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From: TSS ()
Subject: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT]
Date: August 11, 2008 at 7:57 am PST

In Reply to: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT] posted by TSS on October 25, 2007 at 6:50 am:

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


Thursday, July 10, 2008 A New Prionopathy OR more of the same old BSe

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html


3RD FARMER BSE CJD TRANSMISSION STUDIES MRC

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

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

snip...

the committee recognised that this is a CAUSE FOR CONCERN.

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

IN CONFIDENCE

CJD IN TEENAGERS/WORLD IN ACTION PROGRAMME

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

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

IN CONFIDENCE

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

snip...

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

LINE TO TAKE

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

snip...

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

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

IN CONFIDENCE

CJD IN TEENAGERS

DATE: AUGUST 9, 1995

snip...

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

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

snip..

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

snip...end

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

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

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

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

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

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

snip...

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

snip...

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

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

DID you notice how these two lines changed.

went from this ;

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

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

too this ;

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

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

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

T E D EDDY

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

IMPORTANT - CONFIDENTIAL

LINE TO TAKE

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

CJD 17 YEAR OLD CONFIRMED

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

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

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

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

BSE: MEAT WORKER WITH POSSIBLE CJD

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

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

AMAZING what money can buy $$$

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

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

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

FROM THE DIRECTOR GENERAL

STRICTLY PRIVATE AND CONFIDENTIAL

24 AUGUST 1995

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

Dear salutation

UKASTA POLICY ON BSE

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

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

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

Yours sincerely,

J.W. REED

JWR/cg

copied to SMT members

STRICTLY PRIVATE AND CONFIDENTIAL

UKASTA INTERNAL POSITION STATEMENT BOVINE SPONGIFORM ENCEPHALOPATHY

POLICY AIMS

POLICY AIMS

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

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

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

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

STRATEGY ADOPTED/SUCCESS ACHIEVED

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

3. Successful examples of this strategy include:

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

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

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

continued.....

95/8.24/2.2

2

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

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

THE FUTURE

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

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

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

JWR/cg/23.8.95

95/8.24/2.3

SEE full text ;

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

4TH CASE SPORADIC CJD IN A FARMER WITH BSE HERD

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

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

snip...

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

snip...

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

Saturday, December 08, 2007

Transfusion Transmission of Human Prion Diseases

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

Thursday, July 24, 2008

Prion diseases are efficiently transmitted by blood transfusion in sheep

Submitted April 18, 2008 Accepted June 28, 2008

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

RESTRICTED FOURTH CASE OF CJD IN FARMER

snip...

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

snip...

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

snip...

full text ;

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

CJD IN FARMERS

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

4TH ANNUAL CJD REPORT

snip...

7. The Final conclusion of the report is that:

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

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

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

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

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

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

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

snip...

Notes on calculations re cases of CJD in farm workers

Numerators

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

snip...

full text ;

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

RESTRICTED-POLICY CJD IN CATTLE FARMERS

From: D Matthews SVO Date: 10 October 1995

snip...

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

snip...

Conclusions

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

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

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

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

LINE TO TAKE ;

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

RESTRICTED - POLICY

CJD IN ADOLESCENTS

snip...

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

95/10.25/6.1

T E D EDDY

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

To: Dr. J Ironside From: Dr. R Will 1 September 1995

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

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

RESTRICTED - POLICY

BSE

snip...

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

snip...

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

SEAC STATEMENT CJD FARMERS

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

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

26 October 1995

snip...

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

snip...

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

snip...

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

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

T J RENDER

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

IN CONFIDENCE CJD IN YOUNG PEOPLE

* in the USA, a 16 year old in 1978

* in France, a 19 year old in 1982

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

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

* Creutzfeldt's first patient in 1920 was aged 23

snip...

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

4th farmer dies ADVICE

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

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

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

FOURTH CASE OF CJD IN FARMER ''CONFIRMED''

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

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

There are now compelling reasons for such an inquiry as there are now three ''CLUSTERS'' of CJD concurrently:

1. Three teenagers, the most ever in one country

2. Four - Six members of the farming community.

3. One confirmed and two suspected cases in a small geographical area --

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/11/05001001.pdf

YOUNG CASES 13/11/95

TABLE

http://www.bseinquiry.gov.uk/files/yb/1995/11/13011001.pdf

CJD AND OCCUPATION

STUPID IS, AS STUPID DOES (Forest Gump...tss)

http://www.bseinquiry.gov.uk/files/yb/1995/12/08022001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/12/08031001.pdf

PRIVATE AND CONFIDENTIAL TO: DR. FROM DR. R. WILL

DATE 14TH DECEMBER 1995

snip...

So far there are 3 confirmed cases under the age of 30, one probably case aged 38 and 5 other suspect cases under the age of 42. Today an additional 35 year old suspect case was referred from London although the duration of illness in this case is somewhat prolonged.

snip..

Today, at 5pm we heard about a further case of suspect CJD in a 50 year old gentleman who had worked up until 4 years ago in an abbatoir. .....

snip...

http://www.bseinquiry.gov.uk/files/yb/1995/12/14013001.pdf

T E D EDDY 1995 trying to paint a better picture rather than the one-in-a-million CJD

http://www.bseinquiry.gov.uk/files/yb/1995/12/15016001.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

CONFIDENTIAL SUSPECT CASE OF CJD IN AN ABATTOIR WORKER

http://www.bseinquiry.gov.uk/files/yb/1995/12/22007001.pdf

THE BIRTH OF A FALSE MYTH, I.E. the UKBSEnvCJD only theory

4/1/96

NATIONAL CREUTZFELDT-JAKOB DISEASE SURVEILLANCE UNIT PATHOLOGY REPORT

snip...

COMMENT

Examination of the fixed brain confirms the diagnosis of Creutzfeldt-Jakob disease in this patient with spongiform change most evident in the basal ganglia and occipital cortex. The presence of kuru-type and occasional multicentric PrP plaques is somewhat unusual and raises the possibiity of a PrP genetic mutation; multicentric PrP plaques are extemely uncommon in sporadic CJD.

http://www.bseinquiry.gov.uk/files/yb/1996/01/04010001.pdf

no reason to remove beef from school lunch menus

http://www.bseinquiry.gov.uk/files/yb/1996/02/16007001.pdf

Narang and Will urine test does work

http://www.bseinquiry.gov.uk/files/yb/1996/02/21005001.pdf

CJD ADOLESCENTS 1996 AND 2008 COMPARE

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

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

Conclusions

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

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

References

snip.....

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

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

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

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

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

* To whom correspondence should be addressed. E-mail: 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

Communicated by: Terry S. Singeltary Sr.

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

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

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

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

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

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

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

JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

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

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

THE PATHOLOGICAL PROTEIN

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

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

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

http://www.thepathologicalprotein.com/

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

Terry S. Singeltary, Sr Bacliff, Tex

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

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

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

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

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

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

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

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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

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

see full text ;

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

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

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

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

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

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

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

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle

03-025IFA 03-025IFA-2 Terry S. Singeltary

Page 1 of 17

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Thursday, September 08, 2005 6:17 PM

To: fsis.regulationscomments@fsis.usda.gov

Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle

Greetings FSIS,

I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle Broken bones and such may be the first signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;

SUB CLINICAL PRION INFECTION

MRC-43-00

Issued: Monday, 28 August 2000

NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION: IMPORTANT RESEARCH

FINDINGS RELEVANT TO CJD AND BSE

A team of researchers led by Professor John Collinge at the Medical

Research Council Prion Unit1 report today in the Proceedings of the

National Academy of Sciences, on new evidence for the existence of a

"sub-clinical" form of BSE in mice which was unknown until now....

full text 17 pages ;

https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument

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

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

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

USDA CERTIFIED DEAD STOCK DOWNER COW SCHOOL LUNCH PROGRAM, the most HIGH RISK animal for BSE mad cow disease, and even more risky here in the USA, where the last two cases of mad cow disease was of the _atypical_ BSE, in Texas and Alabama. THE atypical BSE is more virulent to humans than the U.K. BSE strain. ...TSS

http://downercattle.blogspot.com/

HISTORY OF DEAD STOCK DOWNER COW PROGRAM

http://downercattle.blogspot.com/2008/04/gao-report-on-humane-methods-of.html

http://downercattle.blogspot.com/2008/03/usda-certified-dead-stock-downer-cow.html

http://downercattle.blogspot.com/2008/03/usda-still-pandering-to-industry-still_27.html

http://downercattle.blogspot.com/2008/03/usda-still-pandering-to-industry-still.html

http://downercattle.blogspot.com/2008/03/recalled-beef-from-chino-slaughterhouse.html

http://downercattle.blogspot.com/2008/03/mad-cow-disease-typical-vs-atypical.html

http://downercattle.blogspot.com/2008/03/downer-cow-blues-senators-want.html

http://downercattle.blogspot.com/2008/03/mr-will-hueston-dvm-on-school-lunch.html

http://downercattle.blogspot.com/2008/03/california-downer-cow-meat-worker-i-was.html

http://downercattle.blogspot.com/2008/03/usda-questions-and-answers.html

http://downercattle.blogspot.com/2008/03/usda-to-hallmark-we-want-our-plaque.html

http://downercattle.blogspot.com/2008/03/house-committee-subpoenas.html

http://downercattle.blogspot.com/2008/03/california-lists-possible-recipients-of.html

http://downercattle.blogspot.com/2008/03/to-hard-working-employees-of-usda-and.html

http://downercattle.blogspot.com/2008/02/beef-recall-nationwide-school-lunch.html

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

NOW, how risky dead stock downer cattle?

let's look at an old (in the field) case study. now, this could never have happened, the USA does not have mad cow disease today, or back then ;-(TSS)...not!

Transmissible Mink Encephalopathy TME (MAD MINK DISEASE)

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

Transmissible Mink Encephalopathy TME (MAD MINK DISEASE)

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

40% DIED OVER 8-10 WEEKS TIME !

O.K., just because your children have all been exposed, and evidently continue to be exposed, could potentially die a horrific death from CJD in the years and or decades to come, could expose who knows how many more people via friendly fire i.e. iCJD in the years and or decades to come, should this be cause for alarm ?

NOW, ask yourself WHY THE HONORABLE PEOPLE OF KOREA DO NOT WANT USDA CERTIFIED BEEF ?

Tuesday, June 17, 2008

Beef Imports to Korea: An Open Letter to President Bush Korean middle school student Chae-song Kim asks that the trade agreement be reconsidered

http://usdavskorea.blogspot.com/2008/06/beef-imports-to-korea-open-letter-to.html

http://usdavskorea.blogspot.com/

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

please see full text with additional comments and links @ ;

http://prionunitusaupdate2008.blogspot.com/

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

Audit Report

Animal and Plant Health Inspection Service

Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II

and

Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.

USDA/OIG-A/50601-10-KC Page 27

observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.

snip...

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

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

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

Progress Report from the National Prion Disease Pathology Surveillance Center

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

April 3, 2008

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

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

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

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

Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program

PLEASE SEE FULL TEXT ;

Monday, June 16, 2008 Mad Cows and Computer Models: The U.S. Response to BSE

http://bse-atypical.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

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

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

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

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

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

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

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

In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.

snip...

see full report here ;

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/yearly_report.pps

Thursday, April 24, 2008

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

http://foiamadsheepmadrivervalley.blogspot.com/2008/04/re-foia-of-declaration-of-extraordinary.html

Chronic Wasting Disease

8. Human susceptibility to CWD

Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle [2]. Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades [51]. Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,

8

*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al. [68] showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. [96], although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains [79]. To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days [41]. In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk [84]. However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indi rect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission [49]. Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans [72], and therefore may not indicate that human prion conversion would occur by CWD.

snip...

11. Disease control challenges posed by CWD

Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control [91]. Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease [50]. An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection [55]. Modelling studies have provided further

10

support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions [75]. Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.

12. Conclusion

CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.

Acknowledgements

I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.

snip...see full text 19 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v08092.pdf

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

Wednesday, June 18, 2008 CHRONIC WASTING DISEASE FOUND IN 24 MORE DEER IN ALBERTA

http://chronic-wasting-disease.blogspot.com/2008/06/chronic-wasting-disease-found-in-24.html

PLEASE NOTE THE PARTIAL AND VOLUNTARY MAD COW FEED BAN OF AUGUST 4, 1997 nothing more than ink on paper ... TSS

Wednesday, April 23, 2008

FDA Strengthens Safeguards for Consumers of Beef Issues Regulation on Animal Feeds with Added Safeguards Against BSE

http://madcowfeed.blogspot.com/

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html

stupid is, as stupid does. ... forest gump. ...tss

to be continued

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


Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


TSS


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