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From: TSS (216-119-128-125.ipset8.wt.net)
Subject: TSEs IN THE USA AND THE INFAMOUS SPORADIC CJD i.e. govCJD
Date: November 19, 2003 at 10:12 am PST

Aguzzi warns of CWD danger

The TSE family of diseases also includes chronic wasting disease (CWD)
in deer, a condition that has spread in the US in recent years (Nature
416, 569; 2002). Speaking at the Days of Molecular Medicine conference
in La Jolla in March, prion expert Adriano Aguzzi issued a strong
warning against underestimating this form of TSE.

"For more than a decade, the US has by-and-large considered mad cows
to be an exquisitely European problem. The perceived need to protect
US citizens from this alien threat has even prompted the deferral of
blood donors from Europe," he said. "Yet the threat-from-within
posed by CWD needs careful consideration, since the evidence that CWD
is less dangerous to humans than BSE is less-than-complete. Aguzzi
went on to point out that CWD is arguably the most mysterious of all
prion diseases.

"Its horizontal spread among the wild population is exceedingly
efficient, and appears to have reached a prevalence unprecedented even
by BSE in the UK at its peak. The pathogenesis of CWD, therefore,
deserves a vigorous research effort. Europeans also need to think
about this problem, and it would be timely and appropriate to increase
CWD surveillance in Europe too." Aguzzi has secured funding from the
National Institutes of Health to investigate CWD, and the effort will
be lead by Christina Sigurdson in his department at the University of
Zurich. KAREN BIRMINGHAM, LONDON

http://www.nature.com/cgi-taf/dynapage.taf?file=/nm/journal/v8/n5/index.html

Prof. Dr. Adriano Aguzzi

http://www.unizh.ch/pathol/neuropathologie/ptn_aag_cv.html

http://www.unizh.ch/pathol/neuropathologie/ptn_aag_rev.html

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

This quote from Dr. Gambetti is especially significant since he is the
rather cautious TSE researcher under contract with the Centers for Disease
Control to examine the brains of individuals who have died of CJD...

Pierluigi Gambetti, director of the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University in Cleveland,
said all deer should be tested for chronic wasting disease before any
processing is done.

"There is no way around it," he said. "Nobody should touch that meat
unless it has been tested."

snip...

http://www.ledger-enquirer.com/

But Byron Caughey, a scientist at the National Institutes of Health's Rocky Mountain lab in Hamilton, Mont., tried to approximate those conditions in a test tube. He and his colleagues took prions from deer and elk with chronic wasting disease and exposed them to normal human proteins.

No one's sure how they work, but prions somehow "convert" ordinary proteins and cause them to fold abnormally. Caughey and his colleagues wanted to see whether deer prions did that to human proteins in test tubes.

They found that some of the human proteins converted to an "apparently toxic" state -- but not many. It was, Caughey says, only about 1/10th to 1/20th the rate they see in deer-to-deer transmission. "It was barely detectable," he says.

To scientists, it was good news, suggesting a "molecular incompatibility" between the species. But skeptics saw it as proof of danger, and one mad cow Web site even posted the story with the headline: "Chronic Wasting Disease transmits to humans."

Says Caughey: "We got a borderline result. As such, people interpret it however they wish, I'm afraid."

To him, it's a mixed message. "We have to acknowledge that there is some risk of CWD going into humans. But it's likely to be relatively low."

Detecting unknowns

If it does transmit to humans, how will anyone know it when they see it?

"Very difficult, at this point, because you ask me to recognize an unknown," says Gambetti. But if it's anything like the mad cow experience, he says, a human form of CWD might look different under the microscope, and possibly act differently, than other human prion illnesses.

snip...

http://www.startribune.com/stories/1751/3391355.html

Conclusions Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.

http://archneur.ama-assn.org/cgi/content/abstract/58/10/1673

Creutzfeldt-Jakob Disease in Deer and Elk Hunters Natalia Murinova, Ali Samii, Melanie Walker, Gregg D. Meekins, Michael Shadlen, Seattle, WA OBJECTIVE: To present the cases of two deer and elk hunters who developed CJD. BACKGROUND: BSE, a prion disease in cattle, has been shown to cause a form of CJD in humans. Recent research has examined the possibility of human infection from deer and elk with Chronic Wasting Disease. DESIGN/METHODS: Case Reports RESULTS: Two recent patients at the Seattle VA hospital developed rapidly progressive dementia. Both patients hunted elk and deer for many years until they became ill. Full text (subscription required): [P03.028] Creutzfeldt-Jakob Disease in Deer and Elk Hunters Natalia Murinova, Ali Samii, Melanie Walker, Gregg D. Meekins, Michael Shadlen, Seattle, WA OBJECTIVE: To present the cases of two deer and elk hunters who developed CJD. BACKGROUND: BSE, a prion disease in cattle, has been shown to cause a form of CJD in humans. Recent research has examined the possibility of human infection from deer and elk with Chronic Wasting Disease. DESIGN/METHODS: Case Reports RESULTS: Two recent patients at the Seattle VA hospital developed rapidly progressive dementia. Both patients hunted elk and deer for many years until they became ill. Neither had a history of travel abroad or iatrogenic exposure to CJD, or a family history of dementia. The first patient, 64, presented to the hospital with mental status changes, including paranoia, fear of poisoning, and inappropriate reactions. He worsened quickly and three months after first presentation, was oriented only to self, followed simple commands, and had an MMSE of 14/30. His neurologic exam was nonfocal. He developed increasing agitation and paranoia, became disoriented and noncommunicative, and developed ataxia and myoclonus. His EEG showed bilateral periodic lateralized epileptiform discharges. A brain MRI with diffusion showed T2 signal abnormalities in patchy distribution in the cerebral cortex. He died almost 4 months after the onset of illness. At autopsy, his brain showed widespread spongiform changes and varying degrees of gliosis sparing no brain region. The patients family stated that he was an avid deer and elk hunter in western Washington. The second patient, 54, presented with balance problems and vertigo dating back several years. Over a two-month period, he developed severe short-term memory loss and quit his job. He had an MMSE of 27/30 at presentation, but on admission two weeks later had a score of 20/30 and was confused and ataxic. His EEG demonstrated diffuse slowing. His brain MRI showed T2 prolongation within the caudate and the putamen nuclei bilaterally. CSF testing for 14-3-3 protein was positive. He died four months after admission. At autopsy, his brain demonstrated diffuse spongiform encephalopathy. Prion protein genotype was homozygous Val/Val at codon 129, and the prion protein was Scrapie protein type 2 by electrophoresis. Per his family, he resided in rural Alaska and hunted deer and elk for food. CONCLUSIONS: Although these cases differ clinically, the neuropathological similarities are striking. Of concern is that they may represent a new entity in the spectrum of prion disease. Neither patient had a known history of consuming deer or elk meat from CWD-endemic areas; however recent reports have expanded the area in which CWD is found in the wild. As it is not currently possible to predict the characteristics of a hypothetical case of CWD-related CJD, the collection and comparison of further CJD cases in consumers of venison will help determine causality and learn more about a potentially devastating emerging disease. Category - Infection/AIDS/Prion Disease SubCategory - Other

http://www.abstracts-on-line.com/abstracts/aan/aolstatement.asp

CONCLUSIONS

With regard to the initial question of the mandate, a theoretical risk for prion
transmission to humans consuming products of CWD affected-cervids of all ages in
countries where CWD exists cannot be excluded. Similarly, transmission risk of prions to
domestic animals cannot be excluded. There is therefore a scientific basis on which to
exclude tissues from animals that carry a CWD risk, from human or animal feed chains.
However, the early and widespread involvement of tissues in CWD infected animals
does not allow to define a SRM list, neither to define any lower age cut off as has been
defined for cattle in relation to BSE. Neither is there sufficient knowledge to define
exclusions or amendment of any SRM rule on the basis of relative genetic resistance to
infection as has been proposed for sheep and goats in the event that evidence would
indicate the probable natural occurrence of BSE in these species 1.
Although available information indicates imports of live Cervidae from NA to EU
and trade in meat products from cervid species as being negligible, it is important to
reach certainty that no transfer of risk takes place through trade of live cervids and its
derived products.
At present, there are no scientific data that CWD is occurring in Cervidae elsewhere
than in those countries from which it has been previously reported. However, systematic
TSE surveillance of cervid populations has either been absent or has only just started in
European countries. Until results of such surveillance become available no conclusion
can be drawn with regard to the occurrence of CWD or similar TSE in the cervid
population of Europe.

RECOMMENDATIONS

Given that the possible risks of exposure relate to the tissues of cervids from NA,
reinforced protection of the cervid population and animal and public health in Europe
could be considered.
Moreover, systematic surveillance is essential to establish the probability of
occurrence and incidence of CWD in the Cervidae populations of Europe. Because of the
complexity of conducting such surveillance on a statistical basis throughout the EU,
initial research should address the susceptibility of European cervid species to TSE’s.
Furthermore, a surveillance programme, which might initially target the examination of
cervids dying in or culled from zoological collections and fallen stock in farmed cervid
populations, prior to decisions on the screening of free-ranging cervids, is recommended.

snip...

http://europa.eu.int/comm/food/fs/sc/ssc/out323_en.pdf

what will it look like cwdCJD ???

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

snip...

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

Asante/Collinge et al, 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;

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

DEPARTMENT OF HEALTH & HUMAN SERVICES PUBLIC HEALTH SERVICE FOOD AND DRUG ADMINISTRATION April 9, 2001 WARNING LETTER 01-PHI-12 CERTIFIED MAIL RETURN RECEIPT REQUESTED Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145 PHILADELPHIA DISTRICT Tel: 215-597-4390 Dear Mr. Raymond: Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23, 2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials. The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act). Our investigation found failure to label your swine feed with the required cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser. In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal. Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain. The above is not intended to be an all-inclusive list of deviations from the regulations. As a manufacturer of materials intended for animal feed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law. We have enclosed a copy of FDA's Small Entity Compliance Guide to assist you with complying with the regulation... blah, blah, blah...

http://www.fda.gov/foi/warning_letters/g1115d.pd

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of
sheep and goats were transmitted to squirrel monkeys (Saimiri
sciureus) that were exposed to the infectious agents only by their
nonforced consumption of known infectious tissues. The asymptomatic
incubation period in the one monkey exposed to the virus of kuru was
36 months; that in the two monkeys exposed to the virus of
Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and
that in the two monkeys exposed to the virus of scrapie was 25 and
32 months, respectively. Careful physical examination of the buccal
cavities of all of the monkeys failed to reveal signs or oral
lesions. One additional monkey similarly exposed to kuru has
remained asymptomatic during the 39 months that it has been under
observation.

PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

1: Dev Biol Stand. 1993;80:9-13. Related Articles, Links

Transmission of human spongiform encephalopathies to experimental
animals: comparison of the chimpanzee and squirrel monkey.

Asher DM, Gibbs CJ Jr, Sulima MP, Bacote A, Amyx H, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of
Neurological Disorders and Stroke, NIH, Bethesda, MD 20992.

The agents of kuru and Creutzfeldt-Jakob disease have been
consistently transmitted from patients with those diseases to
chimpanzees and squirrel monkeys, as well as to other new-world
primates, with average incubation periods of two or three years. No
other animals have been found so consistently susceptible to the
agents in human tissues. More rapid and convenient assays for the
infectious agents would greatly facilitate research on the
spongiform encephalopathies of humans.

PMID: 8270119 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8270119&dopt=Abstract

CWD transmits to primates, humans are primates.

Again, to categorically state that there is no threat from CWD infected
deer to the human population, in my opinion, is completely wrong and
very misleading. This is the exact same thing that was said about BSE
and transmission to humans in the early days.

NOW, we have new phenotypes of BSE/TSE in cattle in Japan, France
and Italy, with from what I understand, negatvie IHC and Histology.
Then you add the new Nor98 sheep scrapie strain recently discovered,
also with negative IHC and Histology, and you have the price of poker
go up. This is very disturbing.

how can it be that all these younger folks that are dying from CJD
in the USA, is just sporadic CJD, when they use to say only the
young get nv/v/bse CJD ? they use to say that kuru type plaques
were only in nv/v/bse CJD, but now that has changed as well.
and the one that really threw a monkey wrench in the gear works
was the nv/v/bse CJD case that was confirmed in a 74 year old,
that too was not suppose to happen, but it did. I think the complete
theory of the nv/v/bse CJD 'ONLY' theory is total cr@p. when mbm
exposed to the BSE agent was exported all over the globe from the UK
and other known BSE countries. BUT to top that, the USA has many
species infected with TSEs that were also rendered into mbm and other
products for human/animal consumption. my question is, how can we
be so lucky as to not have one case of either TSE in cattle or TSE
in humans from cattle, deer, elk, or sheep. It's been well documented
that the USA has TSE in all species above including cattle (re-Marsh
studies). All in all, it's total BSeee;

Bull Shit Encephalopathy...

what will it look like cwdCJD ???

or, what will 'atypical' bse/CJD look like (new atypical strains of
BSE and sheep scrapie, see below)???

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

snip...

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

SCRAPIE - NORWAY: NEW PHENOTYPE
*****************************************
A ProMED-mail post

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

Date: 16 Nov 2003
From: Terry Singletary
Source: The Veterinary Record, 16 Aug 2003 [edited]

[With new information, it appears there is potentially a new phenotype of ‘atypical' BSE/TSE in animals in Japan and France. The article below indicates a new strain of Scrapie, the 'Nor98', which also has negative IHC and histology. Although this article does not suggest a link to the atypical forms seen in cattle, it does seem more than coincidence that there are also appearing different strains, or perhaps atypical strains of scrapie as well. It has been believed for years that there is a link between BSE and scrapie; perhaps this is another bit of research that should be carefully examined. ­ Mod.TG]

Cases of scrapie with unusual features in Norway and designation of a new type, Nor98.
The Veterinary Record, 16 August 2003, vol. 153, no. 7, pp. 202-208(7) Benestad S.L.; Sarradin P.; Thu B.; Schonheit J.; Tranulis M.A.; Bratberg B.

Abstract:
5 cases of scrapie with unusual features have been diagnosed in Norway since 1998. The affected sheep showed neurological signs dominated by ataxia, and had the PrP genotypes homozygous A136 H154 Q171/ A136H154Q171 or heterozygous A136H154Q171/A136R154Q171, which are rarely associated with scrapie. Brain histopathology revealed neuropil vacuolisation essentially in the cerebellar and cerebral cortices; vacuolation was less prominent in the brainstem, and no lesions were observed at the level of the obex. The deposits of PrPSc were mainly in the cortex of the cerebellum and cerebrum, and no PrPSc was detectable by immunohistochemistry or ELISA in the lymphoid tissues investigated.

Western blot analysis showed that the glycotype was different from other known scrapie strains and from the BSE strain. From a diagnostic point of view, these features indicate that this type of scrapie, designated Nor98, could have been overlooked and may be of significance for sampling in scrapie surveillance programmes.

Document Type: Research article ISSN: 0042-4900

DOI (article): NO_DOI
SICI (online): 0042-4900(20030816)153:7L.202;1-
Publisher: BVA Publications

--
ProMED-mail

[see also:
BSE - Japan (08): 9th case, lab findings 20031115.2838
BSE update 2003 (13) 20031110.2782
BSE - Japan (07): 9th case 20031105.2742
BSE, atypical case - Italy: OIE 20031022.2649
BSE - Italy: atypical, suspected 20031012.2576
BSE - Japan (06): atypical 20031009.2547
BSE - Japan (05): atypical 20031008.2526
BSE - Japan (04): atypical 20031007.2511]
.....................tg/pg/dk

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ALSO, this new 'atypical' TSE in cattle has now shown
up in Italy.

SO, my question is, what of the suspected CJD cases with
negative IHC and histology ?

4.5 million with Alzheimer's and how many are misdiagnosed?

up to 13% ???

Subject: Re: Hello Dr. Manuelidis
Date: Fri, 22 Dec 2000 17:47:09 -0500
From: laura manuelidis
Reply-To: laura.manuelidis@yale.edu
Organization: Yale Medical School
To: "Terry S. Singeltary Sr."
References: <39B5561A.87B84A28@wt.net> <39B64574.A4835745@yale.edu> <39B680D8.3872535B@wt.net> <39B66EF1.4CE25685@yale.edu> <39BBB812.425109F@wt.net> <39BE84CB.D7C0C16B@yale.edu> <3A3BA197.7F60D376@wt.net>

Dear Terry,

One of our papers (in Alzheimer's Disease Related Disord. 3:100-109,
1989) in text cites 6 of 46 (13%) of clinical AD as CJD. There may be a
later paper from another lab showing the same higher than expected
incidence but I can't put my hands on it right now. We also have a lot
of papers from 1985 on stating that there are likely many silent
(non-clinical) CJD infections, i.e. much greater than the "tip of the
iceberg" of long standing end-stage cases with clinical symptoms. Hope
this helps.

best wishes for the new year
laura manuelidis
===========

CJD screening may miss thousands of cases

By Steve Mitchell
UPI Medical Correspondent
Published 7/21/2003 3:00 PM
http://www.upi.com/print.cfm?StoryID=20030721-102924-4786r

just what is Alzheimer's ;

PrP in Scrapie and B/A4 in Alzheimer's Disease Show

http://www.bseinquiry.gov.uk/files/mb/m08a/tab29.pdf

1: Ann N Y Acad Sci. 1982;396:131-43. Related Articles, Links

Alzheimer's disease and transmissible virus dementia
(Creutzfeldt-Jakob disease).

Brown P, Salazar AM, Gibbs CJ Jr, Gajdusek DC.

Ample justification exists on clinical, pathologic, and biologic
grounds for considering a similar pathogenesis for AD and the
spongiform virus encephalopathies. However, the crux of the
comparison rests squarely on results of attempts to transmit AD to
experimental animals, and these results have not as yet validated a
common etiology. Investigations of the biologic similarities between
AD and the spongiform virus encephalopathies proceed in several
laboratories, and our own observation of inoculated animals will be
continued in the hope that incubation periods for AD may be even
longer than those of CJD.

PMID: 6758661 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6758661&dopt=Abstract

IN STRICT CONFIDENCE

TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATE

http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf

something to ponder...TSS

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