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From: TSS ()
Subject: MAD COW TSE UPDATE USA NOVEMBER 2008
Date: November 21, 2008 at 1:50 pm PST

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

BSE, BOVINE - CANADA (04): (BRITISH COLUMBIA)
*********************************************
A ProMED-mail post

ProMED-mail is a program of the
International Society for Infectious Diseases

Date: Tue 18 Nov 2008
Source: eFluxMedia [edited]


The Canadian Food Inspection Agency [CFIA] confirmed on Monday [17
Nov 2008] a new case of mad cow disease (bovine spongiform
encephalopathy, BSE) in a 7-year-old dairy cow born well after Ottawa
banned feed practices thought to spread the disease. It is the
country's 15th case. The animal was discovered on a farm in the
Pacific province of British Columbia.

The agency said no part of the animal's carcass entered the human
food or animal feed supply. It also added that regulators are also
tracking down other animals in the cow's herd when it was born.

Canada has been deemed a "controlled risk" country for mad cow
disease by the World Organization for Animal Health (OIE) because of
its surveillance and control measures. The CFIA said the new case
should not affect that classification.

Bovine spongiform encephalopathy or mad-cow disease emerged first in
British cattle. It is deadly and has an unusually long incubation
period of around 4 years. It also appears rarely in humans under a
different form, called new variant Creutzfeldt-Jakob disease (vCJD or nvCJD).

The disease has already killed almost 200 people in Britain. It is
unclear if the disease is transmitted to humans through consumption
of infected meat, but this is the most likely path. The cattle in
Britain get it from being fed the remains of other infected cows. The
misfolded protein called a prion, which carries the disease, is much
more resistant to heat than common viruses or bacteria, and it is
generally believed that normal cooking processes do not destroy it.

Even though its human form is the most common human prion disease, it
is still rare and only occurs in about one out of every one million
people (except if you live in Britain). In more than 85 percent of
cases, the duration of the disease between onset of symptoms and
death is less than 1 year (median: 4 months). It is incurable and fatal.

[Byline: Anna Boyd]

--
Communicated by:
ProMED-mail Rapporteur Brent Barrett

[Although feeding of mammalian tissue back to cattle is believed to
be the method inducing BSE, there is evidence that there are several
forms of the disease. One of those forms is a sporadic form, which
does not seem to be related to mammalian tissue in the feed. Banning
the mammalian tissue in feed is frequently referred to as the "feed ban."

This may be one of the sporadic forms. - Mod.TG

The Pacific province of British Columbia can be located on the
HealthMap/ProMED-mail interactive map of Canada at
. - CopyEd.MJ]

[see also:
BSE, bovine - Canada (03): (AB) 20080819.2580
BSE, bovine - Canada (02): (BC) 20080623.1941
BSE update 2008 (02) 20080529.1738
BSE update 2008 (01) 20080229.0831
BSE, bovine - Canada (AB) 20080226.0786
2007
----
BSE - Japan: new case, milk replacers 20071223.4122
BSE, bovine - Canada (AB) (03) 20071218.4076
BSE, bovine - Canada (BC) 20070502.1430
BSE, bovine - Canada (AB) (02) 20070308.0813
BSE, bovine - New Zealand: new import rules 20070302.0734
BSE, bovine - Canada (AB) 20070208.0499]
...................................tg/mj/dk

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or archived material.
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----- Original Message -----
From: "TERRY SINGELTARY"
To:
Cc:
Sent: Wednesday, November 19, 2008 9:08 AM
Subject: Re: PRO/AH/EDR> BSE, bovine - Canada (04): (BC)

> This may be one of the sporadic forms. - Mod.TG

please note, 'one of the sporadic forms', has _not_ been proven. so to state this in a release, would only be a _hypothetical_. ...please note the following

kind regards, terry

International Journal of Medical Sciences ISSN 1449-1907 www.medsci.org 2008 5(6):347-353 © Ivyspring International Publisher. All rights reserved

Review

Prion propagation in vitro: are we there yet?

Chongsuk Ryou .. and Charles E. Mays Sanders Brown Center on Aging and Department of Microbiology, Immunology & Molecular Genetics, University of Kentucky College of Medicine, Lexington, KY, U.S.A. .. Correspondence to: Dr. Chongsuk Ryou, 800 Rose St. HSRB-326, Lexington, KY 40536. Phone: (859) 257 4016; Fax: (859) 257 8382; E-mail: cryou2@email.uky.edu Received: 2008.11.03; Accepted: 2008.11.10; Published: 2008.11.11

Prion diseases are caused by proteinaceous pathogens termed prions. Although the details of the mechanism of prion propagation are not fully understood, conformational conversion of cellular prion protein (PrPC) to misfolded, disease-associated scrapie prion protein (PrPSc) is considered the essential biochemical event for prion replication. Currently, studying prion replication in vitro is difficult due to the lack of a system which fully recapitulates the in vivo phenomenon. Over the last 15 years, a number of in vitro systems supporting PrPC conversion, PrPSc amplification, or amyloid fibril formation have been established. In this review, we describe the evolving methodology of in vitro prion propagation assays and discuss their ability in reflecting prion propagation in vivo.

snip...

Conclusion Several different in vitro systems have been devised and tested for successful conversion of PrPC or amplification of PrPSc. Using these methods, many previously unknown but fundamental aspects of prion propagation have been studied. However, we are still far away from the complete understading of the mechanistic details of the process despite the efforts reviewed in this article. On the basis of the protein-only hypothesis, prion propagation is believed to faciliated by a biochemical event known as a conformational conversion of PrPC to PrPSc. The ultimate goal of the in vitro systems is to re-create the condition that faithfully recapitulates prion propagation in vivo. In an ideal condition, a test tube containing both PrP isoforms only should be sufficient to reconstitute the replication process. However, the current form of in vitro reconsititution is not the bona fide system respresenting the in vivo phenomenon. One of the major obstacles is involved in unintended inclusion of cellular factors other than PrP isoforms. Furthermore, our limited knowledge on cofactor molecules makes it more difficult to conceive insight into what has occurred in prion propation in vitro. Despite the limitation in the current form of in vitro conversion assays, simplicity of the systems over cell-based and animal systems has been advantageous. Utilization of these tools will slowly unwind the complicated molecular characteristics of prions such as the species barrier and strain properties. They will also be useful in validating the necessary environment for conversion and estimating the transmissibility of disease. By manipulating the systems, the application can be extended to a sensitive diagnosis of prions and a high-throughput screening of potent anti-prion reagents.

http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=2583336&blobtype=pdf

Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro*

Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either "prion diseases" because of the association of a misfolded cellular prion protein in pathogenesis or "transmissible spongiform encephalopathies" (TSEs) because of the spongelike nature of the damaged brain tissue (1).

The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by "spontaneous" misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or "prion."

Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). BSE caused by spontaneous misfolding of the prion protein has not been proven. CREDIT: KATHARINE SUTLIFF/SCIENCE

snip...

Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent--unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD--which is considered by some researchers to begin by spontaneous misfolding of the prion protein--usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady. ...

snip...full text ;

http://www.sciencemag.org/cgi/content/full/sci;305/5692/1918

Release No. 0106.04

Contact: Office of Communications (202) 720-4623

Transcript of Remarks From Technical Briefing on BSE and Related Issues With Agriculture Secretary Ann M. Veneman and USDA Chief Veterinary Officer Dr. Ron DeHaven Washington D.C. - March 15, 2004

snip...

OPERATOR : "Yes. Our next one is coming from Elizabeth Weiss. Please state your company."

ELIZABETH WEISS: "This is Elizabeth Weiss with USA Today."

"I actually had two questions. First off, when you say you're looking for 1 in 10,000 cases, is USDA doing any work to find out the possibility of whether or not BSE exists in a spontaneous form in the way that it does in humans and elk populations?

"And secondly, how will any of this fit into some of the consternation that's been raised in California and with the Midwest packer that wanted to test all of its cattle?

"Thanks."

DR. DEHAVEN: "All right. I think we've got three different questions in there, and I'll try to touch on each one of them.

"First of all, let me correct just a technical issue, and that is you mentioned 1 in 10,000. And actually our surveillance system currently is designed, the one that we have in place now is designed to detect 1 positive in 1 million cattle, and I gave some numbers between 200,000 and 268,000 that would allow us to detect 1 in 10 million as opposed to 1 in 10,000.

"So we would, if we were able to collect in the ballpark of those numbers of samples then we with increasing numbers of samples have an increasingly statistically valid sample from which to determine, one, whether or not the disease exists and, if so, at what prevalence level.

"So our real emphasis is to test as many of those animals as we can, ensure that we get an appropriate geographical distribution, but not setting a specific number as far as a target. Again, consistent with the recommendation from the International Review Team, their recommendation was to test all of them.

"So that's consistent with where we're going is to test as many as we possibly can.

"As far as spontaneous cases, that is a very difficult issue. There is no evidence to prove that spontaneous BSE occurs in cattle; but here again it's an issue of proving a negative. We do know that CJD, the human version of the disease, does occur spontaneously in humans at the rate of about 1 in 1 million. We don't have enough data to definitively say that spontaneous cases of BSE in cattle occur or do not occur.

"Again, it's a very difficult situation to prove a negative.

"So a lot of research is ongoing. Certainly if we do come up with any positive samples in the course of this surveillance we will be looking at that question in evaluating those samples but no scientifically hard evidence to confirm or refute whether or not spontaneous cases of BSE occur.

snip...

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2004/03/0106.html

UK TESTING FINISHED NEXT WEEK

Matthews confirmed that the brain tissue samples from the US animal had arrived at Weybridge. Test results were likely to be ready by the end of next week, he said.

The suspect animal has already undergone a series of tests. A rapid screening test on Nov. 15 returned inconclusive results. Sophisticated immunohistochemistry (IHC) tests cleared the animal of any infection, but a third round of testing using a Western blot procedure showed a "weak positive".

Weybridge will do an IHC test plus three kinds of Western Blot tests on the samples. They will use "methods of slightly different analytical sensitivity that give us the greatest number of opportunities to interpret what we see," he said.

US beef industry leaders say scientists should not speculate about the unusual case.

"There's no evidence that it's atypical ... and there's absolutely no evidence that it's spontaneous," said Gary Weber, head of regulatory affairs at the National Cattlemen's Beef Association.

Matthews noted scientists are still grappling with what is typical and atypical BSE.

"Far too few people have looked at BSE in depth using all of the tests to be able to define 'this is normal and that one isn't'," he said.

Weber noted Japan used the term to describe two very young infected cattle because BSE is usually found in older animals. Italy labeled a case "atypical" because the misshaped prions were found in unexpected parts of the animal's brain. ...snip...end

http://www.agobservatory.org/headlines.cfm?refID=73207

Atypical BSE strain -- In July 2007, the UK Spongiform Encephalopathy Advisory Committee (SEAC) suggested that atypical BSE may be a distinct strain of prion disease. Unlike typical BSE, cases of atypical BSE, according to SEAC, may have risen spontaneously (although transmission through feed or the environment cannot be ruled out). Recently reported French surveillance data support this theory that unlike typical BSE, atypical BSE appears to represent sporadic disease

http://cdc.gov/ncidod/dvrd/bse/

http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf

Spontaneous occurrence 14. Spontaneous cases of classical CJD in humans are found at a rate of about 1/million around the world (Will, 1993), without appreciable racial or geographical variation except in a few specific cases, notably Jews of Libyan origin that have a mutation in the open reading frame of the PRNP gene (Chapman and Korczyn, 1991). Thus it is theoretically possible that spontaneous cases of BSE could occur as a consequence of a germ line mutation, in which case relatives would also have a certain or increased incidence of the disease, or a somatic mutation, which would be unlikely to be detectable unless the appropriate tissue were identified, or after some transformation in the PrP protein in the animal concerned. BSE was unknown prior to its detection in Britain in the mid 1980s, and Index cases found around the world since then can all be explained in terms of export from the UK directly or indirectly of cattle or of feed components. No BSE affected animals have been reported in many developed countries with large cattle populations, including Australia, New Zealand, Norway and Sweden, which have mixed cattle populations; and the only infected animal detected in the US was of Canadian origin. The disease seems to have a highly homogeneous aetiology (e.g. Bruce, 2003). 15. Data from the USA, where the dairy population in particular is highly related to that in GB provide an upper limit to the spontaneous rate. A programme of testing is in place of a target population of adult cattle exhibiting some clinical sign that might be consistent with BSE (animals reported as having CNS or clinical signs of BSE or were non-ambulatory). In the intensive programme from June 2004 over 375000 animals were tested in the following 12 months. No positive results have yet been obtained in these or previous tests (USDA BSE Testing). This implies a putative upper limit of under 10 per million in this target group. Assuming, as analysis has shown, the relative risk in this group is about 30 times higher than in the population as a whole (European Commission, 2002c), then the incidence in the population as a whole is under 3 per 10 million. This figure could possibly be biased downwards if affected animals are diagnosed and disposed of without being tested. Taking account of testing done and the lack of clinical cases seen in many other countries also, it seems highly unlikely that the spontaneous rate can be as much as 3 per 10 million head. Nor can spontaneous occurrence explain incidences of ca. 30 cases of BARBs per year in 2002/4 in the UK adult cattle population of ca. 4 million. [NOTE ADDED 30 JUNE: The recent confirmation of a previously inconclusive case in the USA affects these calculations. If the animal did not have access to infected feed, the calculations have to be revised: they suggest a sporadic incidence in the population of 1/(375000 x 30) or almost 1 per 10 million, with the upper limit under 5 per 10 million.] 16. Calculation of a maximum rate of possible transformation from scrapie to BSE is less feasible. Nevertheless, BSE appears not to have arisen in the UK until around the early 1980s, despite the presence of scrapie in sheep here for at least 200 years. Although a change in the scrapie prion may have been the cause of the initial cases of BSE, the difference between their properties in mice and the uniformity of the BSE brain lesions suggest it is unlikely that more than one such mutation was the source of BSE. It is most unlikely that the same mutation could be occurring often enough to contribute significantly to BARBs cases. Furthermore BARBs cases do not match the geographical distribution of the sheep population. The evidence from the absence of BSE in many countries and the surveillance schemes abroad indicates that most BARBs cases cannot have arisen spontaneously, although the possibility cannot be excluded that a very

http://www.defra.gov.uk/animalh/bse/pdf/hillreport.pdf

242 Atypical and classical BSE are different strains based upon Western blot profiles 243 (Hill, 2004; Normile, 2004; Baron et al., 2006), and this study indicates that disease Page 11 of 23 Journal of Animal Science Downloaded from jas.fass.org by on November 12, 2008. 12 progresses via different routes for these strains. The disparate 244 routes of pathogenesis in 245 atypical BSE can occur by 1 of 2 means. One possibility is that the source of infectivity 246 in atypical BSE is exposure to contaminated feedstuffs, as is the case for classical BSE, 247 but progression occurs in a disparate manner that bypasses the influence of the indel 248 polymorphisms. The other possibility is that atypical BSE is occurring spontaneously in 249 the host. Support for atypical BSE occurring spontaneously are the parallels to sporadic 250 TSE in humans, specifically, occurrence in older hosts and a comparable low incidence 251 rate (Baron and Biacabe, 2006). Furthermore, atypical BSE occurs as isolated, sporadic 252 cases in contrast to the clustering of cases observed for feed borne classical BSE 253 (Donnelly et al., 1997). Interestingly, the only native born cases of BSE in the United 254 States identified to date have been classified as atypical BSE.

255 No experiment can conclusively confirm a spontaneous nature for atypical BSE.

snip...end

http://jas.fass.org/cgi/reprint/jas.2007-0208v1.pdf

2:00 Sporadic CJD and Atypical BSE: Two Children of One Protein Maurizio Pocchiari, Ph.D., Director of Research, Virology, Istituto Superiore Di Sanita The identification of forms of TSE diseases in cattle caused by prion strains different from BSE has raised new concerns on the possibility that these novel agents might induce disease in humans with a phenotype resembling sporadic CJD. The analysis of the distribution of the different molecular subtypes of sporadic CJD might give some answers.

http://www.healthtech.com/Conferences_Overview.aspx?c=518&id=59662&c=518

Sunday, November 16, 2008 Resistance of Bovine Spongiform Encephalopathy (BSE) Prions to Inactivation

http://bse-atypical.blogspot.com/2008/11/resistance-of-bovine-spongiform.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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

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

see full text ;

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

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

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

TSS

Tuesday, November 11, 2008 Transmission of atypical bovine prions to mice transgenic for human prion protein DOI: 10.3201/eid1412.080941

http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html

Tuesday, November 11, 2008 SaBTO Summary of 1st Public Meeting - variant CJD and blood Tuesday 21st October 2008, 2pm-4pm

http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html

Friday, August 29, 2008

CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW

http://madcowtesting.blogspot.com/2008/08/creekstone-vs-usda-court-of-appeals.html

TSS

----- Original Message ----- From: "ProMED-mail" To: Sent: Wednesday, November 19, 2008 7:41 AM Subject: PRO/AH/EDR> BSE, bovine - Canada (04): (BC)

BSE, BOVINE - CANADA (04): (BRITISH COLUMBIA) ********************************************* A ProMED-mail post ProMED-mail is a program of the International Society for Infectious Diseases

Date: Tue 18 Nov 2008 Source: eFluxMedia [edited]

The Canadian Food Inspection Agency [CFIA] confirmed on Monday [17 Nov 2008] a new case of mad cow disease (bovine spongiform encephalopathy, BSE) in a 7-year-old dairy cow born well after Ottawa banned feed practices thought to spread the disease. It is the country's 15th case. The animal was discovered on a farm in the Pacific province of British Columbia.

The agency said no part of the animal's carcass entered the human food or animal feed supply. It also added that regulators are also tracking down other animals in the cow's herd when it was born.

Canada has been deemed a "controlled risk" country for mad cow disease by the World Organization for Animal Health (OIE) because of its surveillance and control measures. The CFIA said the new case should not affect that classification.

Bovine spongiform encephalopathy or mad-cow disease emerged first in British cattle. It is deadly and has an unusually long incubation period of around 4 years. It also appears rarely in humans under a different form, called new variant Creutzfeldt-Jakob disease (vCJD or nvCJD).

The disease has already killed almost 200 people in Britain. It is unclear if the disease is transmitted to humans through consumption of infected meat, but this is the most likely path. The cattle in Britain get it from being fed the remains of other infected cows. The misfolded protein called a prion, which carries the disease, is much more resistant to heat than common viruses or bacteria, and it is generally believed that normal cooking processes do not destroy it.

Even though its human form is the most common human prion disease, it is still rare and only occurs in about one out of every one million people (except if you live in Britain). In more than 85 percent of cases, the duration of the disease between onset of symptoms and death is less than 1 year (median: 4 months). It is incurable and fatal.

[Byline: Anna Boyd]

-- Communicated by: ProMED-mail Rapporteur Brent Barrett

[Although feeding of mammalian tissue back to cattle is believed to be the method inducing BSE, there is evidence that there are several forms of the disease. One of those forms is a sporadic form, which does not seem to be related to mammalian tissue in the feed. Banning the mammalian tissue in feed is frequently referred to as the "feed ban."

This may be one of the sporadic forms. - Mod.TG

The Pacific province of British Columbia can be located on the HealthMap/ProMED-mail interactive map of Canada at . - CopyEd.MJ]

[see also: BSE, bovine - Canada (03): (AB) 20080819.2580 BSE, bovine - Canada (02): (BC) 20080623.1941 BSE update 2008 (02) 20080529.1738 BSE update 2008 (01) 20080229.0831 BSE, bovine - Canada (AB) 20080226.0786 2007 ---- BSE - Japan: new case, milk replacers 20071223.4122 BSE, bovine - Canada (AB) (03) 20071218.4076 BSE, bovine - Canada (BC) 20070502.1430 BSE, bovine - Canada (AB) (02) 20070308.0813 BSE, bovine - New Zealand: new import rules 20070302.0734 BSE, bovine - Canada (AB) 20070208.0499] ...................................tg/mj/dk

*##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Become a ProMED-mail Premium Subscriber at ************************************************************ Visit ProMED-mail's web site at . Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name and affiliation, it may not be posted. Send commands to subscribe/unsubscribe, get archives, help, etc. to: majordomo@promedmail.org. For assistance from a human being send mail to: owner-promed@promedmail.org. ############################################################

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


Archive Number 20081103.3458 Published Date 03-NOV-2008

Subject PRO/AH/EDR> Prion disease update 2008 (12)

http://www.promedmail.org/pls/otn/f?p=2400:1001:2109365826921604::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,74615

http://www.promedmail.org/pls/otn/f?p=2400:1000:

Star Valley Moose Tests Positive For CWD

http://www.promedmail.org/pls/otn/f?p=2400:1202:2109365826921604::NO::F2400_P1202_CHECK_DISPLAY,F2400_P1202_PUB_MAIL_ID:X,74423

Thursday, August 28, 2008

cwd, feeding, and baiting piles

http://chronic-wasting-disease.blogspot.com/2008/08/cwd-feeding-and-baiting-piles.html

Thursday, August 28, 2008

CWD TISSUE INFECTIVITY brain, lymph node, blood, urine, feces, antler velvet and muscle

http://chronic-wasting-disease.blogspot.com/2008/08/cwd-tissue-infectivity-brain-lymph-node.html

Tuesday, August 26, 2008 CWD Stakeholder Advisory Group Wednesday, August 22, 2007 11:31 AM

http://chronic-wasting-disease.blogspot.com/2008/08/cwd-stakeholder-advisory-group.html

Research Project: STRAIN TYPING OF CHRONIC WASTING DISEASE (CWD) AND SCRAPIE BY INTRACEREBRAL INOCULATION INTO TRANSGENIC AND INBRED MOUSE LINES

Location: Animal Diseases Research

2008 Annual Report

1a.Objectives (from AD-416) To identify and differentiate typical and atypical case samples of CWD and Scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice.

1b.Approach (from AD-416) Tissue samples from deer, elk, sheep and goats with Transmissible Spongiform Encephalopathy (TSE) will be administered to mice by intracerebral injection. Multiple tissue types will be included, such as samples of brain, lymph node, blood, urine, feces, antler velvet and muscle. Mouse models used as recipient hosts will include both preexisting and recently created transgenic and inbred mouse lines. Recipient mouse phenotype will be evaluated by measuring clinical response, population disease rate, incubation time, and pathologic profile within the central nervous system (CNS). Pathologic profile of CNS lesion foci is assessed by evaluating anatomic localization, spongiform change, astrocytic gliosis, and deposition of protease resistant prion protein. Documents SCA with U. of WA.

3.Progress Report Strain Typing Of Chronic Wasting Disease (CWD) And Scrapie By Intracerebral Inoculation Into Transgenic And Inbred Mouse Lines. Project number 5348-32000-026-07S. This report serves to document research conducted under a specific cooperative agreement between ARS and the University of Washington. Additional details of research can be found in the report for the parent project 5348-32000-026-00D Transmissible Spongiform Encephalopathies: the role of genetics, strain variation, and environmental contamination in disease control. The purpose of this SCA is to identify and differentiate typical and atypical case samples of CWD and scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice. Mice have been inoculated with tissue from sheep with Nor98-like and classical scrapie and are monitored for incubation time, clinical disease and postmortem evaluation of disease associated changes in the brain and accumulation of abnormal prions in tissues. There are regular interactions between the ADODR, ADRU scientists and University of Washington collaborators through personnel visits, conference calls and electronic exchange of data.

http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=411895&fy=2008

Staining, straining and restraining prions Adriano Aguzzi A recent paper reported the in vitro generation of new prion strains, supporting the idea that ‘strain-ness’ is encoded in the protein structure itself. This lays the groundwork for a reinvigorated study of prion structure–pathology relationships.

The characteristic promiscuity of a given prion strain can be retained on crossspecies jumps, which is why consumption of BSE-infected sheep meat may be dangerous to humans. Also, little is known about the threat posed to humans by chronic wasting disease, a prion strain affecting a high proportion of North American deer and elk. A further intriguing example of idiosyncratic strain behavior relates to the transmission of vCJD and sporadic CJD (sCJD) prions from humans to wild-type mice: the former transmit readily, whereas the latter do not. Conversely, transgenic mice devoid of mouse PrP, but expressing human PrP, show no barrier to human sCJD prions, but do have a pronounced barrier to human vCJD prions, even when the human donors (of the PRNP 129MM allelotype) express prion proteins of identical primary structure. None of the above phenomena can be readily explained, highlighting our limited understanding of prion transmission barriers.

volume 11 | number 11 | NOVEMBER 2008 nature neuroscience

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

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

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

BMJ

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

2 January 2000

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

JOURNAL OF NEUROLOGY MARCH 26, 2003

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

Email Terry S. Singeltary: flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which ar sporadic are all spontaneous, without route/source. We have many TSEs i 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 exposeothers. 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

doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk

Tracking spongiform encephalopathies in North America Xavier Bosch Available online 29 July 2003. Volume 3, Issue 8, August 2003, Page 463

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem."

............................

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html

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

A prion disease of cervids: Chronic wasting disease

Sigurdson CJ.

The recent discovery of chronic wasting disease in cervids (CWD) beyond the borders of Colorado and Wyoming, as far east as New York and including two Canadian Provinces, has led to the emergence of CWD as a prion disease of domestic and international importance. The apparent ease of horizontal transmission, potentially via environmental contamination or by prion-containing saliva, creates enormous challenges for disease management. Ongoing studies of CWD interspecies transmission by exposure of domestic and non-domestic species directly or using transgenic mice have shed light on species barriers. Transgenic mice expressing cervid PrP have also proven useful for assessing the genetic influences of Prnp polymorphisms on CWD susceptibility. Accumulating evidence of CWD pathogenesis indicates that the misfolded prion protein, PrPSc, seems to be widely disseminated in many nonneural organs, and CWD infectivity has been recently detected in blood. This review highlights recent research findings in this disease of free-ranging wildlife.

snip...

3. CWD prion spread and target organs

Collectively, CWD pathogenesis studies have revealed extensive deposition of PrPSc in the central nervous system (CNS) and extraneural tissues (Fig. 1). The only other natural prion diseases that even approach this degree of systemic involvement are variant Creutzfeldt-Jakob disease (vCJD) in humans, sheep scrapie, and transmissible mink encephalopathy [22, 23, 30, 61, 62]. In mule deer, PrPSc is detectable in the retropharyngeal lymph node within only 6 weeks following an oral exposure [76]. In a further study of the kinetics of prion

1http://www.aphis.usda.gov/vs/nahps/cwd/cwd-distribution.html

5

infection in mule deer, Fox et al. showed that PrPSc is widely distributed in lymphoid tissues by 3 months post-oral exposure when it is first detected in brain [17]. By 9 months, PrPSc was detected in the myenteric and submucosal plexi throughout the gastrointestinal tract and in the vagus nerve, and by 16 months, PrPSc deposits were detectable throughout the brain and spinal cord. The Prnp genotype seemed to impact the infection kinetics in that mule deer that were SF heterozygous at codon 225 showed a delay in PrPSc spread; PrPSc was not

detectable in the brain until 16 months post-inoculation which was 13 months later than the 225SS deer. Perhaps the 225F allele confers a dominant negative effect on the kinetics of this CWD strain, as has been described in sheep, where the 171R allele has been shown to have a dominant negative effect on prion susceptibility [20, 33]. CWD pathogenesis seems to vary between deer and elk: PrPSc levels have been found to be lower in lymphoid tissues of elk compared to deer [66]. In a report of 226 CWD-infected elk, 28 had no PrPSc in lymphoid tissues despite having PrPSc in brain [81]. In addition to lymphoid tissues, PrPSc or infectivity has been detected in other non-CNS tissues, including pancreas [17, 77], adrenal gland [17, 77], and skeletal muscle [2]. Recently PrPSc was described in cardiac muscle from 7 of 16 (44%) white-tailed deer and from 12 of 17 (71%) elk [35]. This is the first report of PrPSc in cardiac muscle in any TSE. The cellular and molecular mechanisms of systemic prion spread are under investigation in many laboratories. A recent report showed that blood from CWD-infected deer contained infectivity and could transmit prion disease via a blood transfusion [50]. This finding recapitulates indirect findings of blood infectivity in vCJD affected humans [61] and experimental transfusion studies of scrapie sick sheep [32], and indicates that prion transport

throughout the body may include the blood as a potential vehicle.

snip...

6. CWD strains among deer and elk

Prion strains, such as those seen in sheep scrapie, show distinct incubation periods in differentially susceptible inbred mice and lesions target discrete brain regions [11, 18, 19]. CWD in deer and elk has been considered a single disease entity, and western blot glycoform patterns of PrPSc are similar among deer and elk [67]. However, some new data indicate otherwise, suggesting that conformational variants, or strains, may exist. In a study by Raymond et al., Syrian golden hamsters were infected with mule deer or elk CWD, but with an incomplete attack rate; only 2 of 7 and 0 of 7 hamsters developed terminal disease, respectively. Indeed, second and third passage of the mule deer derived strain resulted in a short incubation period of only 85-89 days, whereas the elk-derived strain led to an incubation period of 408-544 days. Surprisingly, when mule deer CWD was first passaged in hamster PrP expressing transgenic mice and then into hamsters, a slowly replicating strain

with distinct clinical disease and PrPSc deposition patterns in brain ensued. Therefore two different strains could be passaged from a single mule deer CWD isolate, a rapid and a slowly replicating strain with differing disease phenotypes [70]. Alternatively, these strains could have been generated upon interspecies transmission [6].

We have also observed two strains arising from a single CWD-infected mule deer upon

passage in transgenic mice overexpressing murine PrP. Here, mice developed different

PrPSc aggregate morphologies in brain, either dense, congophilic plaques or fine, diffuse aggregates which could be selectively passaged [78]. LaFauci et al. have reported that elk PrP expressing transgenic mice developed phenotypically divergent diseases when inoculated with either mule deer or elk CWD, which was also suggestive of different strains [44]. In each of these studies, it is not clear whether mule deer and elk possess heterogeneous PrP aggregates (strain mixture), or whether the strains may have developed in the new host. However, Safar et al. have reported differing conformational characteristics for PrPSc from CWD-infected white-tailed deer and elk directly, using a conformation dependent immunoassay (CDI) [71], which supports the existence of CWD strains. The possible existence of CWD strains is perhaps not entirely surprising, considering that there are genetic Prnp differences among deer and elk that could influence PrPSc conformation [34, 36, 58].

7

7. Interspecies CWD transmission

Wild predators and scavengers are presumably feeding on CWD-infected carcasses.

Skeletal muscle has been shown to harbor CWD prion infectivity [2], underscoring that other species will almost certainly be exposed to CWD through feeding. However, CWD has not been successfully transmitted by oral inoculation to species outside of the cervid family, suggestive of a strong species barrier for heterologous PrP conversion. Ferrets (family Mustelidae) can be infected with deer CWD after intracerebral (ic) but not oral exposure [5, 80]. Raccoons resisted even ic infection for up to 2 years thus far [24]. Mountain lion (Puma concolor) susceptibility to experimental feeding of CWD prions is currently under investigation (M. Miller and L. Wolfe, personal communication).

Could wild rodents colonizing CWD- or scrapie-infected pastures serve as an environmental reservoir of prion infectivity? Interestingly, bank voles (Clethrionomys glareolus), are readily infected with CWD and sheep scrapie by intracerebral inoculation ([64]; U. Agrimi, unpublished data) and are considered as a potential reservoir for sheep scrapie [64]. Many vole species occur in North America [65, 83] and further research may determine whether voles enhance CWD or scrapie spread through environmental contamination.

Given that environmental contamination with CWD prions likely occurs [55], domestic

ruminants may be exposed to CWD through common grazing areas. However, sheep and

cattle appear to be poorly susceptible to mule deer CWD: ic inoculation with mule deer CWD succeeded to infect only 2 of 8 sheep [28]; likewise cattle have not been infected after cograzing with CWD-infected mule deer, or after a direct oral exposure (over 6 years) (M. Miller, personal communication). Even direct ic inoculation led to CWD infection in only 5 of 13 cattle (38%) after 2-5 years [26]. In contrast, cattle are highly susceptible to white-tailed deer CWD with 12 of 14 animals developing neurologic disease and PrPSc by only 22 months post-ic inoculation (+/-0.5 months) [29]. Further studies are planned to determine whether

cattle are susceptible to white-tailed deer prions after an oral exposure (J. Richt, personal communication). The differential susceptibility of cattle to CWD from mule deer versus whitetailed deer suggests that CWD strains exist, and that CWD may differentially cross species barriers depending on the strain. Nevertheless, to date, natural CWD infections have been detected only in cervids.

Is the converse true, are cervids susceptible to sheep scrapie? Only one study has been performed on cervid susceptibility to sheep scrapie by the ic route, and showed that 3 of 6 elk developed neurologic signs, spongiform encephalopathy and PrPSc in brain [25]. Further experiments to address this question may be interesting since sheep scrapie is considered a possible source for CWD in North America [89, 91].

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 indirect 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=/a

Research Project: Strain Typing of Chronic Wasting Disease (Cwd) and Scrapie by Intracerebral Inoculation into Transgenic and Inbred Mouse Lines

Location: Animal Diseases Research

Project Number: 5348-32000-026-07

Project Type: Specific C/A

Start Date: Sep 28, 2007

End Date: Sep 27, 2012

Objective:

To identify and differentiate typical and atypical case samples of CWD and Scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice.

Approach:

Tissue samples from deer, elk, sheep and goats with Transmissible Spongiform Encephalopathy (TSE) will be administered to mice by intracerebral injection. Multiple tissue types will be included, such as samples of brain, lymph node, blood, urine, feces, antler velvet and muscle. Mouse models used as recipient hosts will include both preexisting and recently created transgenic and inbred mouse lines. Recipient mouse phenotype will be evaluated by measuring clinical response, population disease rate, incubation time, and pathologic profile within the central nervous system (CNS). Pathologic profile of CNS lesion foci is assessed by evaluating anatomic localization, spongiform change, astrocytic gliosis, and deposition of protease resistant prion protein. BSL-1; 9-4-06. Documents SCA with U. of WA.

http://www.ars.usda.gov/research/projects/projects.htm?accn_no=411895

Research Project: Strain Typing of Chronic Wasting Disease (Cwd) and Scrapie by Intracerebral Inoculation into Transgenic and Inbred Mouse Lines

Location: Animal Diseases Research

2007 Annual Report

1a.Objectives (from AD-416)

To identify and differentiate typical and atypical case samples of CWD and Scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice.

1b.Approach (from AD-416)

Tissue samples from deer, elk, sheep and goats with Transmissible Spongiform Encephalopathy (TSE) will be administered to mice by intracerebral injection. Multiple tissue types will be included, such as samples of brain, lymph node, blood, urine, feces, antler velvet and muscle. Mouse models used as recipient hosts will include both preexisting and recently created transgenic and inbred mouse lines. Recipient mouse phenotype will be evaluated by measuring clinical response, population disease rate, incubation time, and pathologic profile within the central nervous system (CNS). Pathologic profile of CNS lesion foci is assessed by evaluating anatomic localization, spongiform change, astrocytic gliosis, and deposition of protease resistant prion protein. BSL-1; 9-4-06. Documents SCA with U. of WA.

3.Progress Report

This report serves to document research conducted under a specific cooperative agreement between ARS and the University of Washington. Additional details of research can be found in the report for the parent project 5348-32000-026-00D Transmissible Spongiform Encephalopathies: the role of genetics, strain variation, and environmental contamination in disease control. The purpose of this new SCA is to identify and differentiate typical and atypical case samples of CWD and scrapie by characterizing the biologic phenotype and pathologic profile of these agents when administered to susceptible lines of transgenic and inbred mice. There will be weekly interactions between the ADODR, ADRU scientists and University of Washington collaborators through personnel visits, conference calls and emails.

http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=411895&showpars=t

From: TSS (216-119-163-189.ipset45.wt.net)

Subject: CWD aka MAD DEER/ELK TO HUMANS ???

Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"

To:

Cc: "Race, Richard (NIH)" ; ; "Belay,

Ermias"

Sent: Monday, September 30, 2002 9:22 AM

Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam,

In the Archives of Neurology you quoted (the abstract of which was

attached to your email), we did not say CWD in humans will present like

variant CJD.

That assumption would be wrong. I encourage you to read the whole

article and call me if you have questions or need more clarification

(phone: 404-639-3091). Also, we do not claim that "no-one has ever been

infected with prion disease from eating venison." Our conclusion stating

that we found no strong evidence of CWD transmission to humans in the

article you quoted or in any other forum is limited to the patients we

investigated.

Ermias Belay, M.D.

Centers for Disease Control and Prevention

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

From:

Sent: Sunday, September 29, 2002 10:15 AM

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG

HUNTERS

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

also,

A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most

serious because of rapid horizontal spread and higher prevalence than BSE in

UK, up to 15% in some populations. Also may be a risk to humans - evidence

that it is not dangerous to humans is thin.

http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summar

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*

*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from:http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

--------------------------------------------------------------------------------

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.

snip...full text ;

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

Volume 12, Number 10–October 2006

Research

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Samantha MaWhinney,* W. John Pape,† Jeri E. Forster,* C. Alan Anderson,‡§ Patrick Bosque,‡¶ and Michael W. Miller#

*University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; †Colorado Department of Public Health and Environment, Denver, Colorado, USA; ‡University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Suggested citation for this article

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40–1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73–1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

snip... full text ;

http://0-www.cdc.gov.mill1.sjlibrary.org/ncidod/EID/vol12no10/06-0019.htm

full text ;

http://chronic-wasting-disease.blogspot.com/2006_12_01_archive.html

CWD

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

CJD QUESTIONNAIRE

http://cjdquestionnaire.blogspot.com/

Monitoring the occurrence of emerging forms of CJD

http://cjdusa.blogspot.com/

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

snip...

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

snip...

ITEM 3 - CURRENT ISSUES

9.

SEAC was informed about the following issues:

. Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.

http://www.seac.gov.uk/papers/101-1.pdf

3 10:10 Current issues

vCJD cluster in Spain Testing of a goat

snip...

In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.

* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.

http://www.seac.gov.uk/agenda/agen151008.htm

The CJD Foundation Newsletter

http://www.cjdfoundation.org/content/newsletters/september2008.pdf

snip...

SEPTEMBER 2008 VOLUME 1, ISSUE 3

The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.

Note 1: Not all new cases and deaths reported are confirmed by autopsy.

Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..

2008

MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS

January 35 24 203 6,424 February 24 9 212 6,848 March 19 19 164 7,492 April 36 14 231 8,427 May 26 20 191 8,839 June 19 17 144 9,646 July 28 14 171 7,791 August 27 16 150 5,323

TOTALS = 214 133 1,466 60,790

P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? help@cjdfoundation.org www.cjdfoundation.org

CJDF Questionnaire Update

With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at help@cjdfoundation.org or 1-800-659-1991. We greatly appreciate your help with this important project!

CJD QUESTIONNAIRE HISTORY

http://cjdquestionnaire.blogspot.com/

Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website

http://www.neuroprion.com/en/patients-events-07-6th-CJD-Conf.html

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

snip...

Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...

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

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

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

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

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

Creutzfeldt Jakob Disease

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

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

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

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

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

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

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

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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

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

see full text ;

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

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

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

Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html

Review on the epidemiology and dynamics of BSE epidemics

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

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;

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

***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

Sunday, March 16, 2008

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

Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html

SEAC 99th meeting on Friday 14th December 2007

snip...

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 - PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: "With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?" 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

snip...

http://www.seac.gov.uk/minutes/99.pdf

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.

10 people killed by new CJD-like disease

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

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip... see full text ;

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

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html

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

http://seac992007.blogspot.com/

snip...

http://www.seac.gov.uk/minutes/99.pdf

TSS

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

http://seac992007.blogspot.com/2008/10/seac-101st-meeting-on-wednesday-15th.html

Alzheimer's and CJD

http://betaamyloidcjd.blogspot.com/

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

http://betaamyloidcjd.blogspot.com/

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


Friday, November 21, 2008
Plasma & Serum Proteins Receive Continued FDA Approval

http://madcowfeed.blogspot.com/2008/11/plasma-serum-proteins-receive-continued.html

http://madcowfeed.blogspot.com/


Friday, November 21, 2008
Amarillo-area (suspect sporadic CJD) case linked to mad cow disease Rumor in Texas

http://cjdtexas.blogspot.com/2008/11/amarillo-area-suspect-sporadic-cjd-case.html


Sunday, November 16, 2008

Resistance of Bovine Spongiform Encephalopathy (BSE) Prions to Inactivation

http://bse-atypical.blogspot.com/2008/11/resistance-of-bovine-spongiform.html


Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein
DOI: 10.3201/eid1412.080941


http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html


Tuesday, November 11, 2008
SaBTO Summary of 1st Public Meeting - variant CJD and blood Tuesday 21st October 2008, 2pm-4pm

http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html


ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE


Oct 23, 2008 at 9:00 AM


http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html

http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html


Friday, November 07, 2008
Human and animal exposure risk related to Transmissible Spongiform Encephalopathies (TSEs) from milk and milk products derived from small ruminants

Opinions


http://scrapie-usa.blogspot.com/2008/11/human-and-animal-exposure-risk-related.html


'France did not do enough to prevent spread of mad cow,' say scientists in stinging attack on own government

By Ian Sparks Last updated at 3:04 PM on 06th November 2008

Comments (0) Add to My Stories French scientists have issued a damning report finally admitting the true scale of France's mad cow disease epidemic.

The full confession that BSE was rife in France in the early 1990s comes a decade after their illegal ban on British beef drove many UK farmers into bankruptcy.

The study ordered by a senior Paris judge and published today by the country's top scientists found the French did not do enough to prevent the spread of BSE when it was first discovered in 1986.

French scientists have launched a stinging attack on their own government over the handling of mad cow disease in the 1990s

It also said that the lives of nine French people who died between 1996 and 2006 from its human form Creuzfelt-Jaokb disease could have been saved it better precautions had been taken.

BSE was officially first detected in Britain in 1986 and steps were taken to combat it.

Former French President Jaques Chirac said the only thing Britain gave to European farming was mad cow

But it only became obligatory for farmers to declare cases in 1990 and the EU lifted a trade embargo on British beef in 1999.

But France continued with an illegal ban on British meat for another seven years, while claiming their own herds were free of the disease.

It has still never paid tens of millions of pounds in fines to the EU for persisting with its unlawful embargo.

Former President Jacques Chirac said in 2003 that the only thing Britain had given European farming was mad cow disease.

But the new report into how nine French people died of vCJD has now uncovered the true extent of the disease across the Channel.

Scientist Jean-Louis Thillier said: 'There was enough available scientific information for the government to have taken measures to protect the public from BSE in 1991.

'But in fact it was not until ten years later that adequate steps were taken.'

Mr Thillier asked why there was an embargo placed on British beef when nothing was done in France.

He said the French government had forgotten its role in guaranteeing the safety of food and that nine people had died as a result.

http://www.dailymail.co.uk/news/worldnews/article-1083643/France-did-prevent-spread-mad-cow-say-scientists-stinging-attack-government.html


BSE FRANCE


https://lists.aegee.org/cgi-bin/wa?S2=BSE-L&X=4031BF620A9567E0D2&Y=flounder9@verizon.net&q=france&s=bse+france&f=&a=&b=


ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Oct 23, 2008 at 9:00 AM


http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html


http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html


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






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