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

Posted Jul 10 2008 10:55am
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://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



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
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