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

Original Article

A novel human disease with abnormal prion protein sensitive to protease

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

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

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

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

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

Abstract

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

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

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

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

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

10.1002/ana.21420 About DOI

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

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

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

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

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


sporadic CJD, the big lie


Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

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

Conclusions

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

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

References

snip.....

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

sporadic CJD, the big lie

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

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

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

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

* To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk.

Accepted 15 April 2007

Abstract

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

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

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

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

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

Sent: Monday May 28, 2007

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

Terry S. Singeltary Sr.

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

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

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

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

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

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

snip...

Sporadic creutzfeldt-jakob disease in two adolescents

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

see full text sporadic CJD the big lie;

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

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

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

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

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

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

Accepted 15 April 2007

Abstract

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

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

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

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

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

Sent: Monday May 28, 2007

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

Terry S. Singeltary Sr.

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

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

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

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

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

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

CONFIRMED CJD IN FARMER WITH BSE COW

line to take, sporadic CJD

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

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

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

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

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

SECOND CASE CJD IN DAIRY FARMER

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

CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE

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

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

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

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

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

IF PRESSED:

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

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

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

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

CONFIDENTIAL

CONFIRMED CASE OF CJD IN DAIRY FARMER

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

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

snip...

HUMAN CASE DETAILS CONFIDENTIAL

snip...

6. CJD IN FARMERS

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

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

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

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

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

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

POLICY IN CONFIDENCE

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

snip...

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

snip...

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

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

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

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

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

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

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

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

OCCUPATIONAL EXPOSURE TO BSE AND CJD

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

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

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

MRC

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

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

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

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

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

INCREASE IN SPORADIC CJD

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

occupational

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

Dealler gets ''dixie chicked' again ;

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

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

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

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

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

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

STACKING THE DECK AGAINST SPORADIC CJD AND SOUND SCIENCE

APPOINTMENTS IN CONFIDENCE

MEMBERSHIP TO SEAC

snip...

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

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

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

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

PROBLEM

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

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

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

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

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

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

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

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

POLICY RESTRICTED

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

BRITISH DEER FARMERS ASSOCIATION

OCTOBER 1994

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

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

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

snip...

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

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

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

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

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

SEE WHERE THIS ;

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

WENT TO THIS;

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

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

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

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

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

snip...

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

snip...

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

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

BSE SCIENTIST WAS 'CENSORED'

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

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

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

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

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

REPORT OF 16 YEAR OLD GIRL WITH CJD

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

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

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

SUGGESTED REPLY

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

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

STATEMENT FROM HOSPITAL

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

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

PREPARING FOR THE STORM 'LINE TO TAKE'

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

BARONESS CUMBERLEGE TRYING TO MANIPULATE THE MEDIA

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

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

MAD COW MEAL DESTROYED MY DAUGHTERS LIFE

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

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

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

GIVE ME BACK MY LIFE

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

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

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

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

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

I have interviewed Mrs Rimmer at my constituency surgery

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

HOUSE OF COMMONS

FROM BARRY JONES, M.P.

22 FEBRUARY 1994

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

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

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

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

now story changes to ;

Advice

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

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

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

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

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

IN light of Asante/Collinge et al findings that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

-------- Original Message -------- Subject: re-BSE prions propagate as

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

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

Dear Terry,

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

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

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

you for your interest in the paper.

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

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

phenotype to type 2 PrPSc, the commonest sporadic CJD.

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

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

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

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

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

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

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

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

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

Emmanuel Asante

<> ____________________________________

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

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

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

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

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

____________________________________

snip...

full text ;

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

WHAT ABOUT U.S.A. ???

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

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

in France, a 19 year old in 1982;

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

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

Creutzfeldt's first patient in 1923 was aged 23.

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

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

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

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

CJD FARMERS WIFE 1989

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

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

cover-up of 4th farm worker ???

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

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

CONFIRMATION OF CJD IN FOURTH FARMER

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

now story changes from;

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

to;

This is not unexpected...

was another farmer expected?

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

4th farmer, and 1st teenager

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

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

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

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

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

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

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

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

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

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

snip...

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

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

snip...

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

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

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

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

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

6:30 Close of Day One

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

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

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

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

BRITISH MEDICAL JOURNAL

BMJ

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

BMJ

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

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

Email Terry S. Singeltary:

[log in to unmask]

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535

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

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

THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

Saturday, March 22, 2008

10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures

http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html

re-Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease

http://betaamyloidcjd.blogspot.com/2008/04/re-association-between-deposition-of.html

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

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

see full text ;

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

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

Friday, June 20, 2008

USDA TO KOREA AND THE WORLD, EAT THAT AND LIKE IT

http://usdavskorea.blogspot.com/2008/06/usda-to-korea-and-world-eat-that-and.html

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

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

IT was the media and the womans doctor here in the USA that first reported she had human form of mad cow disease. and that's what PD NOTEBOOK and many other media outlets published, until the USDA (?) which first announced that it was not nvCJD. ...



Portsmouth woman did not die of mad cow-related condition, ___USDA___ says


By Nancy Young The Virginian-Pilot © May 7, 2008

Preliminary test results indicate that a 22-year-old Portsmouth woman who died in April did not have an illness that has been associated with eating contaminated beef, a U.S. Department of Agriculture official said this week.

"The epidemiologic characteristics of the illness and preliminary results of the neuropathologic testing of brain tissue obtained at autopsy indicate that the patient did not die of" a variant of Creutzfeldt-Jakob Disease (vCJD), which has been associated with mad cow disease, said Richard Raymond, USDA undersecretary for food safety. He was speaking Sunday to a group of Korean and American reporters in Washington.

"The U.S. Centers for Disease Control and Prevention has just provided us with that information, and I felt it was important to share with you today," he told the group. The comment appears on a statement on the safety of the U.S. beef supply that is available on the USDA's Food Safety and Inspection Service Web site.

Aretha Vinson died April 9 at Bon Secours Maryview Medical Center in Portsmouth. She had a degenerative brain condition that can be caused by a wide variety of things, and concerns were raised that she might have had vCJD. The state health department, CDC and the University of Virginia were involved in testing to determine the cause of death and whether it indicated a public health concern.

"There's no threat to the general public," said Michelle Peregoy, a Virginia Department of Health spokeswoman. She would not say more, citing patient confidentiality issues.

Nancy Young, (757) 446-2947, nancy.young@pilotonline.com


http://hamptonroads.com/2008/05/portsmouth-woman-did-not-die-mad-cowrelated-condition-usda-says



Virginia State University student Aretha Vinson has contracted a rare brain disorder called Creutzfeldt-Jakob Disease, a variant of Mad Cow Disease. Virginia state health officials are investigating how Vinson contracted the disease. Doctors suspect Vinson contracted the disease after having gastric bypass surgery, possibly from tainted medical instruments. Vinson's family says her health worsened after having gastric bypass surgery three months prior to the diagnosis.

UPDATE: Vinson passed away at 5:30pm on Wednesday, April 9, 2008.


http://www.mahalo.com/stub/aretha-vinson



The program, which now faces charges for playing up the possibility that the woman died of vCJD, said, “The CDC last Thursday announced the cause of death of Aretha Vinson, who died of symptoms similar to vCJD.” It quoted the CDC as saying although the suspected case received international media attention, the National Prion Disease Pathology Surveillance Center determined that the cause of death was not due to vCJD, a finding, it pointed out, that was similar to an announcement by the Department of Agriculture.


http://english.chosun.com/w21data/html/news/200806/200806170006.html




22 year old sporadic CJD ???

we will never know ;

In a telephone interview with the Chosun Ilbo, CDC spokesman Dave Daigle on Monday said the centers posted the announcement after performing their own checkup once the NPDPSC finished its investigation. He added that because the CDC only provide information on diseases, they have no plans to make a separate press release on the issue including the result of the investigation. ...


http://english.chosun.com/w21data/html/news/200806/200806170006.html




now, the cdc et al usually hide behind patient confidentiality to hide cjd cases. but in this 22 year old, confidentiality was not the case, she was well known around the world, and the cdc et al chose to hide her final diagnosis. why, because it was another young victim in the USA with sporadic CJD ???




TSS





PLEASE SEE ;



Joint Fact-Finding Mission to South Korea Final Report Seoul, South Korea July 21–24, 2008



http://material.ahrchk.net/docs/AHRC-SPR-006-2008-SouthKorea.pdf





http://minbyun.org/?module=file&act=procFileDownload&file_srl=27364&sid=0b0c8d8b3089eef17f3ae37d0d8538d2





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



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





U.K. BSE nvCJD ONLY theory invoked again. it's like still believing the world is flat for pete's sake i.e. the one strain, one country, one age group, one species, one route, only theory. it's pure BSe, and the stench is horrendous. it's the smell of death, for profit only.

THE UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.

snip...

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

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

ONE only has to read how the USDA et al have legally blocked, blundered, botched, mismanaged, bungled, floundered, and flat out manipulated, the testing in the infamous June 2004 enhanced cover-up program for mad cow surveillance and testing. I mean, I am not really to hip on THE INDUSTRY, testing for mad cow disease, and what that program might consist of, but anything is better than nothing at all. ...

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


> since when did usda start releasing cdc data $$$

> i told you it would be anything but nvCJD $$$

> THIS case will be anything but nvCJD. how could it be? the victim never left

> the USA, and the USA does not have BSE $$$ ...TSS


USDA, CDC, NIH, ET AL INVOKE THE UKBSEnvCJD ONLY RULE $$$


Virginia Woman Did not Die of vCJD

Updated Jun.17,2008 08:34 KST

The MBC news program "PD Diary" reported that Aretha Vinson died of variant Creutzfeldt-Jakob Disease (vCJD) in early April when in an interview, Vinson's mother actually said, "The results had come in from the MRI and it appeared that our daughter could possibly have CJD," not vCJD.

The Center explained that as of June 2008, a total of three people were reported to have died from vCJD in the U.S. All three cases ¡°were epidemiologically linked to likely exposures to cattle products contaminated with bovine spongiform encephalopathy (BSE, commonly known as ¡®mad cow disease¡¯) while residing in the United Kingdom (two cases) or Saudi Arabia (one case).¡±

In a telephone interview with the Chosun Ilbo, CDC spokesman Dave Daigle on Monday said the centers posted the announcement after performing their own checkup once the NPDPSC finished its investigation. He added that because the CDC only provide information on diseases, they have no plans to make a separate press release on the issue including the result of the investigation.

The NPDPSC was set up by the CDC and the American Association of Neuropathologists to strengthen checks on various prion diseases. Doctors in the U.S. are recommended to take advantage of the diagnostic service provided by the NPDPSC to check the condition of patients who are suspected of contracting CJD or vCJD. "PD Diary" touched off a public uproar over the import of U.S. beef after it reported on April 29 that the woman, identified as Arlene Vinson was likely to have died of vCJD.

(englishnews@chosun.com )

http://english.chosun.com/w21data/html/news/200806/200806170006.html


He added that because the CDC only provide information on diseases, they have no plans

to make a separate press release on the issue including the result of the investigation.

and that is the way they plan to keep it, all spontaneous, sporadic, no route, no source $$$

DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

snip...end

CJD TEXAS

http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html


This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle

*** and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


The Italian cases (11 and 15 years of age) originally named bovine amyloidotic spongiform encephalopathy (BASE) were characterized by an unglycosylated protein band with a lower molecular mass (thus named L cases) and the predominance of the monoglycosylated band. In addition, immunohistochemical detection of PrPres in these cases found greater deposits in the cerebral cortex and thalamus versus the brain stem. The French cases found a higher molecular mass associated with the unglycosylated protein band and were called H cases (see figure 1). *** The different "strains" are now called atypical BSE. ...

full text, skroll down to page 6 ;

http://www.usaha.org/committees/reports/2006/report-fe-2006.pdf


MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE (the last two cases of mad cow disease in the USA were in Alabama, and Texas, both of which were atypical BSE).

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


Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

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

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45


WHY were they planning to destroy all CJD tissue samples donated ???

Washington Times - Washington,DC,USA NIH may destroy human brain collection

By Steve Mitchell Medical Correspondent

Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.

Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.

Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.

"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE). ...snip...end...tss

http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm


NIH says it will preserve CJD brains By STEVE MITCHELL

WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

snip...

May 10, 2005

The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.

I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) ...snip...end...tss

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


NIH says it will preserve CJD brains

Published: May 31, 2005 at 5:26 PM

By STEVE MITCHELL WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.

That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.

"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.

Cornyn had inquired about the status of the collection in April.

Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.

Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.

Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.

"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.

CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.

Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.

Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.

"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.

"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.

"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.

"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.

Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.

Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.

--

Steve Mitchell is UPI's Medical Correspondent. E-mail: sciencemail@upi.com

© 2005 United Press International. All Rights Reserved. This material may not be reproduced, redistributed, or manipulated in any form.

http://www.upi.com/NewsTrack/Science/2005/05/31/nih_says_it_will_preserve_cjd_brains/6771/print_view/

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

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


CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006

The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.

The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

http://www.upi.com/

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...

http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm

PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125


AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf


NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

http://www.pnas.org/cgi/content/abstract/0502296102v1


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


Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008

1: Vet Res. 2008 Apr 3;39(4):41

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html


Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/


Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html


http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html


Journal of American Medical Association

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

http://jama.ama-assn.org/


2 January 2000 British Medical Journal

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey b y intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


http://www.cjdsupport.net/ Newsletter issue 17 april 2008

the importance of gene types

We have known for many years that a common variation in a gene, known as the prion protein gene, is very important in determining the risk of developing prion diseases and how long it takes for the disease to develop when someone becomes infected. There are three genetic types in the UK population known as MM, VV and MV. So far, vCJD has only affected people with MM genetic type. Around 40% of healthy people in the UK are MM, about 50% are MV and around 10% are VV. It is likely that BSE prions will infect people of the VV and MV types also, but they may have much longer incubation periods (the time taken from being infected with prions until the brain disease becomes apparent) and may also develop a pattern of disease which may be different to vCJD. We suspect this again as result of research in laboratory mice wher e those that had the VV and MV genes had a different type of disease and different types or ‘strains’ of prions developed.

snip...

This unusual finding reminds us of the importance of keeping alert to the possibility that BSE prions will cause disease in individuals with different genetic types, who may develop a disease that may resemble sporadic CJD, or vCJD, or have a new pattern of disease.

Prion disease and gene types

Dr Simon Mead and Professor John Collinge, NHS National Prion Clinic

Reference: Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman Simon Mead; Susan Joiner; Melanie Desbruslais; Jonathan A. Beck; Michael O’ Donoghue; Peter Lantos; Jonathan D. F. Wadsworth; John Collinge Arch Neurol. 2007;64(12):1780-1784.

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


CJD QUESTIONNAIRE

http://cjdquestionnaire.blogspot.com/


THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/


2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html


Wednesday, June 11, 2008

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

* 67th General Session (GS) Resolution No (Res) XVI and Res XI; 69th GS Res XV, and 71st GS Res XXII, 72nd GS Res XXIV and Res XXI..

http://www.oie.int/eng/info/en_statesb.htm?e1d6


IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

http://www.oie.int/eng/Session2007/RF2006.pdf


bought and paid for by your local cattle dealer $$$

IN my opinion the WOAH/OIE is nothing more than a organized bunch of lobbyist for the members Countries in support of there INDUSTRY, bound together as one, with the only purpose of open trade for there precious commodities and futures. Speaking only of BSE, they failed at every corner, and then just said to hell with it, well just trade all strains of TSE globally.

snip...

NOW, ask yourself why not one single mad cow has been documented in the USA since the Honorable Phyllis Fong of the OIG did the end around Johanns, Dehaven et al ??? found two atypical BSE or BASE cases and they flat shut it down i tell you. IF the OIE gives a favorable rating, IF the OIE gives any other rating but the lowest, poorest possible BSE/TSE rating, the OIE will have sealed there fate once and for all, because most of the world knows the truth about the USA and there mad cows. THE OIE will then be able to stand side by side with the USA, and proudly claim to have sold there soul to the devil, all for a buck, commodities and futures, to hell with human health. A 'CONTROLLED' RATING IS EXACTLY what the OIE will get if that is what they classify the USA as a 'CONTROLLED RATING'. IT will be controlled by Johanns, Dehaven, and GW. IT WILL BE RIGGED in other words. but that is nothing new, it's been rigged for years. ...

snip...SEE FULL TEXT with facts and sources @ ;

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


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


sporadic CJD in two adolescents (sCJD, the big lie)

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


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


see full text sporadic CJD the big lie;

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


http://cjdusa.blogspot.com/2007/11/cjd-surveillance-uk-fifteenth-annual.html


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

=====================TSS 2008=====================


----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Tuesday, May 06, 2008 5:55 PM Subject: [BSE-L] re-Portsmouth woman did not die of mad cow-related condition, USDA says

Portsmouth woman did not die of mad cow-related condition, USDA says Posted to: Health and Medicine News Portsmouth

hi nancy,

since when did usda start releasing cdc data $$$

i told you it would be anything but nvCJD $$$

was it sporadic CJD ???

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Monday, May 05, 2008 3:47 PM Subject: STATEMENT OF DR. RICHARD RAYMOND USDA UNDERSECRETARY FOR FOOD SAFETY Regarding the Safety of the U.S. Food Supply May 4, 2008

Monday, May 5, 2008 STATEMENT OF DR. RICHARD RAYMOND USDA UNDERSECRETARY FOR FOOD SAFETY May 4, 2008

STATEMENT OF DR. RICHARD RAYMOND USDA UNDERSECRETARY FOR FOOD SAFETY Regarding the Safety of the U.S. Food Supply

May 4, 2008

"Good evening. I am Dr. Richard Raymond, Under Secretary for Food Safety at the U.S. Department of Agriculture. I appreciate the opportunity to discuss with you the safety of the U.S. beef supply. I want to be sure that you are aware that I will be discussing food safety issues only, and I am not here this evening to discuss negotiations. "The U.S. Government believes the current agreement well addresses the health and food safety concerns of Korean consumers. It provides for Korea's sovereign right to conduct an audit of our facilities and to work with USDA inspection authorities if any food safety concerns are identified. When the OIE gave the United States "controlled risk" status a year ago, it was after the world's BSE experts reviewed the preventative and food safety measures in the United States. "Since the requirements to export to Korea are consistent with science, U.S. requirements as well as those of the OIE require that if any food safety concern is found, it would be fully investigated and immediately corrected by USDA. "I want to assure all consumers - both domestic and abroad - that the U.S. beef supply is among the safest in the world. ...

*please* see full text with some additional information the good Dr. Raymond

seems to have forgotten about. it's about your children. ...

http://usdameatexport.blogspot.com/2008/05/statement-of-dr-richard-raymond-usda.html


Wednesday, April 30, 2008

Health group urges overhaul of US food safety system Calling the US food safety system antiquated and disjointed, a public health advocacy group today urged a major overhaul to make the system stronger, more coherent, and better attuned to today's major threats.

http://www.cidrap.umn.edu/cidrap/content/fs/food-disease/news/apr3008tfah.html


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

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

Dear Member:

Once again we are writing to thank you for your continued support in enhancing surveillance of prion diseases in the United States and to bring you up to date on the National Prion Disease Pathology Surveillance Center (NPDPSC).

In large part because of your support, the number of cases examined by biopsy, autopsy and 14-3-3 protein determination has increased significantly over the years (see Tables 1 and 2). We are now able to establish a definitive diagnosis of prion disease in an estimated 60-70% of the cases in the United States, a percentage which exceeds that in even some major surveillance centers. In addition, we receive from you cerebrospinal fluid (CSF) for 14-3-3 determination, a surrogate protein which is helpful in the diagnosis of prion disease, probably in most if not all cases of suspected Creutzfeldt-Jakob disease (CJD). We are making constant efforts to reach our goal of at least 80% definitively diagnosed cases.

The major obstacle to our further increasing the autopsy rate remains the inadequate reporting of suspected cases of CJD to the NPDPSC or to the State Health Department, which in turn would notify us. Since you are the one likely to request the 14-3-3 test on these cases, please include in your request the information needed to contact you, which we will do if the test proves positive. If your institution uses a referral laboratory to send us the CSF, please provide your name, phone, and fax numbers to the lab, which will in turn submit it to us along with the sample. If this information is missing in the request accompanying the CSF sample (as it happens in about 30% of the cases), we will be unable to contact the caregiving physician. Having your contact information would also allow us to send results directly to you, thus reducing turnaround times. ...

snip...

Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S.

snip...

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

please see full text with additional comments and links @ ;

http://prionunitusaupdate2008.blogspot.com/


Another Young Suspect Vcjd Case In Usa (3rd In Recent Weeks), TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY

THESE cases that have come about recently in the very young are most disturbing.

a 22 year old last week died, she is suspect nvCJD. never left US. PORTSMOUTH, Va. -- A 22-year-old Portsmouth woman is close to dying, and family says doctors believe the human equivalent of Mad Cow Disease could be the reason.

another young female suspect nvCJD that is 26 years old in Alabama, She is in the final stages of CJD. She is at home in a hospital bed...very skinny...and at times in the past month has had some eating and swallowing issues. Sometimes she rallies and starts eating again. She stopped walking at Christmas. they do not expect her to live much longer than May, since that would be 14 months since her first major symptom of CJD (personal communication).

AND now, a 3rd young female, 23 years old. hmmm, i am pondering about just how long all those downers were in the school lunch program, and IS the incubation period catching up now ??? is this a first of many more to come ???

see full text ;

http://organicconsumers.org/forum/index.php?s=71e4aa3ad5237e46d182cdfcdd167cb0&showtopic=1413&pid=5798&st=0&#entry5798


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

Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings

Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings - Scientific Opinion of the Panel on Biological Hazards

Question number: EFSA-Q-2007-110

Adopted date: 17/04/2008

Summary

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/biohaz_op_ej700_bovine_tongue_summary_en.pdf

Opinion

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/biohaz_op_ej700_bovine_tongue_en.pdf

Summary

Following a request from the European Commission, the Panel on Biological Hazards (BIOHAZ) was asked to deliver a scientific opinion on the human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of the findings included in a scientific article recently published on the consumption of beef tongue and the risk for public health.

This scientific article describes the distribution of lymphoid tissue in bovine tongue and the location of bovine lingual tonsil. In addition, it concludes that the method currently prescribed for harvesting bovine tongues in slaughterhouses is not appropriate for removing all specified risk material (SRM) and proposes an alternative harvesting method.

EFSA was requested (i) to evaluate the design of the study and its scientific validity in relation to the distribution of lymphoid tissue in bovine tongue and (ii) to evaluate the conclusions and recommendations of the study in relation to BSE risk from bovine tonsil following the harvesting method currently prescribed by EU legislation compared to the alternative one proposed in the study.

The BIOHAZ Panel reviewed the scientific article and concluded that the study further confirms and extends observations that the lingual tonsil at the base of the tongue may not be entirely eliminated when harvesting tongues by means of the method currently prescribed.

In reply to the second request, the BIOHAZ Panel assessed different parameters in order to quantify the human exposure risk to BSE from bovine tonsil associated with the consumption of bovine tongue. It was concluded that, overall, the level of infectivity in bovine tonsil is low. This, together with the declining and overall low BSE prevalence and the current policy on SRM removal, suggests a very low, if not negligible, human BSE exposure risk associated with exposure to lymphoid tissue in bovine tongue harvested as currently prescribed by EU legislation. The BIOHAZ Panel further concluded that currently there are not sufficient quantitative data available allowing a comparison of the human BSE exposure risk reduction achieved by the alternative tongue harvesting method proposed by the study in comparison to the harvesting method currently prescribed. However, it is likely that the proposed method would only provide a marginal reduction in the risk from bovine tonsil compared to the one currently prescribed.

Following to this, the BIOHAZ Panel made a series of recommendations on the topics that might be addressed in future studies on the subject.

http://www.efsa.europa.eu/EFSA/efsa_locale-1178620753812_1178704312961.htm

Annex 1

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/annex_1_bovine_tongue_translation.pdf

3.1.2. Experimental BSE cases after oral challenge. • In a sequential kill pathogenesis study of BSE in which calves were experimentally infected by oral exposure to 100g of a pool of BSE affected brainstems (a dose considered far in excess of most natural exposures (Arnold et al., 2007)), palatine tonsil was assayed in conventional mouse strains from all time points (2-40 months post-exposure). No infectivity was detected (Wells et al., 1998; Wells et al., 2005). • Palatine tonsil from this sequential kill pathogenesis study was further assayed by intracerebral inoculation of cattle, which provides a 500 fold (log10 2.7) greater sensitivity of detection of the BSE agent than the RIII mouse assay (Wells et al., 2005). A pooled inoculum was prepared from palatine tonsil of cattle (3, 3, 3 and 1 respectively) at each of 6, 10, 18, and 26 month periods post-exposure (corresponding to 10, 14, 22 and 30 months of age) from the oral challenge study. One ml of a ten per cent tissue homogenate in saline was injected by the intracerebral route into groups of 5 calves. Results indicated traces of infectivity in the palatine tonsil of cattle killed ten months after experimental oral exposure, with transmission occurring in 1 of the 5 challenged calves (Wells et al., 2005). An infection rate of 1 out of 5 suggests that the infectivity is close to the limit of detection of the assay and that the titre of infectivity in tonsil is less than 1 cattle i.c. ID50/g. The study was completed in 2006, without further transmission to any of the 4 remaining calves (Veterinary Laboratory Agency, unpublished data). • Palatine tonsil collected from a further sequential-kill pathogenesis study of BSE in which 100 calves were exposed to 100g of a pool of BSE affected brainstems (Arnold et al 2007) was also bioassayed. In this study, Espinosa et al. (2007) inoculated tonsil tissue intracerebrally into mice expressing bovine PrP (BoPrP-Tg110). The inocula originated from pooled samples from three cattle at each of five time points (20, 24, 27, 30 and 33 months) after the oral exposure of calves. Infectivity was detected at all of the time points tested, with infection rates in the mice of 1/6, 1/6, 1/5, 1/6 and 1/6 respectively. Interestingly, this would seem to indicate a relatively low constant plateau level of infectivity in the tonsil throughout this largely preclinical period. The low infection rate (1 out of 6) is consistent with a level of infectivity lower than 1 i.c.ID50 in Tgbov mice. Buschmann and Groschup (2005) provide data on a Tgbov mice model giving a 1.000-10.000 fold greater sensitivity than the cattle bioassay. While the Tgbov mice (tg110) in the study of Espinosa et al. (2007) were different from the TgbovXV mice used by Buschmann and Groschup (2005), their sensitivity is likely to be similar based on comparable levels of over expression of the bovine PrP gene in each model. From data provided by titrations of BSE infectivity in brain by the i.c. and the oral routes in cattle it has been calculated that one bovine oral ID50 = 105.5 bovine i.c. ID50 (Wells et al., 2007). On this basis, the titre of infectivity in tonsil is less than 10-5.5 bovine oral ID50/g; an estimate, using additional data, that is closely similar to that provided previously (EFSA 2004). Given the greater sensitivity of the Tgbov mouse assays than the cattle i.c. assay, it would seem probable that even in cattle after exposure to a 100g oral dose, the titre in terms of bovine oral ID50/g may be at least one order of magnitude less (i.e. 10-6.5 bovine oral ID50/g), for at least part of the incubation period. As the majority of exposures in the epidemic were probably less than 1g (Arnold et al., 2007), the infectivity in tonsil might well have occurred at an even lower titre and have peaked later in incubation. The most optimistic estimate might be that with doses of the order of less than 1g, tonsil never contains detectable levels at any time in the disease course. A table summarising the key parameters of the infectivity studies mentioned in section 3.1 can be found in Appendix A. In summary, the available data on BSE infectivity in tonsil indicate that: • The frequency with which detectable infectivity occurs in tonsillar tissue of a BSE naturally infected animal is difficult to estimate. Although only two cases have been investigated employing biological assay, infectivity has not been detected in palatine tonsil from naturally occurring clinical cases. • From sequential kill pathogenesis studies in experimentally infected cattle, infectivity associated with tonsil could be detected at 10 months post-exposure and, in the preclinical period, 20-33 months post-exposure (Wells et al., 2005; Espinosa et al., 2007), suggesting that, in this model, infectivity in tonsil probably persists throughout the disease course. • In the experimental exposure to a 100g dose, infectivity titer can be estimated from the data available to be 10-6.5 bovine oral ID50 per gram of tonsilar lymphoid tissue. • It must also be noted that all available data on infectivity relate to palatine tonsil. Occurrence and comparable levels of infectivity in lingual tonsil is an assumption. 3.2. Amount of lymphoid tissue associated with the tongue intended for human consumption As mentioned above, the findings of the study from Casteleyn et al. (2007) are consistent with those of other studies carried out in Great Britain (Wells et al., 2005) and Germany (Rebmann et al., submitted for publication), but provide no quantification of lymphoid tissue remaining in tongues intended for human consumption. Examination of 251 tongues, derived from 15 abattoirs in Great Britain after removal of SRM and intended for human consumption, showed that visible identifiable lingual tonsillar tissue, indicated by fossulae, remained in 76.5% of them (192 out of 251) (Wells et al., 2005). Even in the tongues in which no visible tonsillar tissue remained, histological examination revealed lymphoid tissue in more than 90% of them. Variations in the distribution of the lingual tonsil suggested that even after the most rigorous trimming of the tongue traces of tonsillar tissue may remain. However, the histological examination did not extend rostral to the most caudal of the filiform papillae, which occur caudal to the most caudal vallate papillae. In Germany (Rebmann et al., submitted for publication), specimens of the lingual mucosa were taken from thirty cattle immediately after slaughter. The main parameter to identify the lymphoreticular tissue in this study was the immunohistochemical identification of the follicular dendritic cells (FDC). Lymph follicles were detected in areas up to 30 mm rostral to a given macroscopic landmark (i.e. the most caudal of the vallate papilla). This is an area which would not be removed from the tongue when implementing the measures foreseen by Regulation (EC) No 999/2001. Alternative technical approaches for the removal of the lingual tonsil’s tissue, similar to those by Casteleyn et al. in 2007, are proposed by the authors. These scientific studies did not take into account the rostral part of the tongue which can harbor solitary primary lymph nodules or diffuse accumulations of T and B lymphocytes. Some information on more rostral areas of the tongue was provided in a more recent study (Kato and Sawada, 2008). In that study, examination of the bovine tongue was carried out from the tip of the torus linguae to the root of the tongue. The study confirmed previous results in relation to the distribution of lymphoid tissue in bovine tongue. In addition, 1 out of the 20 specimens collected rostrally to the most rostral vallate papilla contained lingual tonsillar tissue. Based on the above data, neither qualitative nor quantitative estimation of the significance of the lymphoid formations located rostrally to the most caudal vallate papilla is feasible at present. In the DNV risk assessment cited in section 3.1 (EFSA, 2004; SEAC, 2003), a total weight of bovine tonsil of 50g is assumed. However, in the DNV risk assessment, it is stated that the weights of the various tissues were taken from the LFRA ruminant products audit (LFRA, 1997). The total weight of tonsillar tissue in a typical bovine, as given from literature derived offal weights in the LFRA audit, is estimated at 200g. This suggests that the value of 50g in the DNV report is in fact referring to the weight of lingual tonsil. If the largest contribution to the total weight of tonsillar material is the palatine tonsil, it would be reasonable to estimate lingual tonsil as approximately 50g. It is further assumed in the DNV risk assessment that, after removal of all visible tonsillar tissue, the realistic upper limit of tissue that would remain in the tongue would be 10% i.e. 5g. Since the palatine tonsil is a circumscribed structure and easily identified, complete removal, compared to the lingual tonsil, is ensured, so this estimate remains valid as the quantity of tonsillar tissue that might not be removed. In conclusion, based on the data available:

• it is not possible to know exactly the quantity of lymphoid tissue remaining in bovine tongue intended for human consumption when harvested according to the harvesting method currently applied, even if it can be estimated to be 5g;

• it is not possible to estimate how much lymphoid tissue is removed by the alternative harvesting method compared with the one currently applied.

snip...full text ;

http://www.efsa.europa.eu/EFSA/Scientific_Opinion/biohaz_op_ej700_bovine_tongue_en.pdf


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http://sciencebushwhacked.blogspot.com/

TSS