transmissible disease. Proc Natl Acad Sci U S A 2007;104:4712– 4717. 12. Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variability is sporadic Creutzfeldt-Jakob disease. Ann Neurol. 1996;39:767–778. 13. Collinge J, Sidle KCL, Meads J, et al. Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 1996;383:685– 690. 14. Head MW, Tissingh G, Uitdehaag BMJ, et al. Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype. Ann Neurol 2001;50:258 –261. DOI: 10.1002/ana.21447 678 Annals of Neurology Vol 63 No 6 June 2008

http://www3.interscience.wiley.com/journal/119882940/abstract

http://www3.interscience.wiley.com/cgi-bin/fulltext/119882940/PDFSTART

IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???

lets look at the full circle, to date ;

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Discussion

The ten cases of CJD in this report are remarkable in that they have a specific neuropathological profile which, to our knowledge, has not been described previously[6,8] and which is so consistent that neuropathological samples from the cases are virtually indistinguishable. The cases are further characterised by having remarkably low ages at onset for CJD and other atypical features, including a generally protracted and unusual clinical course and absence of EEG changes typical of CJD. These findings raise the possibility that the cases represent a new clinicopathological variant of CJD.

http://www.cjd.ed.ac.uk/lancet.htm

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

http://brain.hastypastry.net/forums/showthread.php?t=15076

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

http://brain.hastypastry.net/forums/showthread.php?t=15076

http://brain.hastypastry.net/forums/archive/index.php/t-17057.html

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/novel-human-disease-with-abnormal-prion.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

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Conclusions

We believe that our observation of a previously unrecognised variant of CJD occurring, to date, only in persons under the age of 45 years is a cause for great concern. That it is due to exposure to the BSE agent is perhaps the most plausible interpretation of our findings. However, we emphasise that we do not have direct evidence of such a link and other explanations are possible. That these cases have been observed now because of improved ascertainment cannot be completely dismissed. It seems unlikely, however, that such a distinctive neuropathological pattern would have been missed previously, especially among persons dying at a young age. It is essential to obtain information on the clinical and neuropathological characteristics of young patients with CJD in Europe and elsewhere, and historically in the UK, but proof of an association between BSE and CJD may depend on animal transmission studies and continued epidemiological vigilance. If there is a causal link then, given the potentially long and widespread exposure to the BSE agent, further cases of this new variant of CJD are likely to arise.

We thank J Mackenzie for data management, P Brown for reviewing an early version of the manuscript, J Collinge for assistance with the molecular analysis, and W B Matthews who initiated CJD surveillance in the UK in the 1980 for advice. The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home and Health Department and suported by BBSRC (grant no 15/BS204814). The Concerted Action on CJD Surveillance in Europe was funded through the EC Biomed I Programme. The epidemiological surveillance of CJD would not be possible without the collaboration of neurologists and neuropathologists throughout the UK and Europe.

References

snip.....

http://www.cjd.ed.ac.uk/lancet.htm

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000054/!x-usc:mailto:r.g.will@ed.ac.uk.

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

snip...

Sporadic creutzfeldt-jakob disease in two adolescents

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

see full text sporadic CJD the big lie;

Subject: Sporadic creutzfeldt-jakob disease in two adolescents From: "Terry S. Singeltary Sr." <[log in to unmask]> Reply-To: Sustainable Agriculture Network Discussion Group <[log in to unmask]> Date: Mon, 28 May 2007 10:25:25 -0500 Content-Type: text/plain Parts/Attachments: text/plain (946 lines)

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: [log in to unmask]

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

http://www.bseinquiry.gov.uk/files/yb/1992/08/13002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21005001.pdf

CONFIRMED CJD IN FARMER WITH BSE COW

line to take, sporadic CJD

http://www.bseinquiry.gov.uk/files/yb/1992/10/22004001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05003001.pdf

SECOND CASE CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/00/00001001.pdf

CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE

ii. on page 2 the sentence ''He had drunk pooled milk from the herd which included that from the affected animal'' will mislead the uninformed. It needs to be made clear that milk from a cow which is suspected to be affected with BSE cannot be drunk or added to the bulk milk produced by the rest of the herd.

iii. in the final paragraph I suggest that the phrase ''and a causal link with BSE is at most conjectural'' BE DELETED: the first paragraph of the sentence would then stand as a clear statement that the CJD case was likely to have been a CHANCE PHENOMENON.

http://www.bseinquiry.gov.uk/files/yb/1993/02/15003001.pdf

''DH is aware of a second case of CJD in a dairy farmer who has had BSE in his herd. We cannot comment on the details of the case, but we know of nothing to suggest this is anthing other than a sporadic case of CJD. .........

http://www.bseinquiry.gov.uk/files/yb/1993/07/12001001.pdf

IF PRESSED:

The numbers concerned are very small, and it is not possible to draw any conclusions from such small numbers. This issue is being considered by the Government's expert advisers....

http://www.bseinquiry.gov.uk/files/yb/1993/07/12002001.pdf

THE FARMER IS THOUGHT TO HAVE HAD AT LEAST TWO CASES OF BSE IN HIS HERD, which were diagnosed in 1992. The farmer is reported to have asssisted in calving and to have drunk milk from his herd. This does not suggest that this is anything other than a sporadic case of CJD. ...

http://www.bseinquiry.gov.uk/files/yb/1993/07/12003001.pdf

CONFIDENTIAL

CONFIRMED CASE OF CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/07/14003001.pdf

3. Neither Dr Will nor the CJD surveillance unit intend to disclose the existence of this case or make any comment at present unless it attracts media attention.

snip...

HUMAN CASE DETAILS CONFIDENTIAL

snip...

6. CJD IN FARMERS

The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

BUTCHERS/ABATTOIR WORKERS/OCCUPATION INVOLVING DIRECT CONTACT WITH ANIMAL OR CARCASES 5

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

http://www.bseinquiry.gov.uk/files/yb/1993/07/19001001.pdf

Rocky Mountain oysters, mountain oysters, prairie oysters, Montana tendergroin or swinging sirloin

POLICY IN CONFIDENCE

1. The article in the Daily Mail of 12 August again raises the question of a CAUSATIVE LINK BETWEEN BSE AND CJD. This follows the death of a second farmer from CJD...

snip...

I am, however, concerned about how DH and MAFF would respont to public concern generated if there are further CJD cases among farmers.

snip...

4. Unwelcome, though it maybe to the Tyrrell Committee, I think they must be asked at their next meeting to give further thought to what they might advise the Department and MAFF if ANOTHER FARMER (or TWO) DEVELOPS CJD. OR, if a butcher or abattoir worker develops the disease.

5. Although the Committee were given plenty of advance warning about the second farmer, they may NOT BE SO FORTUNATE NEXT TIME ROUND. Some Contingency planning on the Committee's response to a further case of CJD in a farmer seems essential. At the same time the Committee should consider if there is SPECIAL RISK TO FARMERS, FOR EXAMPLE THEIR HISTORICAL HABIT OF CHEWING CATTLE NUTS, that might be implicated. .....(oh my GOD...tss)

http://www.bseinquiry.gov.uk/files/yb/1993/08/12002001.pdf

Ministers will note from this that experts are of the view, that there is unlikely to be a direct link between the cases of BSE, and the occurance of CJD in the farmer.

(NOTE CJD increasing over 3 years. ...TSS)

http://www.bseinquiry.gov.uk/files/yb/1993/08/18004001.pdf

'AGE AT ONSET' is therefore likely to be a reflection of particulary aetiological factors, about which, for sporadic CJD at least, much is yet unknown. IT has therefore been suggested that examination of the f/d i/p of other groups with TSE's, and comparison with that of CJD subsets might help to elucidate aetiological mechanisms for sporadic CJD in particular; i.e. ALMOST A REVERSAL OF THE ORIGINAL UNDERTAKING.

http://www.bseinquiry.gov.uk/files/yb/1993/08/26001001.pdf

OCCUPATIONAL EXPOSURE TO BSE AND CJD

2. The Tyrrell Committee met on 7 October and the significance of the two cases of CJD reported in dairy farmers who had BSE-affected animals on their farms was discussed at some length, AS WERE THE IMPLICATIONS OF A THIRD (OR FORTH) similar case.

3. The Committee were unable to identify any possible risk factors over and above those that they had already considered, both in general and with particular of TASTING THE FEED does continue but there was no consensus about the value of advising farmers to discontinue this practice. Feed currently in use does not pose a risk because of the ruminant-ruminant feed ban.

http://www.bseinquiry.gov.uk/files/yb/1993/10/11001001.pdf

MRC

STRAIN CHARACTERISATION OF THE CREUTZFELDT-JAKOB DISEASE AGENT BY TRANSMISSION TO MICE

In view of the CONCERN that exposue to BSE OR SCRAPIE MAY POSE A RISK TO HUMANS, it is proposed investigate the relationship between sporadic creutzfeldt-jakob disease.....

http://www.bseinquiry.gov.uk/files/yb/1993/10/12001001.pdf

3. While Committee may have no leads to pursue on why farmers might be at increased risk, I hope they understand the urgency with which they will need to respond if or when a THIRD FARMER DEVELOPS CJD.

http://www.bseinquiry.gov.uk/files/yb/1993/10/18001001.pdf

3RD FARMER BSE CJD TRANSMISSION STUDIES MRC

http://www.bseinquiry.gov.uk/files/yb/1995/08/01004001.pdf

THIS IS THE THIRD CASE OF CJD IN SOMEONE WORKING WITH A HERD OF DAIRY CATTLE IN WHICH BSE HAS BEEN CONFIRMED

snip...

the committee recognised that this is a CAUSE FOR CONCERN.

http://www.bseinquiry.gov.uk/files/yb/1995/01/00002001.pdf

IN CONFIDENCE

CJD IN TEENAGERS/WORLD IN ACTION PROGRAMME

1. DH have been informed of a case of sporadic CJD in a 19 year old male, the first in a teenager in the UK. This cases received some very limited press attention in May 1995 (see Annex A). ...

http://www.bseinquiry.gov.uk/files/yb/1995/08/01005001.pdf

IN CONFIDENCE

5. The current case, now confirmed histopathologically by the hospital in Bath, where he was treated, but not so far by the CJD Surveillance unit in Edinburgh, may renew the media interested generated by the 16 year old girl last year.

snip...

17. DH will discuss the need for more detailed briefing with MAFF as necessary. Also, by mid-August we will have an idea of the contents of the 1994 Annual Report from the CJD Surveillance Unit. The main issue arising from this is likely to be the increased numbers of sporadic cases in 1994 compared with 1993 (see para 6). The 1994 levels are comparable with 1992 figures, and this new rise may fuel the debate.

LINE TO TAKE

18. There is nothing to add to CMO's statement in January 1994, in relation to both the safety of meat and to the diagnosis in the 16 year old girl.

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/08/01006001.pdf

Government holds fast to UKBSEnvCJD only theory, and defies sound science. ...TSS

IN CONFIDENCE

CJD IN TEENAGERS

DATE: AUGUST 9, 1995

snip...

3. We are now aware of a further two cases of sporadic CJD in youngsters, reported in 1988 from Canada, and in 1930 (?from Europe), in a 16 year old and a 21 year old respectively. Although the Canadian case was born in the UK and was reported shortly after BSE appeared, it is highly improbable that a case with an onset in the mid-1980s could be linked to the emergence of BSE. The date of her emigration to Canada is not known. This case has, I understand, been picked up by ''World in Action''.

4. More importantly, a case of possible Sporadic CJD in a teenager in the UK has today been referred to the CJD Surveillance Unit. The 17 year old is still alive and not unusually, a brain biopsy has failed to confirm the diagnosis. The available history does not suggest the presence of any risk factors. The clinical picture is not typical, but could be consistent with a diagnosis of sporadic CJD, or with a number of other neurodegenerative conditions. PrP staining of the brain biopsy material may provide more information within the next few weeks (if the result is positive).

snip..

7. In view of patient confidentiality considerations, and of the potential sensitivity of this possible case, which has arisen so soon after the confirmed case in the 19 year old, I am not copying this widely. ...

snip...end

http://www.bseinquiry.gov.uk/files/yb/1995/08/09001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/08/01006001.pdf

(worried about the Vicky Rimmer case again i.e. sCJD in 16 year old girl. sporadic cjd now in farmers with BSE herds, and teenagers, UKBSEnvCJD only theory falling apart way back then. ...TSS)

http://www.bseinquiry.gov.uk/files/yb/1995/08/11002001.pdf

10. The CMO's advice remains as set out in a press statement on 26 January 1994:

''on the basis of the work done so far, there is no evidence whatever that BSE causes CJD and, similarly, not the slightest evidence that eating beef or hamburgers causes CJD.''

snip...

- a further case in a 16 year old girl occurred in Canada. The onset of disease was no later than 1986 (when the first case of BSE were being reported in the UK). Although the girl was born in the UK, this was long before BSE appeared, and there is no reason to make a connection with BSE.

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/08/11003001.pdf

MAD COW sporadic CJD COVER-UP IN FULL MODE NOW, no going back.

DID you notice how these two lines changed.

went from this ;

Although the Canadian case was born in the UK and was reported shortly after BSE appeared, it is highly improbable that a case with an onset in the mid-1980s could be linked to the emergence of BSE.

http://www.bseinquiry.gov.uk/files/yb/1995/08/09001001.pdf

too this ;

Although the girl was born in the UK, this was long before BSE appeared, and there is no reason to make a connection with BSE.

http://www.bseinquiry.gov.uk/files/yb/1995/08/11003001.pdf

The Today article mentions the fact that the 19 year old boy who died of CJD had had several holidays in the past on a dairy farm at Sissinghurst, Kent. I understand from Mr. Wilesmith that the dairy herd on this farm was disbanded in 1986 and up to that time had no recorded case of BSE. Because the EAR TAG numbers HAVE NOT been retained we are unable to confirm whether or not any of the cattle sold in the dispersal sale subsequently went on to develop BSE.

T E D EDDY

http://www.bseinquiry.gov.uk/files/yb/1995/08/14010001.pdf

IMPORTANT - CONFIDENTIAL

LINE TO TAKE

http://www.bseinquiry.gov.uk/files/yb/1995/08/17006001.pdf

CJD 17 YEAR OLD CONFIRMED

http://www.bseinquiry.gov.uk/files/yb/1995/08/22003001.pdf

TYPICAL CJD GRANDMA COOKED A COWS HEAD ABOUT 5 YEARS AGO, AND SHE ATE THE OCCASIONAL MACDONALDS HAMBURGER

http://www.bseinquiry.gov.uk/files/yb/1995/08/22005001.pdf

AND we in the USA wonder why no farmer or rancher out there wants COOL or any I.D.
the USA policy, they call it the 'TRIPLE SSS' policy, shoot, shovel and shut the hell up. ...TSS

BSE: MEAT WORKER WITH POSSIBLE CJD

http://www.bseinquiry.gov.uk/files/yb/1995/08/17003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/08/17004001.pdf

AMAZING what money can buy $$$

Report on brain biopsy (NOT SURE WHICH ONE...TSS)

http://www.bseinquiry.gov.uk/files/yb/1995/08/16005001.pdf

DATA Charmaine's HD:BSE - AUGUST 95: fill in bse position paper spec

FROM THE DIRECTOR GENERAL

STRICTLY PRIVATE AND CONFIDENTIAL

24 AUGUST 1995

NAME
COMPANY
LINE 1
LINE 2
LINE 3
LINE 4
LINE 5

Dear salutation

UKASTA POLICY ON BSE

At the President's suggestion n the light of recent events, I have reviewed the history of our policy on BSE so as to ensure that it full refects the needs of our supporters in the feed industry.

The Paper inclosed with this letter is the result. For obvious reasons, this is being circulated only to an extremely small circle within UKASTA - basically, the National Executive Council.

IF you have comments on the policy, or the paper, I should be glad to receive them UNDER PRIVATE & CONFIDENTIAL cover.

Yours sincerely,

J.W. REED

JWR/cg

copied to SMT members

STRICTLY PRIVATE AND CONFIDENTIAL

UKASTA INTERNAL POSITION STATEMENT
BOVINE SPONGIFORM ENCEPHALOPATHY

POLICY AIMS

POLICY AIMS

1. These have been consistent, although unstated except In FEC discussions, since at least 1989:-

• To minimize the risk of farmers' claims for compensation from feed compounders.

• To minimize the potential damage to compound feed markets through adverse publicity.

• To maximize freedom of action for feed compounders. notably by maintaining the availability of meat and bone meal as a raw material in animal feeds, and ensuring time is available to make any changes which may be required.

STRATEGY ADOPTED/SUCCESS ACHIEVED

2. Strategy has depended upon the situation at a particular time. UKASTA has sought to anticipate criticism from other industry sectors and action by Government/Brussels as the epidemic has developed and knowledge of the disease increased. Through dose liaison with MAFF. we have to date avoided public statements seriously damaging to the feed Industry and the adoption of policies likely to lead to such damage.

3. Successful examples of this strategy include:

• "Voluntary Ban" on SBO's In all MBM purchase contracts from November 1989. matching the Government ban on SBO'S in human food but anticipating the statutory ban on SBO's in feed which came in only from September 1990;

• Pressing Government for full compensation to farmers, which was finally conceded in February 1990;

• evidence (not Just on BSE) to the Lamming Committee in 1991/92 resulted in their recommending tighter controls over home mixers/integrated operations, and over the processing of fallen animals. Government eventually tightened the fallen animals legislation in December 1992. Other Lamming recommendations could yet be useful to us.

continued.....

95/8.24/2.2

2

• UKASTA pressure dissuaded MAFF from publicly linking voluntary ELISA tests of feed on farms with BAB's to (possibly compulsory) tests at compounders' premises in June/July 1994:

• in August 1995. while tightening the SBO Order and responding to the EU Decision requiring introduction of a testing programme. MAFF has accepted UKASTA proposals for the presentation of the changes to a wider audience, including farmers, and accepted our help in preparing for an EU Commission visit to inspect procedures and controls.

THE FUTURE

4. BSE has for more than seven years posed the greatest single potential threat to feed compounders' profitability. Although the epidemic is in sharp decline (275 cases per week compared to 1000 at the peak). MAFF remains under pressure in Brussels and is not skilled at handling potentially explosive issues.

5. Tests may show that ruminant feeds have been sold which contain illegal traces of ruminant protein. More likely, a few positive test results will turn up but proof that a particular feed mill knowingly supplied it to a particular farm will be difficult if not impossible.

6. The threat remains real and it will be some years before feed compounders are free of it. The longer we can avoid any direct linkage between feed milling practices and actual BSE cases, the more likely it is that serious damage can be avoided. In issue management terms, the aims and the strategy remain valid, but must be kept under review in the light of further events.

JWR/cg/23.8.95

95/8.24/2.3

SEE full text ;

http://www.bseinquiry.gov.uk/files/yb/1995/08/24002001.pdf

4TH CASE SPORADIC CJD IN A FARMER WITH BSE HERD

http://www.bseinquiry.gov.uk/files/yb/1995/09/28001001.pdf

TO: DR. AILSA WIGHT
FROM: DR. R. WILL
DATE: 26TH SEPTEMBER 1995

snip...

As I explained, we have doing some analysis on family history and mutations of the PrP gene for an American company in relation to proposed guidelines for blood donation in the USA. ...

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/09/26001001.pdf

Saturday, December 08, 2007

Transfusion Transmission of Human Prion Diseases

http://vcjdblood.blogspot.com/2006/12/vcjd-case-study-highlights-blood.html

Thursday, July 24, 2008

Prion diseases are efficiently transmitted by blood transfusion in sheep

Submitted April 18, 2008 Accepted June 28, 2008

http://vcjdblood.blogspot.com/2008/07/prion-diseases-are-efficiently.html

RESTRICTED

FOURTH CASE OF CJD IN FARMER

snip...

4. When considering the second case of CJD in a dairy farmer in 1993 the Spongiform Encephalopathy Advisor Committee (SEAC) was told by Professor Smith (London School of Hygine and Tropical Medicine) that if four cases of CJD occurred in farmers over a five year period then ''THE POSSIBILITY THAT THE ASSOCIATION WAS NOT DUE TO CHANCE HAD TO BE GIVEN VERY SERIOUS CONSIDERATION''. The Department of Health is, therefore, convening a special meeting of the SEAC early next week to advise on the response to this possible case of CJD. I will report the outcome of this meeting to the Minister urgently.

snip...

2. SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD WOULD HAVE HAD BSE CASES ON THEIR FARMS. It was difficult to calculate accurately the likelihood of this being due to a series of random events; but looking at all male farmers and farm workers in England and Wales, the chance of four CJD cases occurring randomly since 1990 was around 5/100; the chances of fourth cases of CJD occurring randomly in farmers with BSE in their herds was very much lower, around 3/10,000. The Committee therefore concluded that IT WAS DIFFICULT TO EXPLAIN THE INCIDENCE AS A CHANCE PHENOMENON. This is a change in the Committee's position; it had said that the most likely explanation of the three previous cases of CJD in dairy farmers was that they were chance phenomena. ...

snip...

full text ;

http://www.bseinquiry.gov.uk/files/yb/1995/09/28002001.pdf

CJD IN FARMERS

http://www.bseinquiry.gov.uk/files/yb/1995/09/28003001.pdf

4TH ANNUAL CJD REPORT

snip...

7. The Final conclusion of the report is that:

"The incidence of CJD in the UK has risen significantly since 1990. Comparison with the incidence in other countries suggests that this rise in incidence is most likely to be related to increased ascertainment of cases. Other analyses....do not provide any conclusive evidence of a change in CJD that can be attributable to BSE. The identification of CJD in three dairy farmers with a potential occupational exposure to BSE and the occurrence of CJD in a teenager reinforces the importance of continuing careful surveillance of CJD with particular reference to occupation risk and age incidence.''

http://www.bseinquiry.gov.uk/files/yb/1995/09/29001001.pdf

CJD FOURTH FARMER LINE TO TAKE, preparing for media storm ;

http://www.bseinquiry.gov.uk/files/yb/1995/09/29009001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/09/29013001.pdf

24. Dr. Kimberlin said that the statistics were getting worse and worse. It was not possible to get a handle on any possible link with BSE. There was clearly something going on because of the rate of CJD in farmers in the UK and in other European countries were the same. He pointed out that, ACROSS EUROPE, DAIRY FARMERS SEEMED TO HAVE A HIGHER RISK OF CJD THAN OTHERS.

25. Professor Pattison agreed that all four cases in farmers should be included in the transmission studies, and said that IF A LINE HAD TO BE DRAWN this should be done LATER. Dr. Watson agreed that the transmission studies were crucial.

snip...

Notes on calculations re cases of CJD in farm workers

Numerators

Calculations are based on a total of 5 cases of CJD. Four of these cases were in men. All four worked on farms with cattle (3 dairy, 1 beef suckler). All four worked on farms with confirmed cases of BSE. The fifth case was a woman who worked on farms with (dairy) cattle. No cases of BSE were reported. ...

snip...

full text ;

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

RESTRICTED-POLICY
CJD IN CATTLE FARMERS

From: D Matthews SVO
Date: 10 October 1995

snip...

8. A much higher theoretical risk by accidental ingestion or inhalation might be attributed to the use of SBO derived fertiliser. We have no data on sales of SBO derived meat and bone meal to go on, BUT until November 1991 it was permissible for such material to be sold and used as fertiliser.

snip...

Conclusions

9. All of the above are options that we are still not in a position to evaluate because we have insufficient information about the herds and risks concerned. IN ADDITION, Mr. Bradley has identified one other possible explanation, namely that there are sub-strains of BSE which present lesser or greater risk to man. It is most unlikely that we shall have brain material available from cases on these farms to test such a hypothesis. The planned transmission experiments with the brains from the farmer cases may however help, particularly if strain type appears to be different from both classical CJD and known BSE strains.

http://www.bseinquiry.gov.uk/files/yb/1995/10/10004001.pdf

This potential 4th case is significant because it brings the UK incidence of CJD in farmers to around 2 cases per million population per year, compared to the average of around 0.9 cases per million.

http://www.bseinquiry.gov.uk/files/yb/1995/10/23007001.pdf

LINE TO TAKE ;

http://www.bseinquiry.gov.uk/files/yb/1995/10/23010001.pdf

RESTRICTED - POLICY

CJD IN ADOLESCENTS

snip...

3. The first case is that of CJD in a 19 year old boy. This is already publicly known and was the subject of a ''World In Action'' programme over the summer. The second case is in a 17 year old girl, and is the one I reported to the Minister in my minute of 22 September. This patient is still alive, but CJD has been confirmed by brain biopsy. This will be the first time in which this case had been made public.

95/10.25/6.1

T E D EDDY

http://www.bseinquiry.gov.uk/files/yb/1995/10/25006001.pdf

To: Dr. J Ironside
From: Dr. R Will

1 September 1995

The crucial issue in this case is whether the pathological changes as reported are really atypical for sporadic CJD.

http://www.bseinquiry.gov.uk/files/yb/1995/09/01005001.pdf

RESTRICTED - POLICY

BSE

snip...

(iv) the fact that four farmers all with BSE in their herds had now contracted CJD. The chances of ths occurring naturally were very small indeed;

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/10/25015001.pdf

SEAC STATEMENT CJD FARMERS

http://www.bseinquiry.gov.uk/files/yb/1995/10/00003001.pdf

RESTRICTED
BSE AND CJD: LATEST DEVELOPMENTS
FROM: T J RENDER

26 October 1995

snip...

2. We have had reports of the first suspect case of a spongiform encephalopathy in a tiger.

snip...

Details of the possible case in the tiger are not yet publicly known.

snip...

3. We also learnt from DH that the suspected 4th farmer with CJD DIED YESTERDAY. The post mortem will be carried out by the CJD Surveillance Unit in Edinburgh.

4. DH also learnt today of a case of CJD IN A 28 YEAR OLD MAN. This has been confirmed by brain biopsy, although the man is still alive. It is unusual to see sporadic CJD in someone so young; apart from the two adolescents the Minister is aware of, the previous youngest sporadic CJD sufferer in the UK was 34 years old. Details of this case are NOT publicly known.

T J RENDER

http://www.bseinquiry.gov.uk/files/yb/1995/10/26001001.pdf

IN CONFIDENCE

CJD IN YOUNG PEOPLE

* in the USA, a 16 year old in 1978

* in France, a 19 year old in 1982

* in Canada, a 14 year old of UK origin in 1988

* in Poland, cases in people aged 19, 23 and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

* Creutzfeldt's first patient in 1920 was aged 23

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/10/26005001.pdf

4th farmer dies

ADVICE

http://www.bseinquiry.gov.uk/files/yb/1995/10/27010001.pdf

CASES OF SUSPECTED SPORADIC CJD IN YOUNG PEOPLE NOTIFIED TO CJD SURVIELLANCE UNIT IN 1995

http://www.bseinquiry.gov.uk/files/yb/1995/10/31006001.pdf

FOURTH CASE OF CJD IN FARMER ''CONFIRMED''

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

INCREASE IN SPORADIC CJD

http://www.bseinquiry.gov.uk/files/yb/1993/11/11001001.pdf

occupational

http://www.bseinquiry.gov.uk/files/yb/1994/02/16001001.pdf

Dealler gets ''dixie chicked' again ;

http://www.bseinquiry.gov.uk/files/yb/1993/11/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/08003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/10006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/14003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/16006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/17003001.pdf

STACKING THE DECK AGAINST SPORADIC CJD AND SOUND SCIENCE

APPOINTMENTS IN CONFIDENCE

MEMBERSHIP TO SEAC

snip...

I have informed Dr Tyrrell that we have now written to Dr Hueston to invite him to serve on the Committee and he was very pleased to hear this. He was also in favour of our idea of having a deputy Chairman who could take any emergency meetings eg IF THERE WERE TO BE ANOTHER CJD CASE IN AGRICULTURE. I suggested that either Dr. WIll or Dr Kimberlin were likely candidates and he thought that this was about right. He felt that on balance he would prefer Dr Will who he thought took a more cautious line and was LESS DOGMATIC. .....

http://www.bseinquiry.gov.uk/files/yb/1993/12/01003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/00005001.pdf

CHANGING SCIENCE TO FIT YOUR INDUSTRY NEEDS COVER-UP IN FULL MODE NOW

PROBLEM

7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x).

IP PS(L) wishes to probe this further we think it best to explain the matter VERBALLY. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stores. (or the facts...TSS)

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

A REVISED VERSION WHERE THE FOLLOWING WAS MADE TO BE REMOVED FROM SCIENTIFIC FINDINGS. ...TSS

''This year's findings show a number of associations but the strongest is for veal.''

A BIG LINE WAS DRAWN THROUGH THAT SENTENCE TO BE REMOVED DUE TO THE FOLLOWING. THIS IS THE NEXT SENTENCE ;

''This is of considerable concern given recent developments. In particular, Ministers will be particularly concerned about the European dimension given the recent troubles with the Germans.''

YOU can see the beginning of the ukbsenvCJD only theory beginning to unfold now. full text of this ukbsenvcjd only conspiracy can be seen here. ...TSS

POLICY RESTRICTED

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

BRITISH DEER FARMERS ASSOCIATION

OCTOBER 1994

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is completely independent outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

snip...

The statistical results regarding the consumption of venison was put into perspective in the body of the report and was NOT MENTIONED AT ALL IN THE PRESS RELEASE. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of VENISON was highlighted only by the media i.e. in the News at one television programme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of Venison, would increase, and quite possibly GIVE DAMAGING CREDENCE, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

see buttered and watered down report here that caters to industry instead of human safety...TSS

http://www.bseinquiry.gov.uk/files/yb/1994/10/00004001.pdf

SEE WHERE THIS ;

''This year's findings show a number of associations but the strongest is for veal.''

WENT TO THIS;

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and increased risk of CJD. When some account was taken of possible confounding, the association between veal eating and risk of CJD emerged as the strongest of these associations statistically. These findings concerning dietary history are particulary difficult to interpret for two reasons.

1. .........BSeee...........TSS

2. .........BSeee...........TSS

(I.E. BSeee = bull sh!t encephalopathy or government cover-up i.e. God save the industry at all cost, including human health. ...TSS)

THUS, the reported veal eating habits of confirmed CJD cases appear virtually identical to suspect cases later judged not to have the disease. This provides good circumstantial evidence to support the hypothesis that the apparent association between veal consumption and CJD is due to recall bias. Analysis of other apparent dietary risk factors for CJD, including venison, has provided similar evidence of recall bias.

snip...

In summary, the analysis of the dietary case-control study demonstrates a strong association between a lifetime history of veal consumption and the risk of developing Creutzfeldt-Jakob disease. HOWEVER this result may well be due to recall bias and analysis of clinical and molecular bilogical features does not provide supportive evidence for the hypothesis that veal eating is a risk factor for CJD. ...

snip...

MORE OF THIS BSeee CAN BE READ IN THE FINAL GOVERNMENT DICTATED CJD REPORT OF 1994

http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

BSE SCIENTIST WAS 'CENSORED'

He says that when he worked at MAFF, ''the way it was structurally set up was not that science would drive the politics, but that the politics will drive the science. And that's wrong.''

http://www.bseinquiry.gov.uk/files/yb/1997/12/11001001.pdf

11/3/96 DGRC alerts DTI Ministers and CSA to discovery of nvCJD on basis of letter from MRC Chief Executive dated 11/3/96

BIRTH OF THE UKBSEnvCJD ONLY THEORY, the birth of the 'BIG LIE' begins. ...tss

http://www.bseinquiry.gov.uk/files/db/do01/tab03.pdf

REPORT OF 16 YEAR OLD GIRL WITH CJD

5. This case may raise fears of a link between BSE and CJD. Current advice is that there is no scientific evidence of a link between BSE in cattle and CJD in humans. Furthermore advice from the Spongiform Encephalopathy Advisory Committee is that they are satisified that all necessary safeguards are in place to minimise further spread of spongiform encephalopathies in animals and to prevent any risk of transmission to humans. ...

http://www.bseinquiry.gov.uk/files/yb/1994/01/14005001.pdf

To ask the Secretary of State for Health, how many people under the age of 20 years in each of the last five years have suffered from Creutzfeldt-Jakob disease; and of these how many had not had any growth treatment previously.

SUGGESTED REPLY

We are not aware of any people under the age of 20 in the UK suffering from Creutzfeldt-Jakob Disease in the last five years.

http://www.bseinquiry.gov.uk/files/yb/1994/01/20001001.pdf

STATEMENT FROM HOSPITAL

http://www.bseinquiry.gov.uk/files/yb/1994/01/20005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25001001.pdf

PREPARING FOR THE STORM 'LINE TO TAKE'

http://www.bseinquiry.gov.uk/files/yb/1994/01/25003001.pdf

BARONESS CUMBERLEGE TRYING TO MANIPULATE THE MEDIA

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

MAD COW MEAL DESTROYED MY DAUGHTERS LIFE

A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating a contaminated burger it was claimed last night.

VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).

http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf

GIVE ME BACK MY LIFE

http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf

HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''

http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf

WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY

http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf

I have interviewed Mrs Rimmer at my constituency surgery

IF there is nothing to hide, why is there so much SECRECY? WHY is the Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are taken, surely the problem could be contained, however, as it stands the lack of investigation and interest of the possibility of B.S.E. and C.J.D. being linked is open for speculation and surely someone has to account for peoples lives! WHY is so much trouble being taken to convice people that B.S.E. and C.J.D. are not linked? Guilty Conscience perhaps ? - or cover up?

HOUSE OF COMMONS

FROM BARRY JONES, M.P.

22 FEBRUARY 1994

http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

(now story changes that biopsy shows she does not have CJD...tss)

http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf

now story changes to ;

Advice

7. The Parliamentary Secretary is invited to note the recent statements made on __________ and the present position which remains that CJD cannot be confirmed, in this case at this stage.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf

3. The Medical Director at ___________________ Hospital advised the Department on 6 June that the results of ___________________ brain biopsy had been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital subsequently issued a statement to the press to this effect and this was publicised widely in the press (doc 1). News coverage which followed suggested that the statement made by ________________ Hospital had been misleading (doc 2). Enquires have been made of the Medical Director at _______________ Hospital who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR. The facts are that two pathology reports on the same piece of brain tissue were recieved. The first report indicated that CJD was unlikely, The second report indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive diagnosis could be made before a post mortem was undertaken.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf

(ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM SPORADIC CJD, the same damn thing. ...TSS)

IN light of Asante/Collinge et al findings 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;

-------- Original Message --------

Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To: "[log in to unmask]"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[log in to unmask] (until 9/12/02)

New e-mail: [log in to unmask] (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

WHAT ABOUT U.S.A. ???

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf

20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

NOW BACK TO THOSE FARMERS WITH BSE HERDS THAT DIED FROM SPORADIC CJD

CJD FARMERS WIFE 1989

http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

cover-up of 4th farm worker ???

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf

CONFIRMATION OF CJD IN FOURTH FARMER

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf

4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf

2. snip... Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...

http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

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

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

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

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 2007

http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html

http://nor-98.blogspot.com/

SCRAPIE USA

http://scrapie-usa.blogspot.com/

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

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

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:

[log in to unmask]

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

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texsas USA 77518

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

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

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

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

Greetings,

SOMETHING ELSE TO PONDER HERE, lets go back and look and the other potential cases of CWD transmission to humans and where it was explained away as genetic too i.e. GSS ;

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ˜22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Table 2. Creutzfeldt-Jakob disease patients investigated for a possible causal link of their illness with chronic wasting disease of deer and elk, United States a

LOOK AT 1 AND 3B BOTH GSS. ...TSS

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm#table2

Monday, June 30, 2008

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

please see additional comments and other studies ;

http://chronic-wasting-disease.blogspot.com/2008/06/risk-behaviors-in-rural-community-with.html

http://organicconsumers.org/forum/index.php?showtopic=1659

CWD

http://chronic-wasting-disease.blogspot.com/

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008

2008-07-10T10:55:00.000-07:00

Friday, June 20, 2008

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

Original Article

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/novel-human-disease-with-abnormal-prion.html

UPDATE ;

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease

Pierluigi Gambetti, MD,1 Zhiqian Dong, PhD,1 Jue Yuan, BA,1 Xiangzhu Xiao, PhD,1 Mengjie Zheng, PhD,1 Amer Alshekhlee, MD,1 Rudy Castellani, MD,2 Mark Cohen, MD,1 Marcelo A. Barria, PhD,3 D. Gonzalez-Romero, PhD,3 Ermias D. Belay, MD,4 Lawrence B. Schonberger, MD, MPH,4 Karen Marder, MD,5 Carrie Harris, BA,1 James R. Burke, MD, PhD,6 Thomas Montine, MD,7 Thomas Wisniewski, MD,8 Dennis W. Dickson, MD,9 Claudio Soto, PhD,3 Christine M. Hulette, MD,10 James A. Mastrianni, MD, PhD,11 Qingzhong Kong, PhD,1 and Wen-Quan Zou, MD, PhD1

Objective: To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) that, contrary to the common prion diseases, is predominantly sensitive to protease digestion.

Methods: Eleven subjects were investigated at the National Prion Disease Pathology Surveillance Center for clinical, histopathological, immunohistochemical, genotypical, and PrP characteristics.

Results: Patients presented with behavioral and psychiatric manifestations on average at 62 years, whereas mean disease duration was 20 months. The type of spongiform degeneration, the PrP immunostaining pattern, and the presence of microplaques distinguished these cases from those with known prion diseases. Typical protease-resistant PrP was undetectable in the cerebral neocortex with standard diagnostic procedures. After enrichment, abnormal PrP was detected at concentrations 16 times lower than common prion diseases; it included nearly 4 times less protease-resistant PrP, which formed a distinct electrophoretic profile. The subjects examined comprised about 3% of sporadic cases evaluated by the National Prion Disease Pathology Surveillance Center. Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.

Interpretation: The distinct histopathological, PrP immunohistochemical, and physicochemical features, together with the homogeneous genotype, indicate that this is a previously unidentified type of disease involving the PrP, which we designated “protease-sensitive prionopathy” (or PSPr). Protease-sensitive prionopathy is not rare among prion diseases, and it may be even more prevalent than our data indicate because protease-sensitive prionopathy cases are likely also to be classified within the group of non-Alzheimer’s dementias. Ann Neurol 2008;63:697–708

snip...

Discussion

We report 11 patients affected by a disease that involves abnormal PrP and has homogeneous and distinctive features (Table 2). Based on several lines of evidence, we argue that these features allow for the separation of this condition from all known forms of human prion disease. First, the abnormal PrP associated with this disease is predominantly, and in several brain regions almost exclusively, sensitive to protease or PrPs, and the PK-resistant PrP isoform or PrPr has a distinctive electrophoretic profile. The high sensitivity to PK and the distinctive electrophoretic profile of the abnormal PrP clearly distinguish these cases from each of the five subtypes of sCJD and from sporadic fatal insomnia (sFI), the known human sporadic prion diseases.1 For example, compared with sCJDMM1, the most common and typical sCJD,2 these cases have 16 times less total abnormal PrP, and the fraction of the total abnormal PrP that is PK resistant is nearly 4 times less. Furthermore, the ladder-like electrophoretic profile of the PrPr associated with this condition has not been observed in either sCJD or sFI, all of which instead are characterized by the presence of the well-known PrPr type 1 or 2.1 When present, the traditional PrPr, commonly called PrP27-30, was located in subcortical regions and was of type 1, another combination not observed in sporadic human prion diseases.1 Second, these cases are also homogeneous as for the PrP coding genotype because they are all homozygous for valine at codon 129 of the PrP gene, the site of a common methionine/ valine polymorphism.28 Valine homozygosity in white individuals is the rarest 129 genotype, being found only in 12% of people.28 The sCJD subtypes associated with valine homozygosity, sCJDVV1 and sCJDVV2, have been well characterized and differ from these cases phenotypically and for the characteristics of the abnormal PrP.1 Third, the pattern of PrP immunostaining and the presence of structures with the features of poorly formed plaques that we observed in the cerebellum are to our knowledge unprecedented. Lastly, the clinical presentation and initial course that prominently features relatively slow cognitive deterioration, occasional gait impairment, and incontinence has evoked the diagnoses of normal pressure hydrocepha-lus, diffuse Lewy body disease, or frontotemporal dementia, whereas prion disease was suspected only at a later stage based on the relatively short duration.

Although these cases can be easily distinguished from sporadic prion diseases, some of their features such as overrepresentation of PrPs and the multiple PKresistant PrP fragments, have been reported in GSS.4 However, all cases of GSS reported to date are associated with a mutation in the coding region of the PrP gene or immediately adjacent to it.4 None of these cases carried such mutation. Moreover, the ladder-like, PK-resistant, PrP fragments observed in our cases are preferentially detected with 1E4 but not with 3F4, which obviously separates these cases from GSS carrying the multiple PK-resistant PrP fragments. In a recent study, we observed that although 1E4 and 3F4 have adjacent epitopes along human PrP residues 97- 112, their accessibility to these epitopes is different because of different neighboring N-terminal residues.29 It is possible that the 1E4 selectively detected PKresistant PrP fragments have N-terminal starting sites that are different from those of the well-characterized PrPr types 1 and 2. The earlier evidence clearly indicates that this condition differs from GSS, although the possibility that it represents the long-sought sporadic form of GSS remains to be excluded. Six of the 10 patients with obtainable pedigree had a family history of dementia that cannot be ignored, yet none carried a mutation in the PrP gene ORF. Therefore, at least in some cases, a causative mutation may be located outside the ORF of the PrP gene, a condition never observed in human prion diseases.1

All these considerations argue that the 11 patients were affected by a novel condition involving the PrP that cannot be classified within the spectrum of currently known human prion diseases. We suggest the designation of PSPr to emphasize a major distinctive feature (see Table 2).

Compared with other human prion diseases, PSPr is not exceedingly rare, because it accounts for about 3% of all sCJD and 16% of all valine homozygous CJD accessioned by the National Prion Disease Pathology Surveillance Center during the same time period as these 11 patients, making PSPr about as common as some of the well-known sporadic prion diseases (such as sCJDMM2, sFI, and sCJDVV1).2 Furthermore, because the clinical presentation and the duration of PSPr often do not point to the diagnosis of prion disease, some cases of PSPr may currently be classified within the group of non-Alzheimer’s dementias and not be investigated further. Should this be the case, PSPr may be more common than this study suggests.

The small amount of PrPr associated with PSPr and the finding that about 76% of the detectable abnormal PrP is PK sensitive not only hinders the diagnosis but also has implications concerning origin, pathogenicity, infectivity, and classification of PSPr.

The discovery of PrPs has opened a new chapter in prion diseases.11–15 The demonstration that PrPs forms smaller aggregates than the PrPr counterpart,16 and that apparently it is competent to convert PrPC to PrPr in vitro, as well as to seed the polymerization of recombinant PrP into amyloid,17,18 suggests that PrPs shares defining features with PrPr. However, the pathogenetic mechanisms of PrPs in the absence of PrPr and, therefore, the nature of the prion diseases associated with PrPs currently remain conjectural.

Prion diseases associated with PrPs, in the presence of minimal or no PrPr, have been modeled and studied in detail in a variety of transgenic (Tg) mouse lines carrying mouse homologues of human PrP gene mutants or overexpressing PrPC.12,30–33 Two Tg mouse models appear relevant to these cases.

In the first model, Tg mice expressing high levels of mouse PrP carrying the P101L mutation, the mouse equivalent of the human P102L mutation associated with a GSS phenotype,4,34,35 spontaneously developed a neurodegenerative process characterized by SD and prion plaque formation. After inoculation, they transmitted a disease phenotypically similar to P101Lmutated Tg mice but not to wild-type mice. As in our cases, the affected mice had PrPs but no, or minimal amounts of, PrPr, indicating that PrPs can be associated with a prion disease that is under certain condi-tions transmissible and has a histopathological phenotype displaying general features of prion diseases.12

In the second model, Tg mice carrying the P101L mutation were inoculated with brain homogenate from patients affected by a subtype of GSS P102L characterized by the exclusive presence of an approximately 8kDa PK-resistant fragment reminiscent of the approximately 6kDa fragment observed in small amounts in our cases. The inoculated Tg mice remained largely asymptomatic, but at histological examination, they displayed PrP plaques and had minimal amounts of PrPr.33 They failed to transmit the disease to wild-type mice, but inoculation to P101L-mutated mice resulted in the formation of PrP plaques in the absence of clinical disease.

These mouse models and now our cases raise issues with the definition of prion diseases. Currently, it is unclear whether PSPr is transmissible because timeconsuming transmissibility experiments to different lines of Tg mice and in vitro PrP replication are still ongoing. Should PSPr not be transmissible, the question is whether it is a prion disease. A similar question can be raised for GSS, of which to date only one subtype has been shown to be consistently transmissible. 4 The issue is further compounded by the recent evidence that amyloid , the pathogenic peptide of Alzheimer’s disease, has the propensity to replicate after inoculation into susceptible Tg mice in a conformation-dependent fashion reminiscent of prions. 36 These findings appear to blur the once tight association of prion diseases and transmissibility. It may be more practical to apply the label of prion diseases to all conditions in which the PrP is abnormal and appears to play a central role in the pathology, as in all prion diseases known to date and in PSPr.37 In contrast, one might reserve the qualification of transmissible to those prion diseases that can be transmitted to recipients expressing relatively normal amounts of wild-type PrP.36

The finding that several PSPr patients had firstdegree relatives diagnosed with dementia necessitates a search for an underlying genetic cause. In AD, the discovery of mutations outside the gene of the amyloid precursor protein (the central protein in AD, as PrP is in prion diseases) has provided a wealth of information regarding pathogenetic mechanisms of AD.38 Similarly, the discovery of a mutation outside the PrP gene ORF capable of generating a prion disease may greatly expand our understanding of pathogenetic mechanisms and the role of PrP in prion diseases. Supported by the NIH grants AG14359 and AG08702, Centers for Disease Control and Prevention (CCU 515004), and the Britton Fund to P.G.; NIH Grant NS049173 to C.S.; and the CJD Foundation to W.Q.Z. Drs J. McGeehan and G. Kneale kindly provided g5p. Drs J. Hedreen, L. S. Honig, C. S. Calder, L. P. Goldstick, and W. Longstreth helped in obtaining the cases. P. Scalzo and D. Kofskey provided skillful histological and immunohistochemical preparations. B. Chakraborty assisted in the preparation of the manuscript and illustrations.

References...snip..end...TSS

http://www3.interscience.wiley.com/journal/119883040/abstract?CRETRY=1&SRETRY=0

http://www3.interscience.wiley.com/cgi-bin/fulltext/119883040/PDFSTART

GEE thanks, besides mom dying from hvCJD (Gambetti et al have her brain tissue), my mema, and her son (moms mom, and brother {MY UNCLE}), both had/have moderate Alzheimer's. wonder what i have to look forward too ??? hmm, also, another sporadic TSS i.e. sporadic GSS, i remember the sporadic FFI ??? what's all this leading too ???

ALSO, it seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???

lets look at the full circle, to date ;

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Discussion

The ten cases of CJD in this report are remarkable in that they have a specific neuropathological profile which, to our knowledge, has not been described previously[6,8] and which is so consistent that neuropathological samples from the cases are virtually indistinguishable. The cases are further characterised by having remarkably low ages at onset for CJD and other atypical features, including a generally protracted and unusual clinical course and absence of EEG changes typical of CJD. These findings raise the possibility that the cases represent a new clinicopathological variant of CJD.

http://www.cjd.ed.ac.uk/lancet.htm

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

http://brain.hastypastry.net/forums/showthread.php?t=15076

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

http://brain.hastypastry.net/forums/showthread.php?t=15076

http://brain.hastypastry.net/forums/archive/index.php/t-17057.html

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/novel-human-disease-with-abnormal-prion.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

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Conclusions

We believe that our observation of a previously unrecognised variant of CJD occurring, to date, only in persons under the age of 45 years is a cause for great concern. That it is due to exposure to the BSE agent is perhaps the most plausible interpretation of our findings. However, we emphasise that we do not have direct evidence of such a link and other explanations are possible. That these cases have been observed now because of improved ascertainment cannot be completely dismissed. It seems unlikely, however, that such a distinctive neuropathological pattern would have been missed previously, especially among persons dying at a young age. It is essential to obtain information on the clinical and neuropathological characteristics of young patients with CJD in Europe and elsewhere, and historically in the UK, but proof of an association between BSE and CJD may depend on animal transmission studies and continued epidemiological vigilance. If there is a causal link then, given the potentially long and widespread exposure to the BSE agent, further cases of this new variant of CJD are likely to arise.

We thank J Mackenzie for data management, P Brown for reviewing an early version of the manuscript, J Collinge for assistance with the molecular analysis, and W B Matthews who initiated CJD surveillance in the UK in the 1980 for advice. The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home and Health Department and suported by BBSRC (grant no 15/BS204814). The Concerted Action on CJD Surveillance in Europe was funded through the EC Biomed I Programme. The epidemiological surveillance of CJD would not be possible without the collaboration of neurologists and neuropathologists throughout the UK and Europe.

References

snip.....

http://www.cjd.ed.ac.uk/lancet.htm

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000063/!x-usc:mailto:r.g.will@ed.ac.uk.

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

snip...

Sporadic creutzfeldt-jakob disease in two adolescents

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

see full text sporadic CJD the big lie;

Subject: Sporadic creutzfeldt-jakob disease in two adolescents From: "Terry S. Singeltary Sr." <[log in to unmask]> Reply-To: Sustainable Agriculture Network Discussion Group <[log in to unmask]> Date: Mon, 28 May 2007 10:25:25 -0500 Content-Type: text/plain Parts/Attachments: text/plain (946 lines)

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: [log in to unmask]

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

http://web.archive.org/web/20031216142635/http://www.bseinquiry.gov.uk/files/yb/1992/08/13002001.pdf

http://web.archive.org/web/20031217070430/http://www.bseinquiry.gov.uk/files/yb/1992/08/21002001.pdf

http://web.archive.org/web/20040315035806/http://www.bseinquiry.gov.uk/files/yb/1992/08/21005001.pdf

CONFIRMED CJD IN FARMER WITH BSE COW

line to take, sporadic CJD

http://web.archive.org/web/20031122055743/http://www.bseinquiry.gov.uk/files/yb/1992/10/22004001.pdf

http://web.archive.org/web/20030822195516/http://www.bseinquiry.gov.uk/files/yb/1992/10/22005001.pdf

http://web.archive.org/web/20040315053517/http://www.bseinquiry.gov.uk/files/yb/1992/10/22001001.pdf

http://collections.europarchive.org/tna/20080102232911/http://www.bseinquiry.gov.uk/files/yb/1992/11/05002001.pdf

http://web.archive.org/web/20030726144123/http://www.bseinquiry.gov.uk/files/yb/1992/11/05003001.pdf

SECOND CASE CJD IN DAIRY FARMER

http://collections.europarchive.org/tna/20080103041742/http://www.bseinquiry.gov.uk/files/yb/1993/00/00001001.pdf

CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE

ii. on page 2 the sentence ''He had drunk pooled milk from the herd which included that from the affected animal'' will mislead the uninformed. It needs to be made clear that milk from a cow which is suspected to be affected with BSE cannot be drunk or added to the bulk milk produced by the rest of the herd.

iii. in the final paragraph I suggest that the phrase ''and a causal link with BSE is at most conjectural'' BE DELETED: the first paragraph of the sentence would then stand as a clear statement that the CJD case was likely to have been a CHANCE PHENOMENON.

http://web.archive.org/web/20030513214508/http://www.bseinquiry.gov.uk/files/yb/1993/02/15003001.pdf

''DH is aware of a second case of CJD in a dairy farmer who has had BSE in his herd. We cannot comment on the details of the case, but we know of nothing to suggest this is anthing other than a sporadic case of CJD. .........

http://collections.europarchive.org/tna/20080102155112/http://www.bseinquiry.gov.uk/files/yb/1993/07/12001001.pdf

IN CONFIDENCE

SECOND CASE OF CJD IN DAIRY-FARMER

IF PRESSED:

The numbers concerned are very small, and it is not possible to draw any conclusions from such small numbers. This issue is being considered by the Government's expert advisers....

http://collections.europarchive.org/tna/20080103034651/http://www.bseinquiry.gov.uk/files/yb/1993/07/12002001.pdf

THE FARMER IS THOUGHT TO HAVE HAD AT LEAST TWO CASES OF BSE IN HIS HERD, which were diagnosed in 1992. The farmer is reported to have asssisted in calving and to have drunk milk from his herd. This does not suggest that this is anything other than a sporadic case of CJD. ...

http://collections.europarchive.org/tna/20080103034528/http://www.bseinquiry.gov.uk/files/yb/1993/07/12003001.pdf

CONFIDENTIAL

CONFIRMED CASE OF CJD IN DAIRY FARMER

http://web.archive.org/web/20030513115653/http://www.bseinquiry.gov.uk/files/yb/1993/07/14003001.pdf

3. Neither Dr Will nor the CJD surveillance unit intend to disclose the existence of this case or make any comment at present unless it attracts media attention.

snip...

HUMAN CASE DETAILS CONFIDENTIAL

snip...

6. CJD IN FARMERS

The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

BUTCHERS/ABATTOIR WORKERS/OCCUPATION INVOLVING DIRECT CONTACT WITH ANIMAL OR CARCASES 5

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

http://collections.europarchive.org/tna/20080102155201/http://www.bseinquiry.gov.uk/files/yb/1993/07/19001001.pdf

Rocky Mountain oysters, mountain oysters, prairie oysters, Montana tendergroin or swinging sirloin

POLICY IN CONFIDENCE

CJD AND FARMERS

1. The article in the Daily Mail of 12 August again raises the question of a CAUSATIVE LINK BETWEEN BSE AND CJD. This follows the death of a second farmer from CJD...

snip...

I am, however, concerned about how DH and MAFF would respont to public concern generated if there are further CJD cases among farmers.

snip...

4. Unwelcome, though it maybe to the Tyrrell Committee, I think they must be asked at their next meeting to give further thought to what they might advise the Department and MAFF if ANOTHER FARMER (or TWO) DEVELOPS CJD. OR, if a butcher or abattoir worker develops the disease.

5. Although the Committee were given plenty of advance warning about the second farmer, they may NOT BE SO FORTUNATE NEXT TIME ROUND. Some Contingency planning on the Committee's response to a further case of CJD in a farmer seems essential. At the same time the Committee should consider if there is SPECIAL RISK TO FARMERS, FOR EXAMPLE THEIR HISTORICAL HABIT OF CHEWING CATTLE NUTS, that might be implicated. .....(oh my GOD...tss)

http://web.archive.org/web/20030513183927/http://www.bseinquiry.gov.uk/files/yb/1993/08/12002001.pdf

Ministers will note from this that experts are of the view, that there is unlikely to be a direct link between the cases of BSE, and the occurance of CJD in the farmer.

(NOTE CJD increasing over 3 years. ...TSS)

http://collections.europarchive.org/tna/20080103035512/http://www.bseinquiry.gov.uk/files/yb/1993/08/18004001.pdf

'AGE AT ONSET' is therefore likely to be a reflection of particulary aetiological factors, about which, for sporadic CJD at least, much is yet unknown. IT has therefore been suggested that examination of the f/d i/p of other groups with TSE's, and comparison with that of CJD subsets might help to elucidate aetiological mechanisms for sporadic CJD in particular; i.e. ALMOST A REVERSAL OF THE ORIGINAL UNDERTAKING.

http://collections.europarchive.org/tna/20080103035521/http://www.bseinquiry.gov.uk/files/yb/1993/08/26001001.pdf

OCCUPATIONAL EXPOSURE TO BSE AND CJD

2. The Tyrrell Committee met on 7 October and the significance of the two cases of CJD reported in dairy farmers who had BSE-affected animals on their farms was discussed at some length, AS WERE THE IMPLICATIONS OF A THIRD (OR FORTH) similar case.

3. The Committee were unable to identify any possible risk factors over and above those that they had already considered, both in general and with particular of TASTING THE FEED does continue but there was no consensus about the value of advising farmers to discontinue this practice. Feed currently in use does not pose a risk because of the ruminant-ruminant feed ban.

http://web.archive.org/web/20030326190512/http://www.bseinquiry.gov.uk/files/yb/1993/10/11001001.pdf

MRC

STRAIN CHARACTERISATION OF THE CREUTZFELDT-JAKOB DISEASE AGENT BY TRANSMISSION TO MICE

In view of the CONCERN that exposue to BSE OR SCRAPIE MAY POSE A RISK TO HUMANS, it is proposed investigate the relationship between sporadic creutzfeldt-jakob disease.....

http://web.archive.org/web/20030728000105/www.bseinquiry.gov.uk/files/yb/1993/10/12001001.pdf

OCCUPATIONAL EXPOSURE TO BSE AND CJD

3. While Committee may have no leads to pursue on why farmers might be at increased risk, I hope they understand the urgency with which they will need to respond if or when a THIRD FARMER DEVELOPS CJD.

http://web.archive.org/web/20030326181340/http://www.bseinquiry.gov.uk/files/yb/1993/10/18001001.pdf

INCREASE IN SPORADIC CJD

http://collections.europarchive.org/tna/20080103040526/http://www.bseinquiry.gov.uk/files/yb/1993/11/11001001.pdf

occupational

http://collections.europarchive.org/tna/20080103014125/http://www.bseinquiry.gov.uk/files/yb/1994/02/16001001.pdf

Dealler gets ''dixie chicked' again ;

http://collections.europarchive.org/tna/20080102151107/http://www.bseinquiry.gov.uk/files/yb/1993/11/22001001.pdf

http://web.archive.org/web/20030516065848/http://www.bseinquiry.gov.uk/files/yb/1993/12/08003001.pdf

http://collections.europarchive.org/tna/20080102151046/http://www.bseinquiry.gov.uk/files/yb/1993/12/10006001.pdf

http://web.archive.org/web/20030516070437/http://www.bseinquiry.gov.uk/files/yb/1993/12/14003001.pdf

http://collections.europarchive.org/tna/20080102151051/http://www.bseinquiry.gov.uk/files/yb/1993/12/16006001.pdf

http://collections.europarchive.org/tna/20080102151144/http://www.bseinquiry.gov.uk/files/yb/1993/12/17003001.pdf

STACKING THE DECK AGAINST SPORADIC CJD AND SOUND SCIENCE

APPOINTMENTS IN CONFIDENCE

MEMBERSHIP TO SEAC

snip...

I have informed Dr Tyrrell that we have now written to Dr Hueston to invite him to serve on the Committee and he was very pleased to hear this. He was also in favour of our idea of having a deputy Chairman who could take any emergency meetings eg IF THERE WERE TO BE ANOTHER CJD CASE IN AGRICULTURE. I suggested that either Dr. WIll or Dr Kimberlin were likely candidates and he thought that this was about right. He felt that on balance he would prefer Dr Will who he thought took a more cautious line and was LESS DOGMATIC. .....

http://collections.europarchive.org/tna/20080103034317/http://www.bseinquiry.gov.uk/files/yb/1993/12/01003001.pdf

BUYING THE BEST JUNK SCIENCE YOU CAN FROM THE USA VIA DR. HUESTON

http://web.archive.org/web/20010615140406/http://www.bseinquiry.gov.uk/files/yb/1994/01/00005001.pdf

CHANGING SCIENCE TO FIT YOUR INDUSTRY NEEDS COVER-UP IN FULL MODE NOW

PROBLEM

7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x).

IP PS(L) wishes to probe this further we think it best to explain the matter VERBALLY. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stores. (or the facts...TSS)

http://collections.europarchive.org/tna/20080103020408/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

A REVISED VERSION WHERE THE FOLLOWING WAS MADE TO BE REMOVED FROM SCIENTIFIC FINDINGS. ...TSS

''This year's findings show a number of associations but the strongest is for veal.''

A BIG LINE WAS DRAWN THROUGH THAT SENTENCE TO BE REMOVED DUE TO THE FOLLOWING. THIS IS THE NEXT SENTENCE ;

''This is of considerable concern given recent developments. In particular, Ministers will be particularly concerned about the European dimension given the recent troubles with the Germans.''

YOU can see the beginning of the ukbsenvCJD only theory beginning to unfold now. full text of this ukbsenvcjd only conspiracy can be seen here. ...TSS

POLICY RESTRICTED

http://collections.europarchive.org/tna/20080103020408/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

BRITISH DEER FARMERS ASSOCIATION

OCTOBER 1994

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is completely independent outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

snip...

The statistical results regarding the consumption of venison was put into perspective in the body of the report and was NOT MENTIONED AT ALL IN THE PRESS RELEASE. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of VENISON was highlighted only by the media i.e. in the News at one television programme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of Venison, would increase, and quite possibly GIVE DAMAGING CREDENCE, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

see buttered and watered down report here that caters to industry instead of human safety...TSS

http://web.archive.org/web/20030511211625/http://www.bseinquiry.gov.uk/files/yb/1994/10/00004001.pdf

SEE WHERE THIS ;

''This year's findings show a number of associations but the strongest is for veal.''

WENT TO THIS;

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and increased risk of CJD. When some account was taken of possible confounding, the association between veal eating and risk of CJD emerged as the strongest of these associations statistically. These findings concerning dietary history are particulary difficult to interpret for two reasons.

1. .........BSeee...........TSS

2. .........BSeee...........TSS

(I.E. BSeee = bull sh!t encephalopathy or government cover-up i.e. God save the industry at all cost, including human health. ...TSS)

THUS, the reported veal eating habits of confirmed CJD cases appear virtually identical to suspect cases later judged not to have the disease. This provides good circumstantial evidence to support the hypothesis that the apparent association between veal consumption and CJD is due to recall bias. Analysis of other apparent dietary risk factors for CJD, including venison, has provided similar evidence of recall bias.

snip...

In summary, the analysis of the dietary case-control study demonstrates a strong association between a lifetime history of veal consumption and the risk of developing Creutzfeldt-Jakob disease. HOWEVER this result may well be due to recall bias and analysis of clinical and molecular bilogical features does not provide supportive evidence for the hypothesis that veal eating is a risk factor for CJD. ...

snip...

MORE OF THIS BSeee CAN BE READ IN THE FINAL GOVERNMENT DICTATED CJD REPORT OF 1994

http://collections.europarchive.org/tna/20080103020329/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

BSE SCIENTIST WAS 'CENSORED'

He says that when he worked at MAFF, ''the way it was structurally set up was not that science would drive the politics, but that the politics will drive the science. And that's wrong.''

http://web.archive.org/web/20030526121511/http://www.bseinquiry.gov.uk/files/yb/1997/12/11001001.pdf

11/3/96 DGRC alerts DTI Ministers and CSA to discovery of nvCJD on basis of letter from MRC Chief Executive dated 11/3/96

BIRTH OF THE UKBSEnvCJD ONLY THEORY, the birth of the 'BIG LIE' begins. ...tss

http://web.archive.org/web/20030506080606/http://www.bseinquiry.gov.uk/files/db/do01/tab03.pdf

REPORT OF 16 YEAR OLD GIRL WITH CJD

5. This case may raise fears of a link between BSE and CJD. Current advice is that there is no scientific evidence of a link between BSE in cattle and CJD in humans. Furthermore advice from the Spongiform Encephalopathy Advisory Committee is that they are satisified that all necessary safeguards are in place to minimise further spread of spongiform encephalopathies in animals and to prevent any risk of transmission to humans. ...

http://web.archive.org/web/20030510184139/http://www.bseinquiry.gov.uk/files/yb/1994/01/14005001.pdf

To ask the Secretary of State for Health, how many people under the age of 20 years in each of the last five years have suffered from Creutzfeldt-Jakob disease; and of these how many had not had any growth treatment previously.

SUGGESTED REPLY

We are not aware of any people under the age of 20 in the UK suffering from Creutzfeldt-Jakob Disease in the last five years.

http://web.archive.org/web/20030511045010/http://www.bseinquiry.gov.uk/files/yb/1994/01/20001001.pdf

STATEMENT FROM HOSPITAL

http://collections.europarchive.org/tna/20080102170431/http://www.bseinquiry.gov.uk/files/yb/1994/01/20005001.pdf

As we discussed, a general line could be added, _if pressed_, to the line you have already received, as follows;

"We are confident that no-one from the CJD Surveillance Unit would discuss with any patient's relatives matters relating to BSE or to contact with the press"...(_and consider adding_..."in the way that has been reported."

http://web.archive.org/web/20030515002545/http://www.bseinquiry.gov.uk/files/yb/1994/01/25001001.pdf

PREPARING FOR THE STORM 'LINE TO TAKE'

Handling

4. In addition, Channel 4 is due to broadcast "Dispatches" (made by Fulcrum Productions) tomorrow evening, 26 January. The programme will cover the above issues. Paragraph 2 of a letter from Fulcrum (at D) includes transcript of an interview recorded for the programme with the teenage girl's relative. A copy of the reply from Dr Will, head of the CJD Surveillance Unit, to Fulcrum is also enclosed. The points Dr will makes are fully endorsed by the Department. It is worth noting that Dr Will had earlier spent a considerable amount of time briefing Fulcrum on the CJD Surveillance Unit's activities and on general CJD issues.

5. The implication of a cover-up with respect to the diagnosis in this case and possible links with BSE cannot be substantiated. ...

http://collections.europarchive.org/tna/20080102185543/http://www.bseinquiry.gov.uk/files/yb/1994/01/25003001.pdf

BARONESS CUMBERLEGE TRYING TO MANIPULATE THE MEDIA

http://web.archive.org/web/20030510162629/http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

MAD COW MEAL DESTROYED MY DAUGHTERS LIFE

A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating a contaminated burger it was claimed last night.

VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).

http://web.archive.org/web/20030510205841/http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf

GIVE ME BACK MY LIFE

http://web.archive.org/web/20030511035553/http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf

HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''

http://collections.europarchive.org/tna/20080102185523/http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf

WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY

http://web.archive.org/web/20030513071650/http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf

I have interviewed Mrs Rimmer at my constituency surgery

IF there is nothing to hide, why is there so much SECRECY? WHY is the Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are taken, surely the problem could be contained, however, as it stands the lack of investigation and interest of the possibility of B.S.E. and C.J.D. being linked is open for speculation and surely someone has to account for peoples lives! WHY is so much trouble being taken to convice people that B.S.E. and C.J.D. are not linked? Guilty Conscience perhaps ? - or cover up?

HOUSE OF COMMONS

FROM BARRY JONES, M.P.

22 FEBRUARY 1994

http://collections.europarchive.org/tna/20080103013714/http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

(now story changes that biopsy shows she does not have CJD...tss)

http://web.archive.org/web/20030511045541/http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf

now story changes to ;

Advice

7. The Parliamentary Secretary is invited to note the recent statements made on __________ and the present position which remains that CJD cannot be confirmed, in this case at this stage.

http://web.archive.org/web/20030510165315/http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf

3. The Medical Director at ___________________ Hospital advised the Department on 6 June that the results of ___________________ brain biopsy had been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital subsequently issued a statement to the press to this effect and this was publicised widely in the press (doc 1). News coverage which followed suggested that the statement made by ________________ Hospital had been misleading (doc 2). Enquires have been made of the Medical Director at _______________ Hospital who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR. The facts are that two pathology reports on the same piece of brain tissue were recieved. The first report indicated that CJD was unlikely, The second report indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive diagnosis could be made before a post mortem was undertaken.

http://collections.europarchive.org/tna/20080103015813/http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf

(ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM SPORADIC CJD, the same damn thing. ...TSS)

IN light of Asante/Collinge et al findings 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;

-------- Original Message --------

Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD

Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To: "[log in to unmask]"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[log in to unmask] (until 9/12/02)

New e-mail: [log in to unmask] (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

WHAT ABOUT U.S.A. ???

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://web.archive.org/web/20030326211011/http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf

20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....

http://web.archive.org/web/20030330212925/http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

NOW BACK TO THOSE FARMERS WITH BSE HERDS THAT DIED FROM SPORADIC CJD

CJD FARMERS WIFE 1989

http://collections.europarchive.org/tna/20080103005651/http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf

Dear Bob,

An otherwise enjoyable dinner last night as guest of the Association of Clinical Pathology was marred by a conversation with a neuropathologist who was just about to write CMO with details of a case of CJD he had just seen. When first mentioning the case, he claimed she was only 36, but after a few more glasses of wine he became less certain of that and thought she could have been older. Locally, they made quite an assoctiation with BSE, since she ws a farmers wife on that farm that, atypically for that area of S Yorkshire, had several BSE cases. I was told the clinical and pahtological pictures were typical of CJD. ...

http://collections.europarchive.org/tna/20080102155149/http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

cover-up of 4th farm worker ???

http://web.archive.org/web/20030331213802/http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://web.archive.org/web/20030330175323/http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf

CONFIRMATION OF CJD IN FOURTH FARMER

http://collections.europarchive.org/tna/20080102203608/http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://web.archive.org/web/20030728074919/http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf

4th farmer, and 1st teenager

http://collections.europarchive.org/tna/20080102235004/http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf

2.

snip...

Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator.

If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...

http://web.archive.org/web/20030516181226/http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

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

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

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

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 2007

http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html

http://nor-98.blogspot.com/

SCRAPIE USA

http://scrapie-usa.blogspot.com/

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

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

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:

[log in to unmask]

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

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texsas USA 77518

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

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

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

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

Greetings,

SOMETHING ELSE TO PONDER HERE, lets go back and look and the other potential cases of CWD transmission to humans and where it was explained away as genetic too i.e. GSS ;

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ˜22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Table 2. Creutzfeldt-Jakob disease patients investigated for a possible causal link of their illness with chronic wasting disease of deer and elk, United Statesa

LOOK AT 1 AND 3B BOTH GSS. ...TSS

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm#table2

Monday, June 30, 2008

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

please see additional comments and other studies ;

http://chronic-wasting-disease.blogspot.com/2008/06/risk-behaviors-in-rural-community-with.html

http://organicconsumers.org/forum/index.php?showtopic=1659

CWD

http://chronic-wasting-disease.blogspot.com/

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

10 people killed by new CJD-like disease USA

2008-07-09T12:55:00.000-07:00

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles 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.

As in other spongiform encephalopathies, such as CJD and mad cow disease (BSE), the brain tissue of victims is full of tiny holes. This damage is thought to be caused by the accumulation of prions, misfolded versions of a brain protein called PrP that can convert normal PrP molecules into their own misshapen form.

Some features of the new disease are different, however. All known disease-causing prions resist degradation by proteases - enzymes which digest the normal form of PrP. But prions from patients with the new disease are broken down by the enzymes.

Some very rare forms of CJD run in families and are caused by mutations in the gene for PrP. Six of the cases described in Gambetti's paper were from families with a history of dementia, suggesting a genetic cause. However, these people had no mutations in their PrP genes. "Maybe there are other genes that have an influence on the disease," suggests James Ironside of the UK's National CJD Surveillance Unit in Edinburgh.

Most forms of CJD develop spontaneously, for unknown reasons, but can be spread if someone is exposed to brain material from people with CJD, for instance, by neurosurgery using inadequately sterilised instruments.

One variant of CJD has been linked to the consumption of contaminated meat from cattle with mad cow disease. If the new condition is similarly caused by something in the victims' diet, or another environmental cause, new measures might be needed to protect public health.

Gambetti is now conducting experiments in mice to see how the disease is transmitted. He suspects that there is no cause for alarm. "I believe the disease occurs naturally, and is not due to environmental causes," he says.

###

New Scientist Reporter: Andy Coghlan

IF REPORTING ON THIS STORY, PLEASE MENTION NEW SCIENTIST AS THE SOURCE AND, IF REPORTING ONLINE, PLEASE CARRY A LINK TO: http://www.newscientist.com

UK CONTACT - Claire Bowles, New Scientist Press Office, London: Tel: +44(0)20 7611 1210 or email claire.bowles@rbi.co.uk

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

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.

[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:

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

TSS

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/tubulovesicular-structures-are.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

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

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/novel-human-disease-with-abnormal-prion.html

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

---------- https://lists.aegee.org/cjd-l.html ----------

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

2008-06-21T09:29:00.000-07:00

Greetings,

I kindly reminder, with additional references. ...

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

JUNE 20, 2008

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.

With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE (one strain TSE in cows), and the nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human.

WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

My name is Terry S. Singeltary Sr. and I am no scientist, no doctor and have no PhDs, but have been independently researching human and animal TSEs since the death of my Mother to the Heidenhain Variant of Creutzfeldt Jakob Disease on December 14, 1997 'confirmed'. ...TSS

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

additional new source references and old source references as follows ;

ADDITIONAL NEW REFERENCE SOURCES

Original Article

A novel human disease with abnormal prion protein sensitive to protease

Pierluigi Gambetti, MD 1 *, Zhiqian Dong, PhD 1, Jue Yuan, BA 1, Xiangzhu Xiao, PhD 1, Mengjie Zheng, PhD 1, Amer Alshekhlee, MD 1, Rudy Castellani, MD 2, Mark Cohen, MD 1, Marcelo A. Barria, PhD 3, D. Gonzalez-Romero, PhD 3, Ermias D. Belay, MD 4, Lawrence B. Schonberger, MD, MPH 4, Karen Marder, MD 5, Carrie Harris, BA 1, James R. Burke, MD, PhD 6, Thomas Montine, MD 7, Thomas Wisniewski, MD 8, Dennis W. Dickson, MD 9, Claudio Soto, PhD 3, Christine M. Hulette, MD 10, James A. Mastrianni, MD, PhD 11, Qingzhong Kong, PhD 1, Wen-Quan Zou, MD, PhD 1 * 1Institute of Pathology, Case Western Reserve University, Cleveland, OH 2Department of Pathology, University of Maryland, Baltimore, MD 3Department of Neurology, Neuroscience and Cell Biology, George and Cynthia Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX 4Centers for Disease Control and Prevention, Atlanta, GA 5Department of Neurology, Columbia University, New York, NY 6Department of Medicine, Division of Neurology, Duke University, Durham, NC 7Harborview Medical Center, University of Washington, Seattle, WA 8Department of Neurology, New York University, New York, NY 9Department of Neuropathology, Mayo Clinic College of Medicine, Jacksonville, FL 10Department of Pathology, Duke University, Durham, NC 11Department of Neurology, University of Chicago, Chicago, IL

email: Pierluigi Gambetti (pxg13@case.edu) Wen-Quan Zou (wenquan.zou@case.edu)

*Correspondence to Pierluigi Gambetti, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106

*Correspondence to Wen-Quan Zou, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106

Funded by: NIH; Grant Number: AG14359, AG08702, NS049173 Centers for Disease Control and Prevention; Grant Number: CCU 515004 Britton Fund CJD Foundation

Abstract

Objective To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) that, contrary to the common prion diseases, is predominantly sensitive to protease digestion.

Methods Eleven subjects were investigated at the National Prion Disease Pathology Surveillance Center for clinical, histopathological, immunohistochemical, genotypical, and PrP characteristics.

Results Patients presented with behavioral and psychiatric manifestations on average at 62 years, whereas mean disease duration was 20 months. The type of spongiform degeneration, the PrP immunostaining pattern, and the presence of microplaques distinguished these cases from those with known prion diseases. Typical protease-resistant PrP was undetectable in the cerebral neocortex with standard diagnostic procedures. After enrichment, abnormal PrP was detected at concentrations 16 times lower than common prion diseases; it included nearly 4 times less protease-resistant PrP, which formed a distinct electrophoretic profile. The subjects examined comprised about 3% of sporadic cases evaluated by the National Prion Disease Pathology Surveillance Center. Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.

Interpretation The distinct histopathological, PrP immunohistochemical, and physicochemical features, together with the homogeneous genotype, indicate that this is a previously unidentified type of disease involving the PrP, which we designated protease-sensitive prionopathy (or PSPr). Protease-sensitive prionopathy is not rare among prion diseases, and it may be even more prevalent than our data indicate because protease-sensitive prionopathy cases are likely also to be classified within the group of non-Alzheimer's dementias. Ann Neurol 2008;63:697-708

---------------------------------------------------------------------------- ---- Received: 5 November 2007; Revised: 1 April 2008; Accepted: 4 April 2008 Digital Object Identifier (DOI)

10.1002/ana.21420 About DOI

http://www3.interscience.wiley.com/journal/119883040/abstract

Pages: 677-678 A new prionopathy Robert Will, Mark Head http://www3.interscience.wiley.com/cgi-bin/abstract/119882940/
ABSTRACT

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/novel-human-disease-with-abnormal-prion.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

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

http://brain.hastypastry.net/forums/showthread.php?t=15076

http://brain.hastypastry.net/forums/archive/index.php/t-17057.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

IF BSE is not in the USA (just not documented for many different reasons), and only atypical BSE is in the USA (plus CWD, plus, many strains of Scrapie, and Now the Nor-98 documented in 5 different states, plus TME, then why would human mad cow in the USA look like the UK nvCJD from UK BSE cows ? it was shown long ago in studies at Mission Texas that experimental transmission of USA Scrapie to USA Bovine, DID NOT LOOK LIKE UK BSE. so again, in short, why would human mad cow in the USA look like human mad cow in the UK i.e. the (nvCJD). however, I believe that BSE has been in the USA untested and undocumented for years. why on earth then does the USDA refuse to allow creekstone or anyone else test their product? simple, if you don't look/test, you don't find.

snip...

please see full text ;

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html

3.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE.339 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture.340 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre.341 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle,

*** did not produce the same clinical signs of brain lesions characteristic of BSE. ***

3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988,342 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988–89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA.343 It was also felt that the results of such an experiment would be hard to interpret. While a negative result 337 Fraser, H., Bruce, M., Chree, A., McConnell, I. and Wells, G. (1992) Transmission of Bovine Spongiform Encephalopathy and Scrapie to Mice, Journal of General Virology, 73, 1891–7; Bruce, M., Chree, A., McConnell, I., Foster, J., Pearson, G. and Fraser, H. (1994) Transmission of Bovine Spongiform Encephalopathy and Scrapie to Mice: Strain Variation and the Species Barrier, Philosophical Transactions of the Royal Society of London, Series B, Biological Sciences, 343, 405–11 338 Bruce, M., Will, R., Ironside, J., McConell, I., Drummond, D., Suttie, A., McCordie, L., Chree, A., Hope, J., Birkett, C., Cousens, S., Fraser, H. and Bostock, C. (1997) Transmissions to Mice Indicate that ‘New Variant’ CJD is Caused by the BSE Agent, Nature, 389, 498–501 339 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606–12 340 YB88/10.00/1.1 341 Cutlip, R., Miller, J., Race, R., Jenny, A., Katz, J., Lehmkuhl, H., Debey, B. and Robinson, M. (1994) Intracerebral Transmission of Scrapie to Cattle, Journal of Infectious Diseases, 169, 814–20 342 YB88/6.21/1.2 343 YB88/11.17/2.4 SCIENCE 84 would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE.344 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest.345 3.59 Nevertheless, from the first demonstration of transmissibility of BSE in 1988, the possibility of differences in the transmission properties of BSE and scrapie was clear. Scrapie was transmissible to hamsters, but by 1988 attempts to transmit BSE to hamsters had failed. Subsequent findings increased that possibility.

http://www.bseinquiry.gov.uk/pdf/volume2/chapter3.pdf

NEW SOLUTIONS: A Journal of Environmental and Occupational Health Policy

Issue: Volume 18, Number 2 / 2008 Pages: 145 - 156 URL: Linking Options

Mad Cows and Computer Models: The U.S. Response to BSE

Frank Ackerman and Wendy A. Johnecheck

Abstract:

The proportion of slaughtered cattle tested for BSE is much smaller in the U.S. than in Europe and Japan, leaving the U.S. heavily dependent on statistical models to estimate both the current prevalence and the spread of BSE. We examine the models relied on by USDA, finding that the prevalence model provides only a rough estimate, due to limited data availability. Reassuring forecasts from the model of the spread of BSE depend on the arbitrary constraint that worst-case values are assumed by only one of 17 key parameters at a time. In three of the six published scenarios with multiple worst-case parameter values, there is at least a 25% probability that BSE will spread rapidly. In public policy terms, reliance on potentially flawed models can be seen as a gamble that no serious BSE outbreak will occur. Statistical modeling at this level of abstraction, with its myriad, compound uncertainties, is no substitute for precautionary policies to protect public health against the threat of epidemics such as BSE.

http://baywood.metapress.com/app/home/contribution.asp?referrer=parent&backto=issue,5,18;journal,1,41;linkingpublicationresults,1:300327,1

snip... please see full text ;

http://bse-atypical.blogspot.com/2008/06/mad-cows-and-computer-models-us.html

Thursday, June 05, 2008

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

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

please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html

Sunday, March 16, 2008 MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE March 16, 2008

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Tuesday, June 17, 2008

Portsmouth woman did not die of mad cow-related condition, USDA says UPDATE Updated Jun.17, 2008 08:34 KST

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html

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

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

U.S. slams door on revising S. Korea beef import pact

June 11, 2008, 10:14PM

http://usdavskorea.blogspot.com/2008/06/us-slams-door-on-revising-s-korea-beef.html

Wednesday, June 11, 2008

OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)

http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html

http://organicconsumers.org/forum/index.php?showtopic=1566

Saturday, June 7, 2008

Export Requirements for the Republic of Korea IMPORT HEALTH REQUIREMENTS FOR U.S. BEEF AND BEEF PRODUCTS

http://usdavskorea.blogspot.com/2008/06/export-requirements-for-republic-of.html

Why Americans, As Well as Koreans, Should Be Worried About Mad Cow Tainted USA Beef

By Terry S. Singeltary Sr. May 15, 2008

Straight to the Source

Web Note: This is an important commentary by Terry S. Singeltary Sr., on a recent Business Week story on the controversy in South Korea over their government's lifting on the ban on conventional (non-organic) beef, despite the fact that the USDA is still allowing slaughterhouse waste and blood and manure to be fed to cows, and refusing to test all cows at slaughter. See the Mad Cow section of the OCA website for in-depth information. Terry is a regular blogger on the OCA website on Mad Cow issues.

Ronnie Cummins

One Korean official says the probability of a human being catching a mad cow disease by eating U.S. beef is like the one of a golf player scoring a hole-in-one and then being killed by lightning.

this is typical BSe. you here industry groups comment 'your more likely to get hit by a car than die from CJD'. well, maybe so, but my mother and many more did not die from getting hit by a car, they died from CJD, my mothers being the hvCJD (confirmed), and my neighbors mother died from CJD (confirmed). the UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.

snip...see full text ;

http://www.grassrootsnetroots.org/articles/article_12387.cfm

Tuesday, May 13, 2008

Concerned Americans against Mad Cow Disease STATEMENT OF SOLIDARITY with Koreans May 13, 2008

http://usdavskorea.blogspot.com/2008/05/concerned-americans-against-mad-cow.html

http://flounder068.vox.com/library/post/concerned-americans-against-mad-cow-disease-statement-of-solidarity-with-koreans-may-13-2008.html

http://www.koreantopnews.com/story.php?title=USDA_VS_KOREA_typical_or_atypical_BSe_Concerned_Americans_against_Mad_Cow_Disease_STATEMENT_OF_SOLIDARITY_with_Koreans_May_13_2008

BSE YOUNGEST AGE STATISTICS UNDER 30 MONTHS

http://bseyoungestage.blogspot.com/

http://flounder068.vox.com/library/post/bse-youngest-age-statistics-under-30-months.html

PLEASE NOTE IN REFERENCE TO THE LATEST LONG TERM USDA DOWNER COW SCHOOL LUNCH PROGRAM CASE STUDY FOR VCJD IN CHILDREN

Creutzfeldt-Jakob Disease (Variant) and Bovine Spongiform Encephalopathy (Prion Diseases) Description Since 1996, strong evidence has accumulated for a causal relationship between ongoing outbreaks, primarily in Europe, of a disease in cattle called bovine spongiform encephalopathy (BSE, or “mad cow disease”) and a disease in humans called variant Creutzfeldt-Jakob disease (vCJD). Both disorders, which are caused by an unconventional transmissible agent, are invariably fatal brain diseases with incubation periods typically measured in years (1). Transmission of the BSE agent to humans, leading to vCJD, is believed to occur via ingestion of cattle products contaminated with the BSE agent; the specific foods associated with this transmission are unknown. However, a recently published case-control study involving 132 vCJD cases in the United Kingdom (UK) showed evidence of an increased risk for vCJD associated with the frequency of consuming beef products likely to contain mechanically recovered meat and head meat (such as burgers, meat pies, and sausages) (2). Bioassays and molecular tests have enabled identification of what World Health Organization consultants have classified as “high-infectivity” and “lower infectivity” tissues of cattle with BSE (3). The high-infectivity tissues include the brain, spinal cord, retina, optic nerve, and dorsal root and trigeminal ganglia, suggesting that these tissues can pose a relatively high risk of transmission. The lower infectivity tissues include peripheral nerves (e.g., sciatic and facial nerves), tonsils, nictitating membrane (third eye lid), distal ileum, bone marrow, and possibly thigh muscle. The latter tissue from one cow with BSE transmitted disease to highly BSE-sensitive transgenic mice at a rate indicative of trace levels of infectivity.

http://wwwn.cdc.gov/travel/yellowBookCh4-VariantPrions.aspx

Thursday, February 21, 2008

TRANSCRIPT: Technical Briefing - Hallmark/Westland Meat Packing Company - (02/21/08)

Release No. 0054.08

http://downercattle.blogspot.com/2008/02/transcript-technical-briefing.html

FULL HISTORY OF USDA CERTIFIED DEAD STOCK DOWNER COW SCHOOL LUNCH PROGRAM

http://downercattle.blogspot.com/

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

[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

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.

snip...

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.

snip...

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

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." flounder9@verizon.net To: "Bovine Spongiform Encephalopathy" BSE-L@aegee.org Cc: heggem.daniel@epa.gov; sibert.christopher@epa.gov; denne.jane@epa.gov; hazen.susan@epa.gov; mcrosby@ucsusa.org; erobinson@ucsusa.org; enegin@ucsusa.org; cjdvoice@yahoogroups.com; 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/

White House invokes executive privilege in EPA inquiry The Bush administration refuses to turn over subpoenaed documents related to the agency's decision to prevent California from enacting stricter emissions standards than the federal government. By Richard Simon, Los Angeles Times Staff Writer June 21, 2008 WASHINGTON -- Escalating a fight with Democrats on Capitol Hill, the White House on Friday invoked executive privilege in refusing to turn over documents to a congressional committee investigating the Environmental Protection Agency's decision to deny California permission to implement its own vehicle emission standards.

The Bush administration asserted executive privilege hours before the House Oversight and Government Reform Committee was to vote on whether to bring contempt-of-Congress proceedings against EPA Administrator Stephen L. Johnson and Susan Dudley, administrator of regulatory affairs in the White House Office of Management and Budget, for refusing to turn over subpoenaed documents.

Committee Chairman Henry A. Waxman (D-Beverly Hills) put off a vote on the contempt resolutions while he considers his options.

"I don't think we've had a situation like this since Richard Nixon was president," he said, appearing determined to press ahead, even if it leads to a court fight. "We don't know whether this privilege that's being asserted is valid or not."

Presidents since George Washington have claimed rights to executive branch confidentiality, according to the nonpartisan Congressional Research Service. The Bush White House invoked executive privilege to prevent officials from testifying about the dismissal of nine U.S. attorneys in 2006. President Clinton cited presidential privilege during investigations into the Monica Lewinsky scandal and on other issues.

House and Senate committees have been investigating what role the White House played in EPA decisions preventing California and other states from enacting tougher emissions rules than the federal government and in the EPA's approval of new ozone pollution standards.

The administration's claim of executive privilege is the latest twist in the escalating legal and political battle over California's efforts to implement its own law combating global warming. Critics of the EPA decision contend that it was based on politics, not science or the law.

As Waxman considered his next move in his fight with the White House, another House committee in the room next door grilled former Bush Press Secretary Scott McClellan, who wrote a revealing book about his days in the White House. The hearings were a sign of determination by Democrats not to ease up on their oversight activities, even in the final months of the Bush administration.

In asserting executive privilege in the EPA inquiry, the administration made public a copy of a letter sent to the president by Atty. Gen. Michael B. Mukasey saying that releasing internal documents "could inhibit the candor of future deliberations among the president's staff."

EPA spokesman Tim Lyons said the agency had provided the committee with more than 7,000 documents and devoted 2,200 hours of staff time to responding to requests for information, and he called it "disappointing" that the committee had decided to "politicize environmental regulations."

Jim Nussle, director of the Office of Management and Budget, took issue with Waxman's "sudden and unwarranted" move to consider contempt proceedings, noting that Dudley had appeared before Waxman's committee last month and was asked "only four questions" -- and only one by the panel chairman.

"There is no valid reason for moving from mutual cooperation to unilateral confrontation," Nussle wrote Waxman.

Waxman said: "I am very disappointed and disturbed that the administration is keeping this information from us, and I think we have a right to it."

richard.simon@latimes.com

http://www.latimes.com/news/nationworld/nation/la-na-epa21-2008jun21,0,1939720.story

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

BSE INQUIRY DFAs

http://bseinquiry.blogspot.com/

Sunday, May 18, 2008 BSE Inquiry DRAFT FACTUAL ACCOUNT DFA BSE Inquiry DRAFT FACTUAL ACCOUNTS DFA's

http://bseinquiry.blogspot.com/2008/05/bse-inquiry-draft-factual-account-dfa.html

Sunday, May 18, 2008 BSE, CJD, and Baby foods (the great debate 1999 to 2005)

http://bseinquiry.blogspot.com/2008/05/bse-cjd-and-baby-foods-great-debate.html

Sunday, May 18, 2008 MAD COW DISEASE BSE CJD CHILDREN VACCINES

http://bseinquiry.blogspot.com/2008/05/mad-cow-disease-bse-cjd-children.html

OLD SOURCE REFERENCES

Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies Location: Virus and Prion Diseases of Livestock

Title: Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities

Authors

Kehrli, Marcus O`rourke, Katherine Hamir, Amirali Richt, Juergen Nicholson, Eric Silva, Christopher Edelman, Daniel - FOOD AND DRUG ADMINISTRAT Gay, Cyril

Submitted to: Government Publication/Report Publication Type: Government Publication Publication Acceptance Date: May 1, 2007 Publication Date: July 1, 2007

Citation: Kehrli, Jr., M.E., O'Rourke, K.I., Hamir, A.N., Richt, J.A., Nicholson, E.M., Silva, C.J., Edelman, D., Gay, C.G. 2007.

Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities [government white paper]. Beltsville, MD: Interagency Working Group on Prion Science, Subcommittee on Pathobiology and Diagnostics. USDA, Agriculture Research Service. 33 p.

Technical Abstract:

Transmissible spongiform encephalopathies (TSEs) are fatal neurologic diseases that can affect several animal species and human beings. There are four animal TSE agents found in the United States: scrapie of sheep and goats; chronic wasting disease (CWD) of deer, elk, and moose; transmissible mink encephalopathy (TME) and bovine spongiform encephalopathy (BSE). Although the animal TSEs do not cause major death losses among US livestock populations, they are important because of international trade issues. The experience of the United Kingdom and Europe in dealing with the vast majority of the world's BSE cases, serves as a reminder of the need for continuing vigilance in monitoring risks for public health and research to answer remaining questions around the pathogenesis and transmission of these diseases. There remain questions on 1) cross-species transmissibility of TSEs in livestock and wildlife; 2) the pathobiology of TSEs in natural and secondary hosts; pathogenesis and transmission of CWD; and 4) pathogenesis and ante mortem detection of typical and atypical BSE. Our understanding of the pathogenesis and transmission of these diseases continues to evolve as ongoing, global TSE research efforts focus on defining tissue sites of abnormal prion accumulation, routes of infection, methods of strain differentiation, genetics of susceptibility and ante-mortem diagnostics. In this paper, a Subcommittee on Pathobiology and Diagnostics of TSEs for an Interagency Working Group on Prion Science summarizes the science of animal TSEs in order to identify knowledge gaps for the purpose of prioritizing animal prion research needs. Because of substantial losses involving international trade and potential risk for interspecies transmission to susceptible livestock and possibly humans, the presence of BSE, CWD, scrapie and TME in the United States presents a liability to U.S. domestic and alternative livestock industries. In addition, the proven risk of BSE to agriculture and public health from subclinical or clinically sick animals requires science-based surveillance for any silent, unrecognized epizootic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals. CWD is an example of an uncontrolled expanding epidemic that threatens not only cervids but possibly other livestock. CWD also has elicited public health surveillance programs to monitor for scientific evidence of a prion disease in humans that consume venison. Therefore, some of the research needs are precautionary, but the risks to animal and human health from being caught unaware are high. Efforts are being made by both federal and state regulatory agencies to eradicate scrapie and CWD, and to determine the prevalence of BSE. The effectiveness of these programs will depend heavily on having accurate information about the nature of these diseases, not only in the original hosts, but also in other species that may be in contact with infected animals.

http://arsserv0.tamu.edu/research/publications/Publications.htm?seq_no_115=212488

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

[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

If, on the other hand, atypical BSE continues to occur as typical BSE disappears, this would be a strong indication that it is indeed sporadic, and if in addition at least 1 form of what is presently considered as sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot signature like BASE) were to increase, this would suggest (although not prove) a causal relationship (Figure 5).

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm

Creutzfeldt-Jakob Disease Mortality in Japan, 1979-2004: Analysis of National Death Certificate Data

Yuriko Doi1), Tetsuji Yokoyama2), Miyoshi Sakai2) and Yosikazu Nakamura3)

1) Department of Epidemiology, National Institute of Public Health. 2) Department of Technology Assessment and Biostatistics, National Institute of Public Health. 3) Department of Public Health, Jichi Medical University.

(Received: September 13, 2006) (Accepted: March 18, 2007)

Abstract BACKGROUND: Trend of the mortality rate of Creutzfeldt-Jakob disease (CJD) in Japan is still unclear. This study aimed to estimate annual crude mortality rates due to CJD and examine the CJD mortality trend in Japan during the period of 1979-2004. METHODS: National death certificate data on CJD were used (CJD coded as 046.1 for ICD-9 and A81.0 for ICD-10). Trends in age-standardized mortality rates for CJD were examined by using time series analyses including the joinpoint regression analysis. RESULTS: A total of 1,966 deaths (862 males and 1,104 females) were identified with CJD coded as the underlying-cause-of-death. The annual number of deaths and crude mortality rates peaked in 2004 at 163 (66 for males and 97 for females) deaths and 1.28 (1.06 for males and 1.48 for females) deaths per million population per year, respectively. The age-specific mortality rates rapidly increased with age between 50 and 74 years, especially among females, and sharply declined at 80+ years. Throughout the observed period, there were no significant change points, and the annual percentage changes (95% confidence intervals) were +3.09 (2.18 - 4.02) % for males and +3.90 (2.98-4.83) % and females. The total number of CJD deaths under 50 years of age was 131, and there was found no increase in the annual number of deaths for the past few years in this age group. CONCLUSION: CJD mortality in trend data based on death certificates has significantly increased in Japan during the period of 1979-2004. J Epidemiol 2007; 17: 133-139.

Key words: Creutzfeldt-Jakob Syndrome; Regression Analysis; Mortality; Death Certificate; Japan

snip...

AS demonstrated in this study, we found a significant linear increase in trends for age standardized mortality rates from the disease, with +3-4% of annual percentage change, between 1979 and 2004. In interpreting the results, we should consider some factors that might contribute to a false increase in mortality, such as the change of ICD codes and the enhancement of case findings (e.g., physicians9 recognition of the disease, diagnostic tests, and quality of health care). No revolutionary new diagnostic test for CJD became available throughout the observational period. On the other hand, there were a few critical points of time to consider: in 1991, patients with CJD transmitted by cadaveric dura transplants were identified in Japan9, in 1995, the ICD code for CJD was changed from 9th to 10th version in Japan; and in 1996, a new case of vCJD causally linked to BSE was reported from the United Kingdom.6 Without an abrupt rise of age-standardized mortality rates from CJD after these years for both sexes, however, it is unlikely that these events artificially affected the increase in CJD mortality.

Rather, it may be the true fact that in Japan our results reflect to a large extent a genuine increase in CJD. The number of iCJD cases may still increase even after the total ban on the practice of causal grafts.5,8 Regarding sporadic CJD (sCJD), a recent report from the European Unions collective study on CJD suggests that the mortality rates from sCJD increased with time between 1993 and 2002.20 It is quite probable that this temporal increase of sCJD may also exist in Japan. The increase may have been accompanied to some extent by the improvement of physicians diagnostic skills for CJD since 1997 when a manual for clinical practice on CJD was introduced in our country.20,21

http://www.jstage.jst.go.jp/article/jea/17/4/17_133/_article

http://www.jstage.jst.go.jp/article/jea/17/4/133/_pdf

sporadic cjd

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&P=25276

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES)

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165

An evaluation of scrapie surveillance in the United States From: Terry S. Singeltary Sr. Date: Sun, 5 Aug 2007 13:05

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427

SEAC New forms of Bovine Spongiform Encephalopathy 1 August 2007 From: Terry S. Singeltary Sr. Date: Sun, 5 Aug 2007 13:09:38 -0500

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3573

Owens, Julie From: Terry S. Singeltary Sr. [flounder9@verizon.net] Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98 8/3/2006

Greetings FSIS,

I would kindly like to comment on the following ;

[Federal Register: July 12, 2006 (Volume 71, Number 133)] [Notices] [Page 39282-39283] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr12jy06-35] ----------------------------------------------------------------------- DEPARTMENT OF AGRICULTURE Food Safety and Inspection Service [Docket No. FSIS-2006-0011] Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Update; Notice of Availability and Technical Meeting

snip...

http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-10928.htm

MY comments/questions are as follows ;

1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?

*** Suppressed peer review of Harvard study October 31, 2002 ***

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf

2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today ?

snip...

full text 98 pages ;

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

03-025IFA 03-025IFA-2 Terry S. Singeltary

Page 1 of 17

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Thursday, September 08, 2005 6:17 PM

To: fsis.regulationscomments@fsis.usda.gov

Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle

Greetings FSIS,

I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle Broken bones and such may be the first signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;

SUB CLINICAL PRION INFECTION

MRC-43-00

Issued: Monday, 28 August 2000

NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION: IMPORTANT RESEARCH

FINDINGS RELEVANT TO CJD AND BSE

A team of researchers led by Professor John Collinge at the Medical

Research Council Prion Unit1 report today in the Proceedings of the

National Academy of Sciences, on new evidence for the existence of a

"sub-clinical" form of BSE in mice which was unknown until now....

full text 17 pages ;

https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument

Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS

Docket

No. 04-047-l

No. 04-021ANPR

No. 2004N-0264

NEW BSE SAFEGUARDS

Federal Measures to Mitigate BSE Risks: Considerations for Further Action

http://www.fda.gov/cvm/index/updates/bseanprm.htm

Greetings FDA, USDA and APHIS et al,

I would kindly like to comment on the continued delay of the regulations that have been proposed for years to reduce the risk of BSE/TSE in the USA. Each day that is wasted debating this issue allows this agent to spread, and many many more humans and animals become needlessly exposed to this agent via a multitude of potential routes and sources right here in the USA. TO continue to ignore the new findings from several scientists about the fact that BSE is not the only strain of TSE in cattle, the fact that new atypical strains of TSE are showing up in not only cattle, but sheep and the fact that the new strain of TSE in cattle seems to be more similar to sporadic CJD as opposed to the nv/v CJD, to continue to ignore these findings will only further spread this agent. ..............

full text ;

https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044eb1f/eff9eff1f7c5cf2b87256ecf000df08d

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html

PART 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

EMC 7 Terry S. Singeltary Sr. Vol #: 1

http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm

Docket Management Docket: 02N-0276 - Bioterrorism Preparedness ... General Comments, Subject: Docket No: 02-088-1 RE-Agricultural ... From: Terry S. Singeltary Sr.

To: regulations@aphis.usda.gov

Docket No: 02-088-1 Title: ...

Greetings FDA and public,

if you go to the below site, and search all BSE known countries and check out their air traffic illegal meat they have confiscated, and check out the low number checked, compared to actual passenger traffic, would not take too much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.

[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.]]

if they were to have questioned the terrorist that bombed the Twin Towers with jets, if they were to have questioned them at flight school in the USA, i am sure that they would have said they did not intend to visit the Twin Towers as a flying bomb either. what am i thinking, they probably did ask this? stupid me. ...

full text ;

http://www.fda.gov/ohrms/DOCKETS/dockets/02n0276/02N-0276-EC-254.htm

PDF]Freas, William TSS SUBMISSION

File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [flounder@wt.net] Monday, January 08, 200l 3:03 PM freas ...

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version).

I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here:

remember AIDS/HIV, 'no problem to heterosexuals in the U.S.? no need to go into that, you know of this blunder.

DO NOT make these same stupid mistakes again with human/animal TSE's aka MADCOW DISEASE. I lost my Mom to hvCJD, and my neighbor lost his Mother to sCJD as well (both cases confirmed). I have seen many deaths, from many diseases. I have never seen anything as CJD, I still see my Mom laying helpless, jerking tremendously, and screaming "God, what's wrong with me, why can't I stop this". I still see this, and will never forget. Approximately 10 weeks from 1st of symptoms to death. This is what drives me. I have learned more in 3 years about not only human/animal TSE's but the cattle/rendering/feeding industry/government than i ever wished to.

I think you are all aware of CJD vs vCJD, but i don't think you all know the facts of human/animal TSE's as a whole, they are all very very similar, and are all tied to the same thing, GREED and MAN.

I am beginning to think that the endless attempt to track down and ban, potential victims from known BSE Countries from giving blood will be futile. You would have to ban everyone on the Globe eventually? AS well, I think we MUST ACT SWIFTLY to find blood test for TSE's, whether it be blood test, urine test, eyelid test, anything at whatever cost, we need a test FAST.

DO NOT let the incubation time period of these TSEs fool you.............

full text 6 pages ;

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

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

see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408

MORE OLD SOURCE REFERENCES;

Full Text Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama

Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi

Summary

Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

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...

Discussion

In the apparent absence of a foreign nucleic acid genome

associated with the agents responsible for transmissible

spongiform encephalopathies or prion diseases,

efforts to provide a molecular definition of agent strain

have focused on biochemical differences in the abnormal,

disease-associated form of the prion protein, termed

PrPSc. Differences in PrPSc conformation and glycosylation

have been proposed to underlie disease phenotype

and form the biochemical basis of agent strain. This

proposal has found support in the observation that the

major phenotypic subtypes of sCJD appear to correlate

with the presence of either type 1 or type 2 PrPSc in

combination with the presence of either methionine or

valine at codon 129 of the prion protein gene.2 Similarly,

the PrPSc type associated with vCJD correlates with the

presence of type 2 PrPSc and is distinct from that found in

sCJD because of a characteristically high occupancy of

both N-linked glycosylation sites (type 2B).6,11 The

means by which such conformational difference is detected

is somewhat indirect; relying on the action of proteases,

primarily proteinase K, to degrade the normal

Figure 6. Type 1 PrPSc is a stable minority component of PrPSc from the vCJD

brain. Western blot analysis of PrP in a sample of cerebral cortex from a case

of vCJD during digestion with proteinase K is shown. Time points assayed

are indicated in minutes (T0, 5, 10, 30, 60, 120, 180). Duplicate blots were

probed with 3F4, which detects both type 1 and type 2 PrPSc, and with 12B2,

which detects type 1. The insert shows a shorter exposure of the same time

course study from a separate experiment also probed with 3F4. Both blots

included samples of cerebral cortex from a case of sporadic CJD MM1 (Type

1) and molecular weight markers (Markers) indicate weights in kd.

Figure 7. A minority type 1-like PrPSc is found in vCJD tonsil, vCJD transmitted

to mice and in BSE. Western blot analysis of PrPSc in a concentrated

sample of tonsil from a case of vCJD (Tonsil), in a concentrated brain sample

of a wild-type mouse (C57BL) infected with vCJD and in a sample of cattle

BSE brain (BSE) is shown. Tissue extracts were digested with proteinase K.

Duplicate blots were probed with either 3F4 or 6H4, both of which detect

type 1 and type 2 PrPSc, and with 12B2, which detects type 1. The blots

included samples of cerebral cortex from a case of sporadic CJD MM1 (Type

1) and molecular weight markers (Markers) indicate weights in kd.

Type 1 PrPSc in Variant Creutzfeldt-Jakob Disease 155

AJP January 2006, Vol. 168, No. 1

cellular form of PrP and produce a protease-resistant

core fragment of PrPSc that differs in the extent of its

N-terminal truncation according to the original

conformation.

A complication has recently arisen with the finding that

both type 1 and type 2 can co-exist in the brains of

patients with sCJD.2,5-8 More recently this same phenomenon

has been demonstrated in patients with iatrogenically

acquired and familial forms of human prion disease.

9,10 The existence of this phenomenon is now

beyond doubt but its prevalence and its biological significance

remain a matter of debate.

Conventional Western blot analysis using antibodies

that detect type 1 and type 2 PrPSc has severe quantitative

limitations for the co-detection of type 1 and type 2

PrPSc in individual samples, suggesting that the prevalence

of co-occurrence of the two types might be underestimated.

We have sought to circumvent this problem by

using an antibody that is type 1-specific and applied this

to the sole remaining human prion disease where the

phenomenon of mixed PrPSc types has not yet been

shown, namely vCJD.

These results show that even in vCJD where susceptible

individuals have been infected supposedly by a

single strain of agent, both PrPSc types co-exist: a situation

reminiscent of that seen when similarly discriminant

antibodies were used to analyze experimental BSE in

sheep.14,17 In sporadic and familial CJD, individual

brains can show a wide range of relative amounts of the

two types in samples from different regions, but where

brains have been thoroughly investigated a predominant

type is usually evident.2,6,10 This differs from this report

on vCJD, where type 1 is present in all samples investigated

but always as a minor component that never

reaches a level at which it is detectable without a type

1-specific antibody. It would appear that the relative balance

between type 1 and type 2 is controlled within

certain limits in the vCJD brain. A minority type-1-like

band is also detected by 12B2 in vCJD tonsil, in BSE

brain and in the brains of mice experimentally infected

with vCJD, suggesting that this balance of types is agent,

rather than host or tissue, specific. Interestingly the "glycoform

signature" of the type 2 PrPSc found in vCJD (type

2B) is also seen in the type 1 PrPSc components, suggesting

that it could legitimately be termed type 1B.

PrPSc isotype analysis has proven to be extremely

useful in the differential diagnosis of CJD and is likely to

continue to have a major role in the investigation of human

prion diseases. However, it is clear, on the basis of

these findings, that molecular typing has quantitative limitations

and that any mechanistic explanation of prion

replication and the molecular basis of agent strain variation

must accommodate the co-existence of multiple

prion protein conformers. Whether or not the different

conformers we describe here correlate in a simple and

direct way with agent strain remains to be determined. In

principle two interpretations present themselves: either

the two conformers can be produced by a single strain of

agent or vCJD (and, therefore, presumably BSE) results

from a mixture of strains, one of which generally predominates.

Evidence for the isolation in mice of more than one

strain from individual isolates of BSE has been presented

previously.18,19

One practical consequence of our findings is that the

correct interpretation of transmission studies will depend

on a full examination of the balance of molecular types

present in the inoculum used to transmit disease, in addition

to a thorough analysis of the molecular types that

arise in the recipients. Another consequence relates to

the diagnostic certainty of relying on PrPSc molecular

type alone when considering the possibility of BSE infection

or secondary transmission in humans who have a

genotype other than methionine at codon 129 of the

PRNP gene. In this context it is interesting to note that this

minority type 1B component resembles the type 5 PrPSc

described previously to characterize vCJD transmission

into certain humanized PRNP129VV transgenic mouse

models.12,20 This apparently abrupt change in molecular

phenotype might represent a selection process imposed

by this particular transgenic mouse model. Irrespective of

whether this proves to be the case, the results shown

here point to further complexities in the relationship between

the physico-chemical properties of the prion protein,

human disease phenotype, and prion agent strain.

Acknowledgments

snip...

Type 1 PrPSc in Variant Creutzfeldt-Jakob Disease 157

AJP January 2006, Vol. 168, No. 1 ...TSS

http://ajp.amjpathol.org/cgi/content/abstract/168/1/151maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1136646133963_237&FIRSTINDEX=0&volume=168&issue=1&journalcode=amjpathol

Neuropathology and Applied Neurobiology

(2005),

31

, 565-579 doi: 10.1111/j.1365-2990.2005.00697.x

© 2005 Blackwell Publishing Ltd

565

Blackwell Science, LtdOxford, UKNANNeuropathology and Applied Neurobiology0305-1846Blackwell Publishing Ltd, 2005

316565579

Review article

Phenotypic variability in human prion diseases

J. W. Ironside, D. L. Ritchie and M. W. Head

National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, University of Edinburgh, Edinburgh, UK

J. W. Ironside, D. L. Ritchie and M. W. Head (2005)

Neuropathology and Applied Neurobiology

31,

565-579

Phenotypic variability in human prion diseases

Human prion diseases are rare neurodegenerative disorders

that can occur as sporadic, familial or acquired disorders.

Within each of these categories there is a wide range

of phenotypic variation that is not encountered in other

neurodegenerative disorders. The identification of the

prion protein and its key role in the pathogenesis of this

diverse group of diseases has allowed a fuller understanding

of factors that influence disease phenotype. In particular,

the naturally occurring polymorphism at codon 129

in the prion protein gene has a major influence on the disease

phenotype in sporadic, familial and acquired prion

diseases, although the underlying mechanisms remain

unclear. Recent technical advances have improved our

ability to study the isoforms of the abnormal prion protein

in the brain and in other tissues. This has lead to the concept

of molecular strain typing, in which different isoforms

of the prion protein are proposed to correspond to

individual strains of the transmissible agent, each with

specific biological properties. In sporadic Creutzfeldt-Jakob

disease there are at least six major combinations of codon

129 genotype and prion protein isotype, which appear to

relate to distinctive clinical subgroups of this disease.

However, these relationships are proving to be more complex

than first considered, particularly in cases with more

than a single prion protein isotype in the brain. Further

work is required to clarify these relationships and to

explain the mechanism of neuropathological targeting of

specific brain regions, which accounts for the diversity of

clinical features within human prion diseases.

© 2005 Blackwell Publishing Ltd, Neuropathology and Applied Neurobiology, 31, 565-579

BSE prions propagate as either variant CJD-like or

sporadic CJD-like prion strains in transgenic mice

expressing human prion protein

The EMBO Journal Vol. 21 No. 23 pp. 6358±6366, 2002

Emmanuel A.Asante, Jacqueline M.Linehan,

Melanie Desbruslais, Susan Joiner,

Ian Gowland, Andrew L.Wood, Julie Welch,

Andrew F.Hill, Sarah E.Lloyd,

Jonathan D.F.Wadsworth and

John Collinge1

MRC Prion Unit and Department of Neurodegenerative Disease,

Institute of Neurology, University College, Queen Square,

London WC1N 3BG, UK

1Corresponding author

e-mail: j.collinge@prion.ucl.ac.uk

Variant Creutzfeldt±Jakob disease (vCJD) has been

recognized to date only in individuals homozygous for

methionine at PRNP codon 129. Here we show that

transgenic mice expressing human PrP methionine

129, inoculated with either bovine spongiform

encephalopathy (BSE) or variant CJD prions, may

develop the neuropathological and molecular phenotype

of vCJD, consistent with these diseases being

caused by the same prion strain. Surprisingly, however,

BSE transmission to these transgenic mice, in

addition to producing a vCJD-like phenotype, can also

result in a distinct molecular phenotype that is indistinguishable

from that of sporadic CJD with PrPSc

type 2. These data suggest that more than one BSEderived

prion strain might infect humans; it is therefore

possible that some patients with a phenotype consistent

with sporadic CJD may have a disease arising

from BSE exposure.

snip...

These studies further strengthen the evidence that vCJD

is caused by a BSE-like prion strain. Also, remarkably, the

key neuropathological hallmark of vCJD, the presence of

abundant ¯orid PrP plaques, can be recapitulated on BSE

or vCJD transmission to these mice. However, the most

surprising aspect of the studies was the ®nding that an

alternate pattern of disease can be induced in 129MM

Tg35 mice from primary transmission of BSE, with a

molecular phenotype indistinguishable from that of a subtype

of sporadic CJD. This ®nding has important potential

implications as it raises the possibility that some humans

infected with BSE prions may develop a clinical disease

indistinguishable from classical CJD associated with type 2

PrPSc. This is, in our experience, the commonest molecular

sub-type of sporadic CJD. In this regard, it is of interest

that the reported incidence of sporadic CJD has risen in the

UK since the 1970s (Cousens et al., 1997). This has been

attributed to improved case ascertainment, particularly as

much of the rise is reported from elderly patients and

similar rises in incidence were noted in other European

countries without reported BSE (Will et al., 1998).

However, it is now clear that BSE is present in many

European countries, albeit at a much lower incidence than

was seen in the UK. While improved ascertainment is

likely to be a major factor in this rise, that some of these

additional cases may be related to BSE exposure cannot be

ruled out. It is of interest in this regard that a 2-fold

increase in the reported incidence of sporadic CJD in 2001

has recently been reported for Switzerland, a country that

had the highest incidence of cattle BSE in continental

Europe between 1990 and 2002 (Glatzel et al., 2002). No

epidemiological case±control studies with strati®cation of

CJD cases by molecular sub-type have yet been reported.

It will be important to review the incidence of sporadic

CJD associated with PrPSc type 2 and other molecular subtypes

in both BSE-affected and unaffected countries in the

light of these ®ndings. If human BSE prion infection can

result in propagation of type 2 PrPSc, it would be expected

that such cases would be indistinguishable on clinical,

pathological and molecular criteria from classical CJD. It

may also be expected that such prions would behave

biologically like those isolated from humans with sporadic

CJD with type 2 PrPSc. The transmission properties of

prions associated with type 2 PrPSc from BSE-inoculated

129MM Tg35 mice are being investigated by serial

passage.

We consider these data inconsistent with contamination

of some of the 129MM Tg35 mice with sporadic CJD

prions. These transmission studies were performed according

to rigorous biosafety protocols for preparation of

inocula and both the inoculation and care of mice, which

are all uniquely identi®ed by sub-cutaneous transponders.

However, crucially, the same BSE inocula have been used

on 129VV Tg152 and 129MM Tg45 mice, which are

highly sensitive to sporadic CJD but in which such

transmissions producing type 2 PrPSc were not observed.

Furthermore, in an independent experiment, separate

inbred lines of wild-type mice, which are highly resistant

to sporadic CJD prions, also propagated two distinctive

PrPSc types on challenge with either BSE or vCJD. No

evidence of spontaneous prion disease or PrPSc has been

seen in groups of uninoculated or mock-inoculated aged

129MM Tg35 mice.

While distinctive prion isolates have been derived from

BSE passage in mice previously (designated 301C and

301V), these, in contrast to the data presented here, are

propagated in mice expressing different prion proteins

(Bruce et al., 1994). It is unclear whether our ®ndings

indicate the existence of more than one prion strain in

individual cattle with BSE, with selection and preferential

replication of distinct strains by different hosts, or that

`mutation' of a unitary BSE strain occurs in some types of

host. Western blot analysis of single BSE isolates has not

shown evidence of the presence of a proportion of

monoglycosylated dominant PrPSc type in addition to the

diglycosylated dominant pattern (data not shown).

Extensive strain typing of large numbers of individual

BSE-infected cattle either by biological or molecular

methods has not been reported.

Presumably, the different genetic background of the

different inbred mouse lines is crucial in determining

which prion strain propagates on BSE inoculation. The

transgenic mice described here have a mixed genetic

background with contributions from FVB/N, C57BL/6 and

129Sv inbred lines; each mouse will therefore have a

different genetic background. This may explain the

differing response of individual 129MM Tg35 mice, and

the difference between 129MM Tg35 and 129MM Tg45

mice, which are, like all transgenic lines, populations

derived from single founders. Indeed, the consistent

distinctive strain propagation in FVB and C57BL/6 versus

SJL and RIIIS lines may allow mapping of genes relevant

to strain selection and propagation, and these studies are in

progress.

That different prion strains can be consistently isolated

in different inbred mouse lines challenged with BSE

prions argues that other species exposed to BSE may

develop prion diseases that are not recognizable as being

caused by the BSE strain by either biological or molecular

strain typing methods. As with 129MM Tg35 mice, the

prions replicating in such transmissions may be indistinguishable

from naturally occurring prion strains. It

remains of considerable concern whether BSE has transmitted

to, and is being maintained in, European sheep

¯ocks. Given the diversity of sheep breeds affected by

scrapie, it has to be considered that some sheep might have

become infected with BSE, but propagated a distinctive

strain type indistinguishable from those of natural sheep

scrapie. ...

The EMBO Journal Vol. 21 No. 23 pp. 6358±6366, 2002

6358 ãEuropean Molecular Biology Organization

http://embojournal.npgjournals.com/cgi/reprint/21/23/6358

J Neuropsychiatry Clin Neurosci 17:489-495, November 2005 doi: 10.1176/appi.neuropsych.17.4.489 © 2005 American Psychiatric Publishing, Inc.

Psychiatric Manifestations of Creutzfeldt-Jakob Disease: A 25-Year Analysis Christopher A. Wall, M.D., Teresa A. Rummans, M.D., Allen J. Aksamit, M.D., Lois E. Krahn, M.D. and V. Shane Pankratz, Ph.D. Received April 20, 2004; revised September 9, 2004; accepted September 13, 2004. From the Mayo Clinic, Department of Psychiatry and Psychology, Rochester, Minnesota; Mayo Clinic, Department of Neurology, Rochester, Minnesota. Address correspondence to Dr. Wall, Mayo Clinic, Department of Psychiatry and Psychology, Mayo Building-W11A, 200 First St., SW, Rochester, MN 55905; wall.chris@mayo.edu (E-mail).

This study characterizes the type and timing of psychiatric manifestations in sporadic Creutzfeldt-Jakob disease (sCJD). Historically, sCJD has been characterized by prominent neurological symptoms, while the variant form (vCJD) is described as primarily psychiatric in presentation and course: A retrospective review of 126 sCJD patients evaluated at the Mayo Clinic from 1976-2001 was conducted. Cases were reviewed for symptoms of depression, anxiety, psychosis, behavior dyscontrol, sleep disturbances, and neurological signs during the disease course. Eighty percent of the cases demonstrated psychiatric symptoms within the first 100 days of illness, with 26% occurring at presentation. The most commonly reported symptoms in this population included sleep disturbances, psychotic symptoms, and depression. Psychiatric manifestations are an early and prominent feature of sporadic CJD, often occurring prior to formal diagnosis.

snip...

CONCLUSIONS

Historically, psychiatric manifestations have been described as a relatively infrequent occurrence in the sporadic form of creutzfeldt-Jakob disease. However, our findings suggest otherwise. In this study, a vast majority of the cases were noted to have at least one psychiatric symptom during the course of illness, with nearly one-quarter occurring in the prodromal or presenting phase of the illness. After comparing the frequency of neuropsychiatric symptoms in sporadic CJD to studies describing the variant form of CJD, we found that there are fewer clinical differences than previously reported.5-7 While the age of patients with vCJD presentation is significantly younger and the course of illness is longer, the type and timing of psychiatric manifestations appear similar between these two diseases. ...snip...

http://neuro.psychiatryonline.org/cgi/content/abstract/17/4/489

Personal Communication

-------- Original Message --------

Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To: "'flounder@wt.net'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

e.asante@ic.ac.uk (until 9/12/02)

New e-mail: e.asante@prion.ucl.ac.uk (active from now)

____________________________________

Human Prion Protein with

Valine 129 Prevents Expression

of Variant CJD Phenotype

Jonathan D. F. Wadsworth, Emmanuel A. Asante,

Melanie Desbruslais, Jacqueline M. Linehan, Susan Joiner,

Ian Gowland, Julie Welch, Lisa Stone, Sarah E. Lloyd,

Andrew F. Hill,* Sebastian Brandner, John Collinge.

Variant Creutzfeldt-Jakob disease (vCJD) is a unique and highly distinctive

clinicopathological and molecular phenotype of human prion disease

associated with infection with bovine spongiform encephalopathy (BSE)-like

prions. Here, we found that generation of this phenotype in transgenic mice

required expression of human prion protein (PrP) with methionine 129.

Expression of human PrP with valine 129 resulted in a distinct phenotype and,

remarkably, persistence of a barrier to transmission of BSE-derived prions on

subpassage. Polymorphic residue 129 of human PrP dictated propagation of

distinct prion strains after BSE prion infection. Thus, primary and secondary

human infection with BSE-derived prions may result in sporadic CJD-like or

novel phenotypes in addition to vCJD, depending on the genotype of the prion

source and the recipient.

snip...

3 DECEMBER 2004 VOL 306 SCIENCE

http://www.sciencemag.org

Characterization of two distinct prion strains

derived from bovine spongiform encephalopathy

transmissions to inbred mice

Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais,

Susan Joiner, Jennifer Buckell, Sebastian Brandner,

Jonathan D. F. Wadsworth and John Collinge

Correspondence

John Collinge

j.collinge@prion.ucl.ac.uk

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology,

University College, London WC1N 3BG, UK

Received 9 December 2003

Accepted 27 April 2004

Distinct prion strains can be distinguished by differences in incubation period, neuropathology

and biochemical properties of disease-associated prion protein (PrPSc) in inoculated mice.

Reliable comparisons of mouse prion strain properties can only be achieved after passage in

genetically identical mice, as host prion protein sequence and genetic background are known

to modulate prion disease phenotypes. While multiple prion strains have been identified in

sheep scrapie and Creutzfeldt-Jakob disease, bovine spongiform encephalopathy (BSE) is

thought to be caused by a single prion strain. Primary passage of BSE prions to different lines

of inbred mice resulted in the propagation of two distinct PrPSc types, suggesting that two

prion strains may have been isolated. To investigate this further, these isolates were

subpassaged in a single line of inbred mice (SJL) and it was confirmed that two distinct prion

strains had been identified. MRC1 was characterized by a short incubation time (110±3 days),

a mono-glycosylated-dominant PrPSc type and a generalized diffuse pattern of PrP-immunoreactive

deposits, while MRC2 displayed a much longer incubation time (155±1 days),

a di-glycosylated-dominant PrPSc type and a distinct pattern of PrP-immunoreactive deposits

and neuronal loss. These data indicate a crucial involvement of the host genome in modulating

prion strain selection and propagation in mice. It is possible that multiple disease phenotypes

may also be possible in BSE prion infection in humans and other animals.

snip...

Journal of General Virology (2004), 85, 2471-2478 DOI 10.1099/vir.0.79889-0

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471

Medical Sciences Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco , and Maria Caramelli *

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease.

--------------------------------------------------------------------------------

C.C. and G.Z. contributed equally to this work.

To whom correspondence should be addressed.

E-mail: salvatore.monaco@mail.univr.it .

www.pnas.org/cgi/doi/10.1073/pnas.0305777101

snip...

Phenotypic Similarities Between BASE and sCJD. The transmissibility

of CJD brains was initially demonstrated in primates (27), and

classification of atypical cases as CJD was based on this property

(28). To date, no systematic studies of strain typing in sCJD have

been provided, and classification of different subtypes is based

on clinical, neuropathological, and molecular features (the polymorphic

PRNP codon 129 and the PrPSc glycotype) (8, 9, 15, 19).

The importance of molecular PrPSc characterization in assessing

the identity of TSE strains is underscored by several studies,

showing that the stability of given disease-specific PrPSc types is

maintained upon experimental propagation of sCJD, familial

CJD, and vCJD isolates in transgenic PrP-humanized mice (8,

29). Similarly, biochemical properties of BSE- and vCJDassociated

PrPSc molecules remain stable after passage to mice

expressing bovine PrP (30). Recently, however, it has been

reported that PrP-humanized mice inoculated with BSE tissues

may also propagate a distinctive PrPSc type, with a ''monoglycosylated-

dominant'' pattern and electrophoretic mobility of the

unglycosylated fragment slower than that of vCJD and BSE (31).

Strikingly, this PrPSc type shares its molecular properties with the

a PrPSc molecule found in classical sCJD. This observation is at

variance with the PrPSc type found in MV2 sCJD cases and in

cattle BASE, showing a monoglycosylated-dominant pattern but

faster electrophoretic mobility of the protease-resistant fragment

as compared with BSE. In addition to molecular properties

of PrPSc, BASE and MV2 sCJD share a distinctive pattern of

intracerebral PrP deposition, which occurs as plaque-like and

amyloid-kuru plaques. Differences were, however, observed in

the regional distribution of PrPSc. While inMV2 sCJD cases the

largest amounts of PrPSc were detected in the cerebellum,

brainstem, and striatum, in cattle BASE these areas were less

involved and the highest levels of PrPSc were recovered from the

thalamus and olfactory regions.

In conclusion, decoding the biochemical PrPSc signature of

individual human and animal TSE strains may allow the identification

of potential risk factors for human disorders with

unknown etiology, such as sCJD. However, although BASE and

sCJD share several characteristics, caution is dictated in assessing

a link between conditions affecting two different mammalian

species, based on convergent biochemical properties of diseaseassociated

PrPSc types. Strains of TSE agents may be better

characterized upon passage to transgenic mice. In the interim

until this is accomplished, our present findings suggest a strict

epidemiological surveillance of cattle TSE and sCJD based on

molecular criteria.

http://www.pnas.org/cgi/reprint/0305777101v1

Published online before print March 20, 2001, 10.1073/pnas.041490898

Neurobiology Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*

* Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and Institute for Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000 (received for review October 16, 2000)

Abstract

There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.

snip...

Characterization of the CJD and Scrapie Strains. Controls were set up by transmitting one French and one U.S. scrapie isolate from ruminants as well as French sCJD and iCJD cases from humans. None of these revealed a lesion profile or transmission characteristics similar or close to those of BSE or vCJD, respectively, thus extending to the present French scrapie isolate the previous observation that the BSE agent was different from all known natural scrapie strains (4, 24).

The lesion profiles of sCJD and iCJD differed only slightly in severity of the lesions, but not in shape of the profile, revealing the identity of the causative agents. One of us reported the absence of similarity between sCJD (six cases) and U.K. scrapie (eight cases) in transmission characteristics in mice (4). Herein, we made the striking observation that the French natural scrapie strain (but not the U.S. scrapie strain) has the same lesion profile and transmission times in C57BL/6 mice as do the two human TSE strains studied. This strain "affiliation" was confirmed biochemically. There is no epidemiological evidence for a link between sheep scrapie and the occurrence of CJD in humans (25). However, such a link, if it is not a general rule, would be extremely difficult to establish because of the very low incidence of CJD as well as the existence of different isolates in humans and multiple strains in scrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there is still a possibility that in some instances TSE strains infecting humans do share a common origin with scrapie, as pointed out by our findings.

snip...

http://www.pnas.org/cgi/content/full/041490898v1

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

NOTES

Interspecies Transmission of Chronic Wasting Disease Prions to

Squirrel Monkeys (Saimiri sciureus)

Richard F. Marsh,1? Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4*

Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 537061; Department of

Physical Therapy2 and Department of Medical Microbiology and Immunology,4 Creighton University, Omaha,

Nebraska 68178; and Department of Veterinary Molecular Biology, Montana

State University, Bozeman, Montana 597183

Received 3 May 2005/Accepted 10 August 2005

Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission

to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman

primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The

CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at

31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal

isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported

transmission of CWD to primates.

snip...

JOURNAL OF VIROLOGY, Nov. 2005, p. 13794-13796 Vol. 79, No. 21

0022-538X/05/$08.00!0 doi:10.1128/JVI.79.21.13794-13796.2005

Copyright © 2005, American Society for Microbiology. All Rights Reserved.

=============================================

The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000 © European Molecular Biology Organization

Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3, L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M. Smits2 and B. Caughey1,7

1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840, 3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL, Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences, University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad, Institute for Animal Science and Health, Lelystad, The Netherlands 7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000; revised July 3, 2000; accepted July 5, 2000.

Abstract

Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of deer and elk, and little is known about its transmissibility to other species. An important factor controlling interspecies TSE susceptibility is prion protein (PrP) homology between the source and recipient species/genotypes. Furthermore, the efficiency with which the protease-resistant PrP (PrP-res) of one species induces the in vitro conversion of the normal PrP (PrP-sen) of another species to the protease-resistant state correlates with the cross-species transmissibility of TSE agents. Here we show that the CWD-associated PrP-res (PrPCWD) of cervids readily induces the conversion of recombinant cervid PrP-sen molecules to the protease-resistant state in accordance with the known transmissibility of CWD between cervids. In contrast, PrPCWD-induced conversions of human and bovine PrP-sen were much less efficient, and conversion of ovine PrP-sen was intermediate. These results demonstrate a barrier at the molecular level that should limit the susceptibility of these non-cervid species to CWD.

snip...

Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml

Neurology 1999;52:1757 © 1999 American Academy of Neurology

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Expedited Publication

A subtype of sporadic prion disease mimicking fatal familial insomnia P. Parchi, MD, S. Capellari, MD, S. Chin, MD, PhD, H. B. Schwarz, MD, N. P. Schecter, MD, J. D. Butts, MD, P. Hudkins, MD, D. K. Burns MD, J. M. Powers, MD and P. Gambetti, MD

http://www.neurology.org/cgi/content/abstract/52/9/1757

Transfusion Volume 43 Issue 12 Page 1687 - December 2003 doi:10.1046/j.0041-1132.2003.00586.x

Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathy Larisa Cervenakova, Oksana Yakovleva, Carroll McKenzie, Svetlana Kolchinsky, Lisa McShane, William N. Drohan, and Paul Brown

BACKGROUND:

The possible transmission of variant CJD (vCJD) through blood transfusion or use of plasma-derived products prompted this study comparing infectivity in murine models of vCJD and Gerstmann-Sträussler-Scheinker (GSS) disease, a non-vCJD form of transmissible spongiform encephalopathy (TSE).

STUDY DESIGN AND METHODS:

RIII/Fa/Dk (RIII) or Swiss-Webster (Swiss) mice were inoculated intracerebrally (IC) with mouse-adapted strains of vCJD or GSS (Fukuoka-1) of similar infectivity. Groups of RIII mice were euthanized 17 weeks after inoculation (during the incubation period), and another 23 weeks after inoculation (when symptomatic). Blood was collected, separated into components, and inoculated into groups of healthy mice; brains and spleens from all mice were harvested and tested for the presence of PrPres by Western blot using 6H4 MoAb.

RESULTS:

Levels of 20-30 infectious doses per mL were present in buffy coat and plasma during both the incubation and symptomatic stages of disease; PLT pellet infectivity was lower (10 ID/mL) and RBCs were not infectious. The disease was transmitted more efficiently by IV than IC inoculation of plasma, but there was no difference observed with inoculation of buffy coat. The incubation period was shorter after IC inoculation of GSS- than vCJD-brain inocula. The amount of PrPres in spleens was similar for both TSE agents, but was slightly lower in brains of vCJD than GSS mice.

CONCLUSION:

Infectivity was detected in blood components of mice infected with a human-derived strain of vCJD during both the preclinical and clinical phases of disease in a similarly low range of concentrations as in mice infected with a human-derived nonvariant strain (GSS, Fukuoka-1). Other measures of virulence, including brain infectivity titers, incubation periods, and the accumulation of PrPres in spleens and brains, were also comparable in both experimental models.

http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0041-1132&date=2003&volume=43&issue=12&spage=1687

http://www.blackwell-synergy.com/doi/abs/10.1046/j.0041-1132.2003.00586.x

SEAC

POSITION STATEMENT

EARLY PHASE OF vCJD INFECTION IN BLOOD TRANSFUSION

RECIPIENTS

http://www.seac.gov.uk/pdf/cjd.pdf

SEAC

Summary of SEAC's discussion on the second presumed case of blood

transfusion-associated infection with vCJD

http://www.seac.gov.uk/statements/statement070804.pdf

Transmission of Creutzfeldt-Jakob Disease from Blood and Urine Into Mice

The Lancet, November 9, 1985

Sir,--Professor Manuelidis and his colleagues (Oct 19, p896) report transmission to animals of Creutzfeldt-Jakob disease (CJD) from the buffy coat from two patients. We also transmitted the disease from whole blood samples of a patient (and of mice) infected with CJD.1 Brain, Cornea, and urine from this patient were also infectious, and the clinicopathological findings2 are summarised as follows.

A 70-year-old man was noted to have a slowing of speech and writing and some disorientation, all of which progressed rapidly. Decorticate rigidity, forced grasping, positive snout reflex, and myoclonus appeared within 2 months. Electroencephalogram revealed typical periodic synchronous discharge, and he died of pneumonia and upper gastrointestinal haemorrhage, about 3 months after onset of the symptoms. The Brain weighed 1290g and showed severe histological changes diagnostic of CJD, including spongiform change, loss of nerve cells, and diffuse proliferation of astrocytes. There were no inflammatory cells, microglia, neurofibrillary tangles, and amyloid plaques, although virus-like particles were detected by electron microscopy.

Results of innoculation in Mice

Inocula NO* Incubation period (days)+ Brain 7/10 (4) 789 (+ or - 112) Cornea 1/6 (0) 1037 Blood 2/13 (0) 1080 (+ or - 69) Urine 5/10 (1) 880 (+ or - 55) CSF 0/10

* Number of mice with CJD change/number examined histologically. Number with amyloid plaques shown in parentheses.

+ means + or - SD

Samples were taken aseptically at necropsy. 10% crude homogenates of brain and cornea in saline, whole blood (after crushing a clot), and untreated CSF and urine were innoculated intracerebrally into CF1 strain mice (20 ul per animal). Some mice showed emaciation, bradykinesia, rigidity of the body and tail, and sometimes tremor after long incubation periods. Tissues obtained after the animal died (or was killed) were studied histologically (table). Animals infected by various inocula showed common pathological changes, consisting of severe spongiform changes, glial proliferation, and a moderate loss of nerve cells. A few mice inoculated with brain tissue or urine had the same amyloid plaques found in patients and animals with CJD.3

In our long-term experiments, inoculating materials taken from twenty patients with CJD or Gerstmann-Straussler-Scheinker's disease (GSS) into rodents, positive results were obtained in seventeen cases, including this patient. Brain tissue transmitted the disease most frequently within the shortes incubation period, except for one case where the lymph node was the most infectious. Transmission through the cornea has been noted in man4 and in guineapigs.5 Whole blood samples taken from three patients were inoculated and a positive transmission occured only in the case recorded here. Mouse-to-mouse transmission through blood inoculation was successful after a mean incubation period of 365 days.1 Transmission through urine was positive in this patient only, and negative in one other patient and in many infected animals. Transmission through the CSF from eight patients was negative, yet transmission via the CSF of infected rats was positive.1

As viraemia has been proved in guineapigs,6 mice,1,7 and lately in patients with CJD, blood for transfusion or blood products for medical use must be tested for unconventional pathogens. For this purpose, we inoculated blood products inot rodents.8 The CJD pathogen was not found in the products examined. However, this approach takes too long to be of practical value. More efficient methods must be developed to detect pathogens and to eliminate them from blood. One proposal9 is to apply membrane filtration to the pruification protocol of human growth hormone suspected of being contaminated with CJD. Similar methods are needed for blood contamination.

Department of Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka812, Japan

JUN TATEISHI

1. Tateishi J, Sato Y, Kaga M. Doi H, Ohta M. Experimental transmission of human subacute spongiform encephalopathy to small rodents 1: Clinical and histological observations. Acta Neuropathol (Berl) 1980; 51: 127.

2. Shibayama Y, Sakaguchi Y, Nakata K, et al, Creutzfeldt-Jakob disease with demonstration of virus-like particles. Acta pathol Jpn 1982;32: 695.

3. Tateishi J, Nagara H, Hikita K, Sato Y. Amyloid plaques in the brains of mice with Creutzfeldt-Jakob disease. Ann Neurol 1984; 15: 278.

4. Duffy P, Wolf J, Colings G, DeVoe AG, Streeten B, Cowen D. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974; 290: 692.

5. Manuelidis EE, Angelo JN, Gorgacz EJ, Kim JH, Manuelidis L. Experimental Creutzfeldt-Jakob disease transmitted via the eye with infected cornea. N Engl J Med 1977; 296: 1334.

6. Manuelidis EE, Gorgacz EJ, Manuelidis L. Viremia in experimental Creutzfeldt-Jakob disease. Science 1978: 200: 1069.

7. Kuroda Y, Gibbs CJ Jr, Amyx HL, Gajdusek DC. Creutzfeldt-Jakob disease in mice. Persistent viremiam and preferential replication of virus in low-density lymphocytes. Infect Immun 1983; 41: 154.

8. Tateishi J, Tsuji S. Unconventional pathogens causing spongiform encephalopathis absent in blood products. J Med Virol 1985; 15: 11.

9. Tateishi J, Kitamoto T, Hiratani H. Creutzfeldt-Jakob disease pathogen in growth hormone preparations is eliminatable. Lancet (in press).

http://www.thelancet.com/

LANCET INFECTIOUS DISEASE JOURNAL

Volume 3, Number 8 01 August 2003

Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

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.

49-year-old Singeltary is one of a number of people who have remained

largely unsatisfied after being told that a close relative died from a

rapidly progressive dementia compatible with spontaneous

Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

encephalopathy (BSE), Americans might get a similar disorder from

chronic wasting disease (CWD) the relative of mad cow disease seen among

deer and elk in the USA. Although his feverish search did not lead him

to the smoking gun linking CWD to a similar disease in North American

people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralised at the few attempts to monitor the

occurrence of CJD and CWD in the USA. Only a few states have made CJD

reportable. Human and animal TSEs should be reportable nationwide and

internationally, he complained in a letter to the Journal of the

American Medical Association (JAMA 2003; 285: 733). I hope that the CDC

does not continue to expect us to still believe that the 85% plus of all

CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small

region in Colorado. But since early 2002, it has been reported in other

areas, including Wisconsin, South Dakota, and the Canadian province of

Saskatchewan. Indeed, the occurrence of CWD in states that were not

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

lymphoid tissue before invasion of the central nervous system. Yet the

plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD

is only reported in those areas known to be endemic foci of CWD.

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

prohibiting material from CWD-positive animals from being used as an

ingredient in feed for any animal species, epidemiological control and

research in the USA has been quite different from the situation in the

UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling

teeth, Singeltary argues. You get it when they want you to have it,

and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunters two of whom were friends who died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very

limited.

http://infection.thelancet.com/journal/journal.isa

he complained in a letter to the Journal of the American Medical

Association (JAMA 2003; 285: 733). I hope that the CDC does not

continue to expect us to still believe that the 85% plus of all CJD

cases which are sporadic are all spontaneous, without route or source.<<< 8="includes" 1="3" 6="7" humans =" sCJD" 2005 =" 0.69" safety ="="="="="="="="="="="="="="="="" b =" atypical" c =" case" ip ="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="" 2001 ="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="="">104-fold range. The CDI has been automated, which not only improves accuracy and reproducibility (10) but also allows numerous samples to be analyzed, as reported here. Western blots are difficult to automate and are labor intensive.

Our studies show that only the CDI detected PrPSc in all regions examined in 24 sCJD and 3 fCJD(E200K) brains (Figs. 2 and 6). Comparative analyses demonstrated that the CDI was vastly superior to histology and IHC. When 18 regions of 8 sCJD and 2 fCJD(E200K) brains were compared, we discovered that histology and IHC were unreliable diagnostic tools except for samples from a few brain regions. In contrast, the CDI was a superb diagnostic procedure because it detected PrPSc in all 18 regions in 8 of 8 sCJD and 2 of 2 fCJD(E200K) cases (Tables 1 and 2).

Histologic changes in prion disease have been shown to follow the accumulation of prions as measured by bioassay of infectivity and by PrPSc accumulation (18-22). Because low levels of PrPSc are not associated with neuropathologic changes, some discrepancy between vacuolation and PrPSc was expected. In contrast to histology, IHC measures PrP immunostaining after autoclaving tissue sections exposed to formic acid. Because IHC measures PrP, we expected the sensitivity of this procedure might be similar to the CDI, but that proved not to be the case. Whether exposure of formic acid-treated tissue sections to elevated temperature destroys not only PrPC but also sPrPSc and only denatures rPrPSc remains to be determined. Such a scenario could account for the lower sensitivity of IHC compared with CDI or bioassay (Tables 1 and 2).

Studies of the white matter in CJD brains were particularly informative with respect to the sensitivity of the CDI, where PrPSc levels were low but readily detectable, 10- to 100-fold above the threshold value (Fig. 4). Because animal studies have shown that PrPSc and infectivity are transported anterogradely from one brain region to another along neuroanatomical pathways (23-25), we expected to find PrPSc in white matter as demonstrated by the CDI but not IHC. Axonal transport of PrPSc is also suggested by diffusion-weighted MRI scans of CJD cases, which show high-intensity signals in analogous neocortical regions of the right and left cerebral hemispheres (26). This symmetry of neuroradiological abnormalities is consistent with spread of PrPSc to the contralateral cortex by means of callosal commissural pathways.

Most immunoassays that detect HuPrPSc do so only after subjecting the sample to limited proteolysis to form PrP 27-30, followed by denaturation. Because the CDI measures the immunoreactivity before and after denaturation to an epitope that is exposed in native PrPC but buried in PrPSc, limited proteolysis to eliminate PrPC is unnecessary. Assays based on limited proteolysis underestimate the level of PrPSc because they digest sPrPSc, which represents 80-90% of PrPSc in CJD and scrapie brains (Fig. 4 and Table 5).

Gerstmann-Sträussler-Scheinker, an inherited human prion disease, is caused by the P102L mutation in the PRNP gene. In mice expressing the Gerstmann-Sträussler-Scheinker mutant PrP transgene, the CDI detected high levels of sPrPSc(P101L) as well as low levels of rPrPSc(P101L) long before neurodegeneration and clinical symptoms occurred (9). sPrPSc(P101L) as well as low concentrations of rPrPSc(P101L) previously escaped detection (27). Whether a similar situation applies in other genetic forms of prion disease, sCJD, or variant CJD remains to be determined. Because most of the PrPSc in the brains of sCJD patients is protease-sensitive (Fig. 4), it is likely that the lower sensitivity of IHC is due to its inability to detect sPrPSc. Presently, we have no information about the kinetics of either sPrPSc or rPrPSc accumulation in human brain. Limited information on the kinetics of PrPSc accumulation in livestock comes from studies of cattle, sheep, and goats inoculated orally, but most of the bioassays were performed in non-Tg mice (28-30) in which prion titers were underestimated by as much as a factor of 104 (10).

The studies reported here are likely to change profoundly the approach to the diagnosis of prion disease in both humans and livestock (31-33). The superior performance of the CDI in diagnosing prion disease compared to routine neuropathologic examination and IHC demands that the CDI be used in future diagnostic evaluations of prion disease. Prion disease can no longer be ruled out by routine histology or IHC. Moreover, the use of IHC to confirm cases of bovine spongiform encephalopathy after detection of bovine PrPSc by the CDI (10) seems an untenable approach in the future. Clearly, the CDI for HuPrPSc is as sensitive or more sensitive than bioassays in Tg(MHu2M) mice (Fig. 1).

Our results suggest that using the CDI to test large numbers of samples for human prions might alter the epidemiology of prion diseases. At present, there is limited data on the frequency of subclinical variant CJD infections in the U.K. population (34). Because appendixes and tonsils were evaluated only by IHC, many cases might have escaped detection (Tables 1 and 2). Equally important may be the use of CDI-like tests to diagnose other neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease, and the frontotemporal dementias. Whether IHC underestimates the incidence of one or more of these common degenerative diseases is unknown. Moreover, CDI-like tests may help determine the frequency with which these disorders and the prion diseases occurs concomitantly in a single patient (35, 36).

Acknowledgements

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http://www.pnas.org/

Volume 349:1812-1820 November 6, 2003 Number 19

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

Background In patients with sporadic Creutzfeldt-Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt-Jakob disease is important for classification and diagnosis and perhaps even for prevention.

Methods We used a highly sensitive method of detection - involving the concentration of PrPSc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude - to search for PrPSc in extraneural organs of 36 patients with sporadic Creutzfeldt-Jakob disease who died between 1996 and 2002.

Results PrPSc was present in the brain tissue of all patients. In addition, we found PrPSc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrPSc in both spleen and muscle specimens. Patients with extraneural PrPSc had a significantly longer duration of disease and were more likely to have uncommon molecular variants of sporadic Creutzfeldt-Jakob disease than were patients without extraneural PrPSc.

Conclusions Using sensitive techniques, we identified extraneural deposition of PrPSc in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt-Jakob disease. Extraneural PrPSc appears to correlate with a long duration of disease.

Source Information

From the Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital of Zurich, Zurich, Switzerland.

Dr. Glatzel and Mr. Abela contributed equally to the article.

Address reprint requests to Dr. Aguzzi at the Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12, CH-8091 Zurich, Switzerland, or at adriano@pathol.unizh.ch .

http://content.nejm.org/cgi/

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A. Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano Aguzzi, MD, PhD 6, Herbert Budka, MD 1 * 1Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria 2National Institute of Psychiatry and Neurology, Budapest, Hungary 3Department of Pathology, School of Medicine, Federal University of Rio de Janeiro 4Department of Neurology, School of Medicine, Federal University of Rio de Janeiro 5Department of Neurology, School of Medicine, Federal University of Sao Paulo, Brazil 6Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland email: Herbert Budka (h.budka@akh-wien.ac.at )

*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J, Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by: European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523 EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837

Abstract

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrPSc in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become prominent when PrPC is abundantly available as substrate, as in inclusion body myositis muscle.

--------------

Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11 September 2003 Digital Object Identifier (DOI)

10.1002/ana.10813 About DOI

http://www3.interscience.wiley.com/

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please note;

EPA's EDOCKET has been migrated into a federal-wide system to better serve citizens EDOCKET Replaced by FDMS

As of Friday, November 25, 2005 at 8 am, EDOCKET became permanently unavailable.

Transfusion Volume 43 Page 1687 - December 2003 doi:10.1046/j.0041-1132.2003.00586.xVolume 43 Issue 12 Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathyLarisa Cervenakova, Oksana Yakovleva, Carroll McKenzie, Svetlana Kolchinsky, Lisa McShane, William N. Drohan, and Paul Brown

BACKGROUND: The possible transmission of variant CJD (vCJD) through blood transfusion or use of plasma-derived products prompted this study comparing infectivity in murine models of vCJD and Gerstmann-Sträussler-Scheinker (GSS) disease, a non-vCJD form of transmissible spongiform encephalopathy (TSE).

STUDY DESIGN AND METHODS: RIII/Fa/Dk (RIII) or Swiss-Webster (Swiss) mice were inoculated intracerebrally (IC) with mouse-adapted strains of vCJD or GSS (Fukuoka-1) of similar infectivity. Groups of RIII mice were euthanized 17 weeks after inoculation (during the incubation period), and another 23 weeks after inoculation (when symptomatic). Blood was collected, separated into components, and inoculated into groups of healthy mice; brains and spleens from all mice were harvested and tested for the presence of PrPres by Western blot using 6H4 MoAb.

RESULTS: Levels of 20-30 infectious doses per mL were present in buffy coat and plasma during both the incubation and symptomatic stages of disease; PLT pellet infectivity was lower (10 ID/mL) and RBCs were not infectious. The disease was transmitted more efficiently by IV than IC inoculation of plasma, but there was no difference observed with inoculation of buffy coat. The incubation period was shorter after IC inoculation of GSS- than vCJD-brain inocula. The amount of PrPres in spleens was similar for both TSE agents, but was slightly lower in brains of vCJD than GSS mice.

CONCLUSION: Infectivity was detected in blood components of mice infected with a human-derived strain of vCJD during both the preclinical and clinical phases of disease in a similarly low range of concentrations as in mice infected with a human-derived nonvariant strain (GSS, Fukuoka-1). Other measures of virulence, including brain infectivity titers, incubation periods, and the accumulation of PrPres in spleens and brains, were also comparable in both experimental models.

http://www.blackwell-synergy.com/doi/abs/10.1046/j.0041-1132.2003.00586.x

Subject: SCRAPIE USA REPORT UPDATE AS AT NOVEMBER 30, 2005 Date: January 12, 2006 at 11:29 am PST SCRAPIE USA REPORT UPDATE AS AT NOVEMBER 30, 2005

Infected and Source Flocks

As of November 30, 2005 there were 95 scrapie infected and source flocks (Figure 3). There were 2 new infected and source flocks reported in November (Figure 4) with a total of 12 flocks reported for FY 2006 (Figure 5). The total infected and source flocks that have been released in FY 2006 are 16 (Figure 6), with 9 flocks released in November. The ratio of infected and source flocks released to newly infected and source flocks for FY 2006 = 1.33 : 1. In addition, as of November 30, 2005, 67 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 7 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in November 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005. New infected flocks, source flocks, and flocks released for FY 2006 are depicted in Figure 10. New infected and source statuses from 1997 to 2006 are depicted in Chart 3.

Regulatory Scrapie Slaughter Surveillance (RSSS) +

RSSS started April 1, 2003. It is targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. Samples have been collected from 67,840 sheep since April 1, 2003, of which results have been reported for 64,034. Samples have been submitted from 81 plants. There have been 215 NVSL confirmed positive sheep since the beginning of RSSS. In FY 2006 samples have been collected from 5,339 sheep and there have been 7 NVSL confirmed positive cases through November 2005. Face colors of FY 2006 confirmed positives are 6 black and 1 mottled. During November 2005, 2,429* animals were sampled and test results were reported on 3088 samples. Five confirmed positives were reported by NVSL in November 2005. Cumulative regional sample collection numbers are shown in Figure 11 and are based upon the State in which the animal was tagged. The number of RSSS animals collected with traceable identification for FY 2005 by month, by region where collected is shown in Figure 12. A retrospective 6 month rolling average of the % positive tested black-faced sheep sampled at slaughter is shown in Figure 13.

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full text;

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html

Published online before print October 20, 2005

Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102 Medical Sciences

A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

( sheep prion transgenic mice )

Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte , Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude * *Virologie Immunologie Moléculaires and Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. 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.

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Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper.

A.L.D. and V.B. contributed equally to this work.

To whom correspondence should be addressed.

Hubert Laude, E-mail: laude@jouy.inra.fr

www.pnas.org/cgi/doi/10.1073/pnas.0502296102

http://www.pnas.org/cgi/content/abstract/0502296102v1

12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.

The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.

It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.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

03-025IFA 03-025IFA-2 Terry S. Singeltary

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally, OF ALL AGES...TSS

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

----- Original Message ----- From: Terry S. Singeltary Sr. To: science_editors@aaas.org Sent: Tuesday, December 06, 2005 9:57 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

----- Original Message ----- From: Terry S. Singeltary Sr. To: sdavies@bmj.com Sent: Thursday, December 22, 2005 3:01 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Cc: Sent: Tuesday, January 17, 2006 2:35 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease UKBSEnvCJD only ? (JAMA Feedback Form)

----- Original Message ----- From: Terry S. Singeltary Sr. To: PNASnews@nas.edu Cc: pnas@nas.edu Sent: Tuesday, January 31, 2006 2:58 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow

I wish to submit the following to PNAS for publication. NO other authors except me. thank you. ///

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Thursday, August 02, 2007 12:22 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

----- Original Message ----- From: Terry S. Singeltary Sr. To: office@jsvs.or.jp Cc: zsocj@a1.rimnet.ne.jp Sent: Sunday, April 02, 2006 2:50 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory.doc

----- Original Message ----- From: Terry S. Singeltary Sr. To: plosone@plos.org Sent: Tuesday, October 03, 2006 11:02 AM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory.doc

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Cc: Sent: Wednesday, December 21, 2005 10:52 PM Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease the UKBSEnvCJD theory (JAMA Feedback Form)

can't say i am not persistent ;-). ...end...June 2008...TSS

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

A novel human disease with abnormal prion protein sensitive to protease (prionopathy)

2008-06-20T19:16:00.000-07:00

Original Article

A novel human disease with abnormal prion protein sensitive to protease

Pierluigi Gambetti, MD 1 *, Zhiqian Dong, PhD 1, Jue Yuan, BA 1, Xiangzhu Xiao, PhD 1, Mengjie Zheng, PhD 1, Amer Alshekhlee, MD 1, Rudy Castellani, MD 2, Mark Cohen, MD 1, Marcelo A. Barria, PhD 3, D. Gonzalez-Romero, PhD 3, Ermias D. Belay, MD 4, Lawrence B. Schonberger, MD, MPH 4, Karen Marder, MD 5, Carrie Harris, BA 1, James R. Burke, MD, PhD 6, Thomas Montine, MD 7, Thomas Wisniewski, MD 8, Dennis W. Dickson, MD 9, Claudio Soto, PhD 3, Christine M. Hulette, MD 10, James A. Mastrianni, MD, PhD 11, Qingzhong Kong, PhD 1, Wen-Quan Zou, MD, PhD 1 * 1Institute of Pathology, Case Western Reserve University, Cleveland, OH 2Department of Pathology, University of Maryland, Baltimore, MD 3Department of Neurology, Neuroscience and Cell Biology, George and Cynthia Mitchell Center for Neurodegenerative Diseases, University of Texas Medical Branch, Galveston, TX 4Centers for Disease Control and Prevention, Atlanta, GA 5Department of Neurology, Columbia University, New York, NY 6Department of Medicine, Division of Neurology, Duke University, Durham, NC 7Harborview Medical Center, University of Washington, Seattle, WA 8Department of Neurology, New York University, New York, NY 9Department of Neuropathology, Mayo Clinic College of Medicine, Jacksonville, FL 10Department of Pathology, Duke University, Durham, NC 11Department of Neurology, University of Chicago, Chicago, IL

email: Pierluigi Gambetti (pxg13@case.edu) Wen-Quan Zou (wenquan.zou@case.edu)

*Correspondence to Pierluigi Gambetti, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106

*Correspondence to Wen-Quan Zou, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106

Funded by: NIH; Grant Number: AG14359, AG08702, NS049173 Centers for Disease Control and Prevention; Grant Number: CCU 515004 Britton Fund CJD Foundation

Abstract

Objective To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) that, contrary to the common prion diseases, is predominantly sensitive to protease digestion.

Methods Eleven subjects were investigated at the National Prion Disease Pathology Surveillance Center for clinical, histopathological, immunohistochemical, genotypical, and PrP characteristics.

Results Patients presented with behavioral and psychiatric manifestations on average at 62 years, whereas mean disease duration was 20 months. The type of spongiform degeneration, the PrP immunostaining pattern, and the presence of microplaques distinguished these cases from those with known prion diseases. Typical protease-resistant PrP was undetectable in the cerebral neocortex with standard diagnostic procedures. After enrichment, abnormal PrP was detected at concentrations 16 times lower than common prion diseases; it included nearly 4 times less protease-resistant PrP, which formed a distinct electrophoretic profile. The subjects examined comprised about 3% of sporadic cases evaluated by the National Prion Disease Pathology Surveillance Center. Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.

Interpretation The distinct histopathological, PrP immunohistochemical, and physicochemical features, together with the homogeneous genotype, indicate that this is a previously unidentified type of disease involving the PrP, which we designated protease-sensitive prionopathy (or PSPr). Protease-sensitive prionopathy is not rare among prion diseases, and it may be even more prevalent than our data indicate because protease-sensitive prionopathy cases are likely also to be classified within the group of non-Alzheimer's dementias. Ann Neurol 2008;63:697-708

-------------------------------------------------------------------------------- Received: 5 November 2007; Revised: 1 April 2008; Accepted: 4 April 2008 Digital Object Identifier (DOI)

10.1002/ana.21420 About DOI

http://www3.interscience.wiley.com/journal/119883040/abstract

Pages: 677-678 A new prionopathy Robert Will, Mark Head http://www3.interscience.wiley.com/cgi-bin/abstract/119882940/ABSTRACT

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

http://brain.hastypastry.net/forums/showthread.php?t=15076

http://brain.hastypastry.net/forums/archive/index.php/t-17057.html

sporadic CJD, the big lie

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Conclusions

We believe that our observation of a previously unrecognised variant of CJD occurring, to date, only in persons under the age of 45 years is a cause for great concern. That it is due to exposure to the BSE agent is perhaps the most plausible interpretation of our findings. However, we emphasise that we do not have direct evidence of such a link and other explanations are possible. That these cases have been observed now because of improved ascertainment cannot be completely dismissed. It seems unlikely, however, that such a distinctive neuropathological pattern would have been missed previously, especially among persons dying at a young age. It is essential to obtain information on the clinical and neuropathological characteristics of young patients with CJD in Europe and elsewhere, and historically in the UK, but proof of an association between BSE and CJD may depend on animal transmission studies and continued epidemiological vigilance. If there is a causal link then, given the potentially long and widespread exposure to the BSE agent, further cases of this new variant of CJD are likely to arise.

We thank J Mackenzie for data management, P Brown for reviewing an early version of the manuscript, J Collinge for assistance with the molecular analysis, and W B Matthews who initiated CJD surveillance in the UK in the 1980 for advice. The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home and Health Department and suported by BBSRC (grant no 15/BS204814). The Concerted Action on CJD Surveillance in Europe was funded through the EC Biomed I Programme. The epidemiological surveillance of CJD would not be possible without the collaboration of neurologists and neuropathologists throughout the UK and Europe.

References

snip.....

http://www.cjd.ed.ac.uk/lancet.htm

sporadic CJD, the big lie

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk.

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

snip...

Sporadic creutzfeldt-jakob disease in two adolescents

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

see full text sporadic CJD the big lie;

Subject: Sporadic creutzfeldt-jakob disease in two adolescents From: "Terry S. Singeltary Sr." <[log in to unmask]> Reply-To: Sustainable Agriculture Network Discussion Group <[log in to unmask]> Date: Mon, 28 May 2007 10:25:25 -0500 Content-Type: text/plain Parts/Attachments: text/plain (946 lines)

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: [log in to unmask]

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

http://www.bseinquiry.gov.uk/files/yb/1992/08/13002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21005001.pdf

CONFIRMED CJD IN FARMER WITH BSE COW

line to take, sporadic CJD

http://www.bseinquiry.gov.uk/files/yb/1992/10/22004001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05003001.pdf

SECOND CASE CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/00/00001001.pdf

CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE

ii. on page 2 the sentence ''He had drunk pooled milk from the herd which included that from the affected animal'' will mislead the uninformed. It needs to be made clear that milk from a cow which is suspected to be affected with BSE cannot be drunk or added to the bulk milk produced by the rest of the herd.

iii. in the final paragraph I suggest that the phrase ''and a causal link with BSE is at most conjectural'' BE DELETED: the first paragraph of the sentence would then stand as a clear statement that the CJD case was likely to have been a CHANCE PHENOMENON.

http://www.bseinquiry.gov.uk/files/yb/1993/02/15003001.pdf

''DH is aware of a second case of CJD in a dairy farmer who has had BSE in his herd. We cannot comment on the details of the case, but we know of nothing to suggest this is anthing other than a sporadic case of CJD. .........

http://www.bseinquiry.gov.uk/files/yb/1993/07/12001001.pdf

IF PRESSED:

The numbers concerned are very small, and it is not possible to draw any conclusions from such small numbers. This issue is being considered by the Government's expert advisers....

http://www.bseinquiry.gov.uk/files/yb/1993/07/12002001.pdf

THE FARMER IS THOUGHT TO HAVE HAD AT LEAST TWO CASES OF BSE IN HIS HERD, which were diagnosed in 1992. The farmer is reported to have asssisted in calving and to have drunk milk from his herd. This does not suggest that this is anything other than a sporadic case of CJD. ...

http://www.bseinquiry.gov.uk/files/yb/1993/07/12003001.pdf

CONFIDENTIAL

CONFIRMED CASE OF CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/07/14003001.pdf

3. Neither Dr Will nor the CJD surveillance unit intend to disclose the existence of this case or make any comment at present unless it attracts media attention.

snip...

HUMAN CASE DETAILS CONFIDENTIAL

snip...

6. CJD IN FARMERS

The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

BUTCHERS/ABATTOIR WORKERS/OCCUPATION INVOLVING DIRECT CONTACT WITH ANIMAL OR CARCASES 5

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

http://www.bseinquiry.gov.uk/files/yb/1993/07/19001001.pdf

Rocky Mountain oysters, mountain oysters, prairie oysters, Montana tendergroin or swinging sirloin

POLICY IN CONFIDENCE

1. The article in the Daily Mail of 12 August again raises the question of a CAUSATIVE LINK BETWEEN BSE AND CJD. This follows the death of a second farmer from CJD...

snip...

I am, however, concerned about how DH and MAFF would respont to public concern generated if there are further CJD cases among farmers.

snip...

4. Unwelcome, though it maybe to the Tyrrell Committee, I think they must be asked at their next meeting to give further thought to what they might advise the Department and MAFF if ANOTHER FARMER (or TWO) DEVELOPS CJD. OR, if a butcher or abattoir worker develops the disease.

5. Although the Committee were given plenty of advance warning about the second farmer, they may NOT BE SO FORTUNATE NEXT TIME ROUND. Some Contingency planning on the Committee's response to a further case of CJD in a farmer seems essential. At the same time the Committee should consider if there is SPECIAL RISK TO FARMERS, FOR EXAMPLE THEIR HISTORICAL HABIT OF CHEWING CATTLE NUTS, that might be implicated. .....(oh my GOD...tss)

http://www.bseinquiry.gov.uk/files/yb/1993/08/12002001.pdf

Ministers will note from this that experts are of the view, that there is unlikely to be a direct link between the cases of BSE, and the occurance of CJD in the farmer.

(NOTE CJD increasing over 3 years. ...TSS)

http://www.bseinquiry.gov.uk/files/yb/1993/08/18004001.pdf

'AGE AT ONSET' is therefore likely to be a reflection of particulary aetiological factors, about which, for sporadic CJD at least, much is yet unknown. IT has therefore been suggested that examination of the f/d i/p of other groups with TSE's, and comparison with that of CJD subsets might help to elucidate aetiological mechanisms for sporadic CJD in particular; i.e. ALMOST A REVERSAL OF THE ORIGINAL UNDERTAKING.

http://www.bseinquiry.gov.uk/files/yb/1993/08/26001001.pdf

OCCUPATIONAL EXPOSURE TO BSE AND CJD

2. The Tyrrell Committee met on 7 October and the significance of the two cases of CJD reported in dairy farmers who had BSE-affected animals on their farms was discussed at some length, AS WERE THE IMPLICATIONS OF A THIRD (OR FORTH) similar case.

3. The Committee were unable to identify any possible risk factors over and above those that they had already considered, both in general and with particular of TASTING THE FEED does continue but there was no consensus about the value of advising farmers to discontinue this practice. Feed currently in use does not pose a risk because of the ruminant-ruminant feed ban.

http://www.bseinquiry.gov.uk/files/yb/1993/10/11001001.pdf

MRC

STRAIN CHARACTERISATION OF THE CREUTZFELDT-JAKOB DISEASE AGENT BY TRANSMISSION TO MICE

In view of the CONCERN that exposue to BSE OR SCRAPIE MAY POSE A RISK TO HUMANS, it is proposed investigate the relationship between sporadic creutzfeldt-jakob disease.....

http://www.bseinquiry.gov.uk/files/yb/1993/10/12001001.pdf

3. While Committee may have no leads to pursue on why farmers might be at increased risk, I hope they understand the urgency with which they will need to respond if or when a THIRD FARMER DEVELOPS CJD.

http://www.bseinquiry.gov.uk/files/yb/1993/10/18001001.pdf

INCREASE IN SPORADIC CJD

http://www.bseinquiry.gov.uk/files/yb/1993/11/11001001.pdf

occupational

http://www.bseinquiry.gov.uk/files/yb/1994/02/16001001.pdf

Dealler gets ''dixie chicked' again ;

http://www.bseinquiry.gov.uk/files/yb/1993/11/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/08003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/10006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/14003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/16006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/17003001.pdf

STACKING THE DECK AGAINST SPORADIC CJD AND SOUND SCIENCE

APPOINTMENTS IN CONFIDENCE

MEMBERSHIP TO SEAC

snip...

I have informed Dr Tyrrell that we have now written to Dr Hueston to invite him to serve on the Committee and he was very pleased to hear this. He was also in favour of our idea of having a deputy Chairman who could take any emergency meetings eg IF THERE WERE TO BE ANOTHER CJD CASE IN AGRICULTURE. I suggested that either Dr. WIll or Dr Kimberlin were likely candidates and he thought that this was about right. He felt that on balance he would prefer Dr Will who he thought took a more cautious line and was LESS DOGMATIC. .....

http://www.bseinquiry.gov.uk/files/yb/1993/12/01003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/00005001.pdf

CHANGING SCIENCE TO FIT YOUR INDUSTRY NEEDS COVER-UP IN FULL MODE NOW

PROBLEM

7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x).

IP PS(L) wishes to probe this further we think it best to explain the matter VERBALLY. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stores. (or the facts...TSS)

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

A REVISED VERSION WHERE THE FOLLOWING WAS MADE TO BE REMOVED FROM SCIENTIFIC FINDINGS. ...TSS

''This year's findings show a number of associations but the strongest is for veal.''

A BIG LINE WAS DRAWN THROUGH THAT SENTENCE TO BE REMOVED DUE TO THE FOLLOWING. THIS IS THE NEXT SENTENCE ;

''This is of considerable concern given recent developments. In particular, Ministers will be particularly concerned about the European dimension given the recent troubles with the Germans.''

YOU can see the beginning of the ukbsenvCJD only theory beginning to unfold now. full text of this ukbsenvcjd only conspiracy can be seen here. ...TSS

POLICY RESTRICTED

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

BRITISH DEER FARMERS ASSOCIATION

OCTOBER 1994

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is completely independent outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

snip...

The statistical results regarding the consumption of venison was put into perspective in the body of the report and was NOT MENTIONED AT ALL IN THE PRESS RELEASE. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of VENISON was highlighted only by the media i.e. in the News at one television programme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of Venison, would increase, and quite possibly GIVE DAMAGING CREDENCE, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

see buttered and watered down report here that caters to industry instead of human safety...TSS

http://www.bseinquiry.gov.uk/files/yb/1994/10/00004001.pdf

SEE WHERE THIS ;

''This year's findings show a number of associations but the strongest is for veal.''

WENT TO THIS;

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and increased risk of CJD. When some account was taken of possible confounding, the association between veal eating and risk of CJD emerged as the strongest of these associations statistically. These findings concerning dietary history are particulary difficult to interpret for two reasons.

1. .........BSeee...........TSS

2. .........BSeee...........TSS

(I.E. BSeee = bull sh!t encephalopathy or government cover-up i.e. God save the industry at all cost, including human health. ...TSS)

THUS, the reported veal eating habits of confirmed CJD cases appear virtually identical to suspect cases later judged not to have the disease. This provides good circumstantial evidence to support the hypothesis that the apparent association between veal consumption and CJD is due to recall bias. Analysis of other apparent dietary risk factors for CJD, including venison, has provided similar evidence of recall bias.

snip...

In summary, the analysis of the dietary case-control study demonstrates a strong association between a lifetime history of veal consumption and the risk of developing Creutzfeldt-Jakob disease. HOWEVER this result may well be due to recall bias and analysis of clinical and molecular bilogical features does not provide supportive evidence for the hypothesis that veal eating is a risk factor for CJD. ...

snip...

MORE OF THIS BSeee CAN BE READ IN THE FINAL GOVERNMENT DICTATED CJD REPORT OF 1994

http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

BSE SCIENTIST WAS 'CENSORED'

He says that when he worked at MAFF, ''the way it was structurally set up was not that science would drive the politics, but that the politics will drive the science. And that's wrong.''

http://www.bseinquiry.gov.uk/files/yb/1997/12/11001001.pdf

11/3/96 DGRC alerts DTI Ministers and CSA to discovery of nvCJD on basis of letter from MRC Chief Executive dated 11/3/96

BIRTH OF THE UKBSEnvCJD ONLY THEORY, the birth of the 'BIG LIE' begins. ...tss

http://www.bseinquiry.gov.uk/files/db/do01/tab03.pdf

REPORT OF 16 YEAR OLD GIRL WITH CJD

5. This case may raise fears of a link between BSE and CJD. Current advice is that there is no scientific evidence of a link between BSE in cattle and CJD in humans. Furthermore advice from the Spongiform Encephalopathy Advisory Committee is that they are satisified that all necessary safeguards are in place to minimise further spread of spongiform encephalopathies in animals and to prevent any risk of transmission to humans. ...

http://www.bseinquiry.gov.uk/files/yb/1994/01/14005001.pdf

To ask the Secretary of State for Health, how many people under the age of 20 years in each of the last five years have suffered from Creutzfeldt-Jakob disease; and of these how many had not had any growth treatment previously.

SUGGESTED REPLY

We are not aware of any people under the age of 20 in the UK suffering from Creutzfeldt-Jakob Disease in the last five years.

http://www.bseinquiry.gov.uk/files/yb/1994/01/20001001.pdf

STATEMENT FROM HOSPITAL

http://www.bseinquiry.gov.uk/files/yb/1994/01/20005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25001001.pdf

PREPARING FOR THE STORM 'LINE TO TAKE'

http://www.bseinquiry.gov.uk/files/yb/1994/01/25003001.pdf

BARONESS CUMBERLEGE TRYING TO MANIPULATE THE MEDIA

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

MAD COW MEAL DESTROYED MY DAUGHTERS LIFE

A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating a contaminated burger it was claimed last night.

VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).

http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf

GIVE ME BACK MY LIFE

http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf

HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''

http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf

WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY

http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf

I have interviewed Mrs Rimmer at my constituency surgery

IF there is nothing to hide, why is there so much SECRECY? WHY is the Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are taken, surely the problem could be contained, however, as it stands the lack of investigation and interest of the possibility of B.S.E. and C.J.D. being linked is open for speculation and surely someone has to account for peoples lives! WHY is so much trouble being taken to convice people that B.S.E. and C.J.D. are not linked? Guilty Conscience perhaps ? - or cover up?

HOUSE OF COMMONS

FROM BARRY JONES, M.P.

22 FEBRUARY 1994

http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

(now story changes that biopsy shows she does not have CJD...tss)

http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf

now story changes to ;

Advice

7. The Parliamentary Secretary is invited to note the recent statements made on __________ and the present position which remains that CJD cannot be confirmed, in this case at this stage.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf

3. The Medical Director at ___________________ Hospital advised the Department on 6 June that the results of ___________________ brain biopsy had been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital subsequently issued a statement to the press to this effect and this was publicised widely in the press (doc 1). News coverage which followed suggested that the statement made by ________________ Hospital had been misleading (doc 2). Enquires have been made of the Medical Director at _______________ Hospital who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR. The facts are that two pathology reports on the same piece of brain tissue were recieved. The first report indicated that CJD was unlikely, The second report indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive diagnosis could be made before a post mortem was undertaken.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf

(ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM SPORADIC CJD, the same damn thing. ...TSS)

IN light of Asante/Collinge et al findings 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;

-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To: "[log in to unmask]"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[log in to unmask] (until 9/12/02)

New e-mail: [log in to unmask] (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

WHAT ABOUT U.S.A. ???

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf

20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

NOW BACK TO THOSE FARMERS WITH BSE HERDS THAT DIED FROM SPORADIC CJD

CJD FARMERS WIFE 1989

http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

cover-up of 4th farm worker ???

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf

CONFIRMATION OF CJD IN FOURTH FARMER

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf

4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf

2. snip... Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...

http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

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

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

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:

[log in to unmask]

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

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texsas USA 77518

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

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

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

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

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

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518