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

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

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