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From: TSS ()
Subject: CWD Monitoring Identifies Illegal Importation of Deer from New York State, Deer Confiscated
Date: November 16, 2005 at 7:28 am PST

Sgt. Bruce Bonenfant: (603) 271-3127
Jane Vachon: (603) 271-3211
Kent Gustafson: (603) 271-2461
November 9, 2005

CWD Monitoring Identifies Illegal Importation of Deer from New York State
Deer Confiscated; Hunters Reminded to Keep N.H. CWD-Free

CONCORD, N.H. -- Officials from the New Hampshire Fish and Game Department announced today that two hunters have been cited for illegally importing whole deer from New York State, a state where Chronic Wasting Disease (CWD) has been detected. The two deer were confiscated and destroyed as part of ongoing attempts to protect New Hampshire's deer and moose populations from the threat of CWD, a disease -- fatal to some members of the deer family -- that is found in 16 U.S. states and Canadian provinces.

"The threat posed by CWD to New Hampshire's deer herd is of serious concern to us," said Lee Perry, Fish and Game's executive director. "Hunters who hunt out of state need to abide by the rules, which are designed to allow people to bring their deer back to New Hampshire without putting the state's herd at risk." The confiscated deer from New York, not yet butchered, were incinerated to destroy any potentially contagious material.

Current N.H. regulations allow for the importation into New Hampshire of only deboned meat, antlers, upper canine teeth and/or hides or capes with no part of the head attached of deer and elk, from the 16 states and provinces where CWD has been confirmed. These include Alberta, Canada; Colorado; Illinois; Kansas; Minnesota; Montana; Nebraska; New Mexico; New York; Oklahoma; Saskatchewan, Canada; South Dakota; Utah; Wisconsin; West Virginia (the newest addition to the list) and Wyoming. Antlers attached to skull caps or canine teeth must have all soft tissue removed. More information on CWD is found on pages 6 and 60 of the current New Hampshire Hunting Digest (to download the digest, click here and on the orange publication cover), or click here for New Hampshire-related questions and answers about CWD.

A CWD monitoring and testing program for wild deer has been conducted in New Hampshire by Fish and Game biologists since 2002. There is no evidence that this disease exists in the New Hampshire deer herd, and the rules and testing program are designed to prevent exposure via infected animals being imported from other areas.

CWD is a contagious neurological disease that is fatal to deer, moose, elk, and other members of the cervid (deer) family. It is classified as a transmissible spongiform encephalopathy or TSE, and it attacks the brains of infected animals, resulting in their becoming emaciated, exhibiting abnormal behavior and eventually dying.

State officials remind hunters and others who enjoy eating venison that CWD is a wildlife management issue, not a public health issue. There is no evidence that CWD is linked to disease in humans.


Chronic Wasting Disease (CWD) in New Hampshire:
Frequently Asked Questions
(updated October 20, 2005)


why were these two deer not tested for CWD before incineration???

>>>State officials remind hunters and others who enjoy eating venison that CWD is a wildlife management issue, not a public health issue. There is no evidence that CWD is linked to disease in humans. <<<

yep, i remember the same rhetoric with BSE/nvCJD. the above statement is nothing more than wishful thinking. i hope it's true, but the evidence speaks for itself, and the above statement is not 'sound science'. it's the same old song and dance, 'protect the industry at all cost'. why dont you just say, we don't know, but the present science to date shows that ;

The EFSA Journal (2004) 70, 1-7, Opinion on a surveillance programme

for Chronic Wasting Disease in the European Union

3 of 7


1.1. Scientific Steering Committee opinion

At its meeting of 6-7 March 2003, the Scientific Steering Committee (SSC)

produced an opinion on “Chronic Wasting Disease and tissues that might carry a

risk for human and animal feed chains”. In this opinion, the SSC recommended

the instigation of a surveillance program in the EU, 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.


8. Even though human TSE-exposure risk through consumption of game from European

cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn

due to the overall lack of scientific data. In particular the US data do not clearly exclude

the possibility of human (sporadic or familiar) TSE development due to consumption of

venison. The Working Group thus recognizes a potential risk to consumers if a TSE

would be present in European cervids. It might be prudent considering appropriate

measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues

from the human food chain, which would greatly reduce any potential risk for consumers.

However, it is stressed that currently, no data regarding a risk of TSE infections from

cervid products are available.

Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies

Location: Virus and Prion Diseases of Livestock

Title: Experimental Second Passage of Chronic Wasting Disease (Cwd-Mule Deer) Agent to Cattle


Hamir, Amirali
Kunkle, Robert - bob
Miller, Janice - ARS RETIRED
Greenlee, Justin
Richt, Juergen

Submitted to: Journal Of Comparative Pathology
Publication Acceptance Date: July 25, 2005
Publication Date: N/A

Interpretive Summary: To compare the findings of experimental first and second passage of chronic wasting disease (CWD) in cattle, 6 calves were inoculated into the brain with CWD-mule deer agent previously (first) passaged in cattle. Two other uninoculated calves served as controls. Beginning 10-12 months post inoculation (PI), all inoculates lost appetite and weight. Five animals subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months PI, all cattle had been euthanized because of poor prognosis. None of the animals showed microscopic lesions of spongiform encephalopathy (SE) but the CWD agent was detected in their CNS tissues by 2 laboratory techniques (IHC and WB). These findings demonstrate that inoculated cattle amplify CWD agent but also develop clinical CNS signs without manifestation of microscopic lesions of SE. This situation has also been shown to occur following inoculation of cattle with another TSE agent, namely, sheep scrapie. The current study confirms previous work that indicates that the diagnostic tests currently used for confirmation of bovine spongiform encephalopathy (BSE) in the U.S. would detect CWD in cattle, should it occur naturally. Furthermore, it raises the possibility of distinguishing CWD from BSE in cattle due to the absence of microscopic lesions and a unique multifocal distribution of PrPres, as demonstrated by IHC, which in this study, appears to be more sensitive than the WB.
Technical Abstract: To compare clinicopathological findings of first and second passage of chronic wasting disease (CWD) in cattle, a group of calves (n=6) were intracerebrally inoculated with CWD-mule deer agent previously (first) passaged in cattle. Two other uninoculated calves served as controls. Beginning 10-12 months post inoculation (PI), all inoculates lost appetite and lost weight. Five animals subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months PI, all cattle had been euthanized because of poor prognosis. None of the animals showed microscopic lesions of spongiform encephalopathy (SE) but PrPres was detected in their CNS tissues by immunohistochemistry (IHC) and Western blot (WB) techniques. These findings demonstrate that intracerebrally inoculated cattle not only amplify CWD PrPres but also develop clinical CNS signs without manifestation of morphologic lesions of SE. This situation has also been shown to occur following inoculation of cattle with another TSE agent, scrapie. The current study confirms previous work that indicates the diagnostic techniques currently used for confirmation of bovine spongiform encephalopathy (BSE) in the U.S. would detect CWD in cattle, should it occur naturally. Furthermore, it raises the possibility of distinguishing CWD from BSE in cattle due to the absence of neuropathologic lesions and a unique multifocal distribution of PrPres, as demonstrated by IHC, which in this study, appears to be more sensitive than the WB.

----- Original Message -----
From: "Terry S. Singeltary Sr."
Sent: Wednesday, October 19, 2005 10:47 AM
Subject: Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)

##################### Bovine Spongiform Encephalopathy

From: TSS ()
Subject: Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)
Date: October 19, 2005 at 8:33 am PST

0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel
Monkeys (Saimiri sciureus)
Richard F. Marsh,1, Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C.
Department of Animal Health and Biomedical Sciences, University of
Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of
Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska
68178,4 Department of Veterinary Molecular Biology, Montana State
University, Bozeman, Montana 597183

Received 3 May 2005/ Accepted 10 August 2005

Chronic wasting disease (CWD) is an emerging prion disease of deer and elk.
The risk of CWD transmission to humans following exposure to CWD-infected
tissues is unknown. To assess the susceptibility of nonhuman primates to
CWD, two squirrel monkeys were inoculated with brain tissue from a
CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a
progressive neurodegenerative disease and were euthanized at 31 and 34
months postinfection. Brain tissue from the CWD-infected squirrel monkeys
contained the abnormal isoform of the prion protein, PrP-res, and displayed
spongiform degeneration. This is the first reported transmission of CWD to


* Corresponding author. Mailing address: Department of Medical Microbiology
and Immunology, Creighton University, 2500 California Plaza, Omaha, NE
68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail: .



Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21
0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

##################### Bovine Spongiform Encephalopathy #####################

Chronic wasting disease moves west
Casper Star Tribune November 3, 2005

LANDER -- State and now tribal game managers have detected three new cases
of chronic wasting disease, a fatal brain disease that can affect all
members of Wyoming’s deer family. The new locations north and west of
Thermopolis. The disease had not previously been detected in this area. “We’
re always concerned when we have a geographic expansion of this disease,”
said Terry Cleveland, director of the Wyoming Game and Fish Department.

Well, duh , how about closing down your 23 elk winter feedlots in Wyoming --
another year or two and this disease is really going to explode out of the
feedlots into Yellowstone and Grant Tetons Natl Parks.

The Ten Biggest Lies in CWD:

1- "CWD occurs naturally at a low background level in wild animals." [All
CWD to date -- including zoo and game farm -- traces back to the original
scrapie sheep-to-deer transmission at CDOW Foothills Research Station in
1967. No natural prion disease has ever been found in any animal species
anywhere in the world, outside of human.]

2- "CWD does not transmit to human, so hunters are not at risk." [Too soon
to say, TSEs can have 50 year incubations. No test -- no one knows how to
distinguish CWD in human from sporadic CJDs. Epidemiology has poor power.
Preclinical cases can propagate without species barrier via blood and tissue
donation. Same was said about mad cow disease, wrongly as it turned out.]

3- "I've hunted for years in the CWD hot zone, eaten brain sausage, and I
feel great, therefore it must be ok." [Needs no comment, too stupid, yet 2
WY and 1 CO researcher have said this repeatedly to Denver Post.]

4- "The deer I shot tested negative, so no problem." [Preclinical animals
may be *more* infectious than overtly sick animals because of higher prion
abundance in more commonly eaten tissues. Game processors commonly pool 100
animals prior to a rendering run; there is no effective way to decontaminate
equipment between batches. False negatives are quite common when only obex
is used. Hunters commonly sever the spinal cord without wearing Biohazard
Level III clothing.]

5- "Absence of evidence is evidence of absence" [Needs no comment, too
stupid, yet most common spin put out to journalists by CDC, Colorado

6- "CWD is restricted to cervids; a species barrier prevents transmission to
bears, wolves, wolverines, cougars, livestock etc." [If you don't look, you
don't find: zero testing has been on predators and scavengers, other than
preliminary cougar and raccoon work. Species barriers are initial
inefficiencies, not absolute; second and later passages are highly

7- "Trust me, I'm an expert." [Few officials have read or understood the
7028 published papers on prion disease. Few veterinarians and
epidemiologists are trained in protein chemistry and molecular biology, the
realm of prion disease.]

8- "You can test your way out of this disease." [Facilities like game farms
become hopelessly contaminated; culling clinically ill animals is too late;
live animal tests are difficult and not sufficiently sensitive.]

9- "CWD is self-limited to the Tri-State area because of natural selection."
[Animals with CWD can reach reproductive age before dying; migrations cover
huge areas; game farm trafficking is effectively unregulated; escapes,
releases, entrapment of wild animals and rut breakin are common.]

10-- "There's scientific uncertainty about transmission mechanisms, so
nothing can be done about CWD in the wild." [Containment facilities are
notorious for high incidence of CWD; artifically high winter densities --
the govt runs 23 huge elk feedlots in Wyoming -- mimic game farms and
research facilities. The Grand Teton feedlots could be closed and elk

Possible CWD Surprises (over next year or two):

-- A live animal blood test might be approved. If sufficiently sensitive (no
false negatives, esp. animals early on in infection), animal roundups for
testing might be presented as an option. However, concentration of animals
in a confinement facility such as winter feedlot would only result in
further spread of CWD prior to ability to cull and incinerate infected

-- Transmission to cows or sheep on public (or private) lands might be
documented. This would bring calls for eradication of deer and elk or
restriction to national parks, rather than allotment retirement. So far,
experimental transmission to cattle has been difficult. Back transmission to
sheep has not been tested. There are many evolving strains of CWD as each
species has a unique prion protein sequence. Species barriers can change
dramatically upon passage to a new species such as moose.

-- Transmission to humans might be proven. This could cause a plunge in
out-fof-state hunting tags which in Colorado account fo over half the Fish
and Game budget. Right now, CWD has no distinctive signature (unlike florid
plaques of mad cow disease) that separates it from sporadic CJD. It is all
but impossible to prove epidemiologically as hunters are hard to track over
long incubation periods and often have other exposures such as decades of
eating mutton or beef or UK visits. Asymptomatic hunters could spread the
disease widely into non-hunters through blood donations, corneal
transplants, endoscopes, root canals, etc.

What species have gotten CWD already? Note CWD has already passaged to
carnivore and primate:

CWD-Donor CWD-Recipient Method Year
sheep white-tail deer shared pen at Foothills 1967+
sheep black-tail deer shared pen at Foothills 1967+
sheep mule deer shared pen at Foothills 1967+
sheep rocky mtn elk shared pen at Foothills 1967+
sheep rocky mtn elk intracerebral injection 2004
cervid moose unknown Colorado wild 2005
mule deer cow intracerebral injection 2001
white-tail cow in vitro conversion 2000
white-tail sheep in vitro conversion 2000
mule deer goat intracerebral injection 2001
mule deer sheep intracerebral injection 2001
white-tail human in vitro conversion 2000
mule deer squirrel monkey intracerebral injection 2005
white-tail ferret intracerebral injection 1998
ferret hamster intracerebral injection 1998
elk humanized mice intracerebral injection 2005*
elk elkized mice intracerebral injection 2005
mule deer muleized mice intracerebral injection 2004

*first passage negative, second passage underway

Abstracts for published scientific articles on CWD: PubMed using "chronic
wasting disease" as search term. Also you can sign up for automatic email
alerts there.

Extensive history of CWD: use 'advanced search' google restricted to url, Some great eat-more-rotten-venison-today newspaper
quotes from CO and WY game and fish staffers. An email/phone directory to
about 200 scientists working in prion disease can be found at

Some recent abstracts

Vet Rec. 2003 Jul 26;153(4):121-3. [no abstract, unclear if raccoon, another
Carnivora, became infected]
Experimental inoculation of scrapie and chronic wasting disease agents in
raccoons (Procyon lotor).
Hamir AN, Miller JM, Cutlip RC, Stack MJ, Chaplin MJ, Jenny AL, Williams ES.
National Animal Disease Center, ARS, USDA, 2300 Dayton Avenue, PO Box 70,
Ames, IA 50010, USA.

PLOS Biology 2005: "Unlike BSE, CWD is not thought to be transmitted through
feed. But three species of cervids are naturally susceptible, and the
question arises of how many other species might be in danger. To help answer
that question, Michael Samuel and colleagues at the University of Wisconsin
are staking out deer carcasses to see which scavengers come to feed. With
flashlit photography, they've discovered “an amazing cast of characters,”
including hawks, owls, crows, dogs, cats, coyotes, raccoons, skunks, mink,
foxes, and opossums (Figure 2). Mammalian scavengers in the state's
CWD-affected region will later be examined for disease."

J Vet Diagn Invest. 2004 Jul;16(4):316-21. [cwd looks just like scrapie, no
surprise given inadvertent transmission at Foothills Research Station]
Transmission of sheep scrapie to elk (Cervus elaphus nelsoni) by
intracerebral inoculation: final outcome of the experiment.
Hamir AN, Miller JM, Cutlip RC, Kunkle RA, Jenny AL, Stack MJ, Chaplin MJ,
Richt JA.
National Animal Disease Center, ARS, USDA, Ames, IA 50010, USA.

This is a final report of an experimental transmission of sheep scrapie
agent by intracerebral inoculation to Rocky Mountain elk (Cervus elaphus
nelsoni). It documents results obtained in experimental (n = 6) and control
(n = 2) elk. During the first 2 years postinoculation (PI), 3 animals died
or were euthanized because of infection or injuries other than spongiform
encephalopathy (SE). In years 3 and 4 PI, 3 other inoculated elk died after
brief terminal neurological episodes. Necropsy of these animals revealed
moderate weight loss but no other gross lesions. Microscopically,
characteristic lesions of SE were seen throughout the brain and spinal cord,
and the tissue was positive for proteinase K-resistant prion protein
(PrPres) by immunohistochemistry (IHC) and by Western blot.
Scrapie-associated fibrils (SAF) were observed by negative-stain electron
microscopy in the brain of elk with neurologic signs. PrPres and SAF were
not detected in the 3 inoculated elk necropsied during the first 2 years or
in the 2 control animals. Retrospective analysis of the gene-encoding cervid
PrP revealed a polymorphism at codon 132. The elk with SE were either
homozygous (MM) or heterozygous (LM). These findings confirm that
intracerebral inoculation of sheep scrapie agent results in SE with
accumulations of PrPres in the central nervous system of elk. Based on
morphologic and IHC findings, the experimentally induced SE cannot be
distinguished from chronic wasting disease of elk with currently available
diagnostic techniques.

J Gen Virol. 2005 Aug;86(Pt 8):2127-34. [explores possible natural occurence
of genetic resistance due to prion gene polymorphisms]
Low frequency of PrP genotype 225SF among free-ranging mule deer (Odocoileus
hemionus) with chronic wasting disease.
Jewell JE, Conner MM, Wolfe LL, Miller MW, Williams ES.
Department of Veterinary Sciences, University of Wyoming, Wyoming State
Veterinary Laboratory, Laramie, WY.

The prion protein (PrP) gene was characterized in 1482 free-ranging mule
deer (Odocoileus hemionus) from Wyoming and Colorado. Using DNA sequences
from 363 deer, dimorphisms at codons 20 (aspartate/glycine) and 225 [serine
(S)/phenylalanine (F)] were found; silent changes occurred at codons 131
(tyrosine) and 247 (isoleucine). The remaining samples were surveyed for
codon 225 genotype and all were characterized for chronic wasting disease
(CWD) infection status. A total of 112 deer with the genotype 225SF or FF
were found, of which one was CWD-positive; 1370 were 225SS, with 289
positive for CWD. Among CWD-negative deer, the frequency of 225SF/FF
genotypes was 9.3 % but among CWD-positive deer it was only 0.3 %. For all
samples combined, CWD status was not independent of codon 225 genotype
(P<0.0001). The odds that a deer of the 225SS genotype was CWD-infected were
30 times greater (95 % confidence intervals=4-213) than for a 225SF deer.
The proportion of 225SF animals in sampled subpopulations varied from 0 to
18 %; the CWD prevalence varied from 0 to 25 %. However, no relationship was
observed between genotype frequency and CWD prevalence in different areas.
The PrP sequences of experimentally infected mule deer were analysed from
pre-existing projects and 10 animals were found with 225SF genotypes, all of
which were positive for CWD. Data available from some of these animals
suggest that the 225SF genotype could be associated with longer incubation
periods in CWD infection compared with the 225SS genotype.

J Vet Diagn Invest. 2003 Jul;15(4):320-3. [O'Rourke monoclonal antibody can
be used in highly inconvenient live test using tonsil biopsy]
Abundant PrP(CWD) in tonsil from mule deer with preclinical chronic wasting
O'Rourke KI, Zhuang D, Lyda A, Gomez G, Williams ES, Tuo W, Miller MW.
Agricultural Research Service, U.S. Department of Agriculture Pullman, WA
99164, USA.

A monoclonal antibody dot-blot assay was used to evaluate detergent lysates
of tonsil tissue from mule deer to detect PrP(CWD), the marker for the
cervid transmissible spongiform encephalopathy chronic wasting disease
(CWD). Samples of formalin-fixed brain and tonsil tissues from mule deer
were examined for PrP(CWD) using immunohistochemistry (IHC) with Mab
F99/97.6.1, the gold standard for diagnosis of preclinical CWD. The
contralateral tonsil from each of the 143 deer was prepared for confirmatory
IHC and as a 10% (wt/vol) detergent lysate without purification or
enrichment steps for monoclonal antibody dot-blot assay. PrP(CWD) was
detected by dot-blot assay in 49 of 50 samples considered positive by IHC.
Forty-eight of the positive samples were evaluated with a quantitative
dot-blot assay calibrated with recombinant PrP. Tonsillar PrP(CWD)
concentrations ranged from 34 to 1,188 ng per 0.5 mg starting wet weight of
tissue. The abundant PrP(CWD) in mule deer tonsil will facilitate
development and validation of high-throughput screening tests for CWD in
large populations of free-ranging deer.

J Vet Diagn Invest. 2003 May;15(3):274-7 [no transmission observed yet
deer-to-cattle in the field]
Survey of cattle in northeast Colorado for evidence of chronic wasting
disease: geographical and high-risk targeted sample.
Gould DH, Voss JL, Miller MW, Bachand AM, Cummings BA, Frank AA.
Department of Pathology, Veterinary Medicine and Biomedical Sciences,
Colorado State University, Ft. Collins, CO.

A geographically targeted survey of potentially high-risk, adult cattle in
chronic wasting disease (CWD)-endemic areas in Colorado was initiated to
assess the possibility of the spread of CWD from deer to cattle under
natural conditions. Surveyed cattle were sympatric with free-roaming deer in
geographically defined areas where CWD occurs and where CWD prevalence has
been estimated. To qualify for inclusion in the survey, cattle had to be at
least 4 years old and had to have spent a minimum of 4 years in surveyed
areas. Brains from culled cattle were examined microscopically and
immunohistochemically for tissue alterations indicative of a transmissible
spongiform encephalopathy (TSE). Two hundred sixty-two brains were suitable
for evaluation and were found to lack changes indicative of a TSE infection.
Prion deposition was not demonstrable using a method involving formic acid
and proteinase-K treatment before application of monoclonal antibody to
bovine prion protein (F99/97.6.1). Some incidental neuropathologic changes
unrelated to those of TSEs were detected. Findings from this study suggest
that large-scale spread of CWD from deer to cattle under natural range
conditions in CWD-endemic areas of northeast Colorado is unlikely.

J Gen Virol. 2004 May;85(Pt 5):1339-46.
Polymorphisms in the prion precursor functional gene but not the pseudogene
are associated with susceptibility to chronic wasting disease in
white-tailed deer.
O'Rourke KI, Spraker TR, Hamburg LK, Besser TE, Brayton KA, Knowles DP.
US Department of Agriculture, Agricultural Research Service Pullman, WA
99164, USA.

Chronic wasting disease (CWD) status and PrP genotypes were determined for a
group of 133 wild white-tailed deer in a 780 acre enclosure in western
Nebraska, USA. Approximately half of the deer tested showed evidence of PrPd
in the brainstem or lymphoid tissues. Four PRNP alleles encoding amino acid
substitutions were identified, with substitutions at residues 95 (Q-->H), 96
(G-->S) or 116 (A-->G), each with serine (S) at residue 138. In addition, a
processed pseudogene with two alleles encoding five or six copies of the
octapeptide repeat was identified in 26 % of the deer. Both alleles encoded
asparagine (N) at residue 138. The functional gene alleles sorted into five
major diploid genotypes and four rare genotypes. Although all five major
diploid genotypes were found in deer with CWD, unaffected deer were less
likely to have the allele QGAS and more likely to have QSAS compared with
CWD-affected deer. Late-stage disease (PrPd in brainstem) was noted in deer
less than 1 year of age, although no single genotype was associated with
this rapid neuroinvasion. Early-stage disease (PrPd distribution limited to
the lymphoid system) was observed in deer estimated to be more than 5 years
old, suggesting that they were infected as adults or that the incubation
time might be extremely long in some individuals. The pseudogene was found
in deer of all major PRNP genotypes and was not correlated with CWD status.
The large number of susceptible genotypes and the possibility of
adult-to-adult transmission suggest that much of the white-tailed deer
population may be at risk for disease following exposure to CWD, despite the
association of specific genotypes with CWD noted here.

Nature. 2003 Sep 4;425(6953):35-6. [incidence of cwd in confined herds
becomes astronomic]
Prion disease: horizontal prion transmission in mule deer.
Miller MW, Williams ES.
Colorado Division of Wildlife, Wildlife Research Center, Fort Collins,
Colorado 80526, USA.

Epidemics of contagious prion diseases can be perpetuated by horizontal
(animal to animal) and maternal (dam to offspring, before or after birth)
transmission, but the relative importance of each mechanism is unclear. Here
we compare the incidence of chronic wasting disease (CWD) in captive mule
deer (Odocoileus hemionus) that is attributable to horizontal or maternal
transmission. We find that horizontal transmission is remarkably efficient,
producing a high incidence of disease (89%) in a cohort of deer in which
maternal transmission was improbable. Our results indicate that horizontal
transmission is likely to be important in sustaining CWD epidemics.

Vet Rec. 2004 Sep 4;155(10):295-302. [obex testing alone seriously
understates actual cwd incidence]
Variable patterns of distribution of PrP(CWD) in the obex and cranial
lymphoid tissues of Rocky Mountain elk (Cervus elaphus nelsoni) with
subclinical chronic wasting disease.
Spraker TR, Balachandran A, Zhuang D, O'Rourke KI.
Colorado State University, Fort Collins, CO, USA.

Sections of medulla oblongata, taken at the level of the obex, palatine
tonsil and medial retropharyngeal lymph node from 10,269 captive Rocky
Mountain elk (Cervus elaphus nelsoni), were examined by immunohistochemical
staining with monoclonal antibody for the prion protein associated with the
transmissible spongiform encephalopathy of cervids, chronic wasting disease
(PrP(CWD)). The protein was detected in 226 of them. On the basis of the
anatomical location of the deposits in the brainstem of 183 elk, four
distinct patterns of distribution of PrP(CWD) within the parasympathetic
region of the dorsal motor nucleus of the vagus nerve and the adjacent
nuclei were observed. Mild gross lesions of chronic wasting disease (serous
atrophy of fat) were observed in only three elk, all with spongiform
degeneration; the other elk were considered to be in the preclinical stage
of the disease. In contrast with the relatively predictable distribution of
prion protein (PrP) in the brain and cranial nodes of sheep and mule deer,
the distribution of PrP(CWD) in the brain and nodes of the elk was more
variable and unrelated to their PrP genotype. One hundred and fifty-five of
the 226 positive elk had deposits of PrP(CWD) in the brainstem and lymphoid
tissues, 43 had deposits only in the lymphoid tissue and 28 had deposits
only in the brainstem. ........snip.........end


----- Original Message -----
From: "Terry S. Singeltary Sr."
Sent: Thursday, November 03, 2005 9:17 PM
Subject: Wyoming Feedgrounds Double as CWD Time Bomb

##################### Bovine Spongiform Encephalopathy

From: TSS ()
Subject: Wyoming Feedgrounds Double as CWD Time Bomb
Date: November 3, 2005 at 7:02 pm PST

Wyoming Feedgrounds Double as CWD Time Bomb

By Bill Schneider, 11-03-05

Elk hunters in the Greater Yellowstone Area should start thinking like they’
re living below a huge dam with cracks in it. They know what’s going to
happen. It’s only a matter of time, and it’s guaranteed to be devastating.

That dam is located in northwestern Wyoming, mostly in the Upper Green River
Basin, but also around Jackson. It manifests itself in the form of
twenty-two state-managed feedgrounds, where the State of Wyoming
collectively feeds up to 20,000 elk during severe winters, and the famous
National Elk Refuge managed by the U.S. Fish and Wildlife Service.

It’s a time-honored principle of wildlife management that when you feed
wildlife, you pay a big price for it. For elk hunters—and everybody else who
likes seeing elk—that bill will soon be due because Wyoming’s feedgrounds
double as a Chronic Wasting Disease time bomb ready to explode.

“That’s true,” answers Robert Hoskins, president of the Dubois Wildlife
Association and long-time opponent to the feedgrounds, when asked if he
agrees. “It is a time bomb because the State of Wyoming refuses to consider
any proposal to close the feedgrounds. We’re just waiting for CWD to hit. We
’re doing everything we can to stop the feeding, but we are rebuffed every
time. The livestock industry is in complete control of this whole thing.”

“It’s an eventuality,” agrees Tom Roffe, chief of wildlife health and
veterinarian for the Fish and Wildlife Service in Bozeman. “It’s not a
matter of if, but when.”

And when it happens, what will we do? The answer to that question should
send a shiver up the spine of every elk hunter because the solution could be
worse than the problem.

Wyoming has documented cases of CWD in both elk and mule deer dating back
thirty years. The disease has been slowly moving northwest from the
southeastern corner of the state. In recent years, there have been several
documented CWD cases in the Thermopolis area, which is only about thirty air
miles from the Upper Green River Basin, but still east of the Continental
Divide. Roffe finishes the grim picture by pointing out that radio-collared
elk have been documented traveling from the feedgrounds back and forth over
the Divide. For elk hunters, this is like looking out your window and seeing
water starting to leak through cracks in the dam.

In CWD outbreaks on game farms, the disease has killed up to 50 percent of
the animals, but in wild populations, mortality is much lower. Roffe likens
the feedgrounds to a situation somewhere between free-ranging elk and
captive herds on game farms, so mortality will be high, but probably not 50
percent. “Nobody knows what it will be,” he says.

But even 50 percent mortality on the feedgrounds might seem like a minor
problem compared to the solution to a CWD epidemic. Montana, for example,
has formalized a CWD response plan that includes an option for killing all
elk in the area around the outbreak. Wyoming has a plan, too, but it does
not include the “depopulation” option. Not yet!

Wyoming’s plan is, according to both Roffe and Hoskins, basically waiting
for the disease to hit the feedgrounds and then deal with it in three
ways—reduce density, reduce feeding, and spread out the elk herd.

“Why would you not do that before it gets there,” Roffe asks. “Wyoming’s
plan will be too little, too late.” He compares it to waiting for avian flu
pandemic instead of trying to prevent it from happening.

Hoskins points out the absence of barriers for elk carrying CWD from moving
from the feedgrounds to Idaho and Montana and infecting populations there.
“It’s a slow process, but it will get to Montana and Idaho.”

“Prevention is the best action,” Roffe insists. “If we want to have a
success, there has to be a prevention plan.”

The punch line is: Nobody has a good solution for dealing with an outbreak
of CWD in our wild elk herds, and it’s almost guaranteed to happen in
Wyoming. The best case scenario might be letting the disease run its course
and lose a huge percentage of our elk populations. The worse case scenario
might be trying to eradicate it. Wildlife officials are understandably
skittish about saying this out loud, but any attempt to eradicate the
disease would involve killing lots of elk. On the Wyoming feedgrounds, in
fact, it could easily mean killing many thousands.

There are ways to minimize the risk in advance, and stopping the feeding
tops the list. In reality, the feedgrounds are no different than game farms,
which have been banned in Wyoming since the early 1970s. They concentrate
wildlife and create circumstances favorable to spreading the disease.
Hoskins, never lost for words or energy to stop the feeding, describes the
feedgrounds as “petri dishes for spreading disease.”

Roffe agrees. “The feedgrounds are bringing elk into close proximity and
enhancing the probability of spreading CWD.”

Let’s be clear on one key point. This is not a Wyoming problem. Instead, it’
s a massive threat to elk populations throughout the Rocky Mountain West. We
can only hope Wyoming sees the gravity of the situation and acts before it’s
too late.

Wyoming has resisted placing feedgrounds east of the Continental Divide.
Instead, the state has concentrated on protecting key winter range to keep
the elk population high, a similar strategy used successfully in Montana.
Now, Wyoming wildlife officials and conservation organizations need to grow
the backbone necessary to take on the livestock lobby for the benefit of
wildlife and hunters and close the feedgrounds west of the Divide.



Animal Prion Diseases Relevant to Humans (unknown types?)
Thu Oct 27, 2005 12:05

About Human Prion Diseases /
Animal Prion Diseases Relevant to Humans

Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.

Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)

Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here;

p.s. please note the 47 PENDING CASES to Sept. 2005

p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ???

p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???

p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN???

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

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."


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.




#################### ####################


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:


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.


Risk for Transmission to Humans
Epidemiologic Studies
The increasing detection of CWD in a wider geographic area and the presumed foodborne transmission of BSE to humans, resulting in cases of vCJD, have raised concerns about the possible zoonotic transmission of CWD (32). In the late 1990s, such concerns were heightened by the occurrence of CJD among three patients 30 years of age who were deer hunters or ate deer and elk meat harvested by family members (Table 2). However, epidemiologic and laboratory investigations of these case-patients indicated no strong evidence for a causal link between CWD and their CJD illness (33). None of the patients were reported to have hunted deer or eaten deer meat harvested in the CWD-endemic areas of Colorado and Wyoming. Such a history in unusually young CJD patients, if present, would have supported a causal link with CWD. Moreover, the testing of brain tissues from >1,000 deer and elk harvested from areas where the patients hunted or their venison originated did not show any evidence of CWD (33). In addition, the lack of homogeneity in the clinicopathologic manifestation and codon 129 of the prion protein gene among the three patients suggested that their illnesses could not be explained by exposure to the same prion strain. In vCJD, homogeneity of the genotype at codon 129 and the clinical and pathologic phenotype were attributed to the patients' exposure to the same prion strain, the agent of BSE.

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ≈22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.

In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient's disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36).

The second patient died from an illness lasting <16 months. The patient's illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient's death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient's parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin.

Recently, rare neurologic disorders resulting in the deaths of three men who participated in "wild game feasts" in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick's disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming.

In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35).

To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient's CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient's illness was consistent with the MM1 CJD phenotype.

Despite the decades-long endemicity of CWD in Colorado and Wyoming, the incidence of CJD and the age distribution of CJD case-patients in these two states are similar to those seen in other parts of the United States. From 1979 to 2000, 67 CJD cases from Colorado and 7 from Wyoming were reported to the national multiple cause-of-death database. The average annual age-adjusted CJD death rate was 1.2 per million persons in Colorado and 0.8 in Wyoming. The proportion of CJD patients who died before age 55 in Colorado (13.4%) was similar to that of the national (10.2%). The only CJD case-patient <30 years of age in Colorado had iatrogenic CJD linked to receipt of human growth hormone injections. CJD was not reported in persons <55 years of age in Wyoming during the 22-year surveillance period.


The lack of evidence of a link between CWD transmission and unusual cases of CJD, despite several epidemiologic investigations, and the absence of an increase in CJD incidence in Colorado and Wyoming suggest that the risk, if any, of transmission of CWD to humans is low. Although the in vitro studies indicating inefficient conversion of human prion protein by CWD-associated prions raise the possibility of low-level transmission of CWD to humans, no human cases of prion disease with strong evidence of a link with CWD have been identified. However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease. The level and frequency of human exposure to the CWD agent may increase with the spread of CWD in the United States. Because the number of studies seeking evidence for CWD transmission to humans is limited, more epidemiologic and laboratory studies should be conducted to monitor the possibility of such transmissions. Studies involving transgenic mice expressing human and cervid prion protein are in progress to further assess the potential for the CWD agent to cause human disease. Epidemiologic studies have also been initiated to identify human cases of prion disease among persons with an increased risk for exposure to potentially CWD-infected deer or elk meat (47). If such cases are identified, laboratory data showing similarities of the etiologic agent to that of the CWD agent would strengthen the conclusion for a causal link. Surveillance for human prion diseases, particularly in areas where CWD has been detected, remains important to effectively monitor the possible transmission of CWD to humans. Because of the long incubation period associated with prion diseases, convincing negative results from epidemiologic and experimental laboratory studies would likely require years of follow-up. In the meantime, to minimize the risk for exposure to the CWD agent, hunters should consult with their state wildlife agencies to identify areas where CWD occurs and continue to follow advice provided by public health and wildlife agencies. Hunters should avoid eating meat from deer and elk that look sick or test positive for CWD. They should wear gloves when field-dressing carcasses, bone-out the meat from the animal, and minimize handling of brain and spinal cord tissues. As a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified.



Issued: Monday, 28 August 2000

A team of researchers led by Professor John Collinge at the Medical
Research Council Prion Unit1 report today in the Proceedings of the
National Academy of Sciences, on new evidence for the existence of a
'sub-clinical' form of BSE in mice which was unknown until now.

The scientists took a closer look at what is known as the 'species
barrier' - the main protective factor which limits the ability of
prions2 to jump from one species to infect another. They found the mice
had a 'sub-clinical' form of disease where they carried high levels of
infectivity but did not develop the clinical disease during their normal
lifespan. The idea that individuals can carry a disease and show no
clinical symptoms is not new. It is commonly seen in conventional
infectious diseases.

Researchers tried to infect laboratory mice with hamster prions3 called
Sc237 and found that the mice showed no apparent signs of disease.
However, on closer inspection they found that the mice had high levels
of mouse prions in their brains. This was surprising because it has
always been assumed that hamster prions could not cause the disease in
mice, even when injected directly into the brain.

In addition the researchers showed that this new sub-clinical infection
could be easily passed on when injected into healthy mice and hamsters.

The height of the species barrier varies widely between different
combinations of animals and also varies with the type or strain of
prions. While some barriers are quite small (for instance BSE easily
infects mice), other combinations of strain and species show a seemingly
impenetrable barrier. Traditionally, the particular barrier studied here
was assumed to be robust.

Professor John Collinge said: "These results have a number of important
implications. They suggest that we should re-think how we measure
species barriers in the laboratory, and that we should not assume that
just because one species appears resistant to a strain of prions they
have been exposed to, that they do not silently carry the infection.
This research raises the possibility, which has been mentioned before,
that apparently healthy cattle could harbour, but never show signs of, BSE.

"This is a timely and unexpected result, increasing what we know about
prion disease. These new findings have important implications for those
researching prion disease, those responsible for preventing infected
material getting into the food chain and for those considering how best
to safeguard health and reduce the risk that theoretically, prion
disease could be contracted through medical and surgical procedures."


(OFFICE HOURS) OR 07818 428297 OR 0385 774357 (OUT-OF-OFFICE-HOURS) OR
ON 00 61 3 8344 3995 (DURING OFFICE HOURS) OR 00 61 3 9443 0009


Professor Collinge is a consultant neurologist and Director of the newly
formed MRC Prion Unit based at The Imperial College School of Medicine
at St Mary's Hospital. He is also a member of the UK Government's
Spongiform Encephalopathy Advisory Committee (SEAC). The MRC prion unit
is was set up in 1999, and its work includes molecular genetic studies
of human prion disease and transgenic modelling of human prion diseases.

Prions are unique infectious agents that cause fatal brain diseases such
as Creutzfeldt-Jakob disease (CJD) in humans and scrapie and BSE (mad
cow disease) in animals. In some circumstances prions from one species
of animals can infect another and it is clear that BSE has done this to
cause the disease variant CJD in the UK and France. It remains unclear
how large an epidemic of variant CJD will occur over the years ahead.

The strain of prion used here to infect the mice is the Sc237 strain
(also known as 263K) which infects hamsters, and until now was assumed
not to infect mice.

This research was funded by the Medical Research Council and Wellcome Trust.

The Medical Research Council (MRC) is a national organisation funded by
the UK tax-payer. Its business is medical research aimed at improving
human health; everyone stands to benefit from the outputs. The research
it supports and the scientists it trains meet the needs of the health
services, the pharmaceutical and other health-related industries and the
academic world. MRC has funded work which has led to some of the most
significant discoveries and achievements in medicine in the UK. About
half of the MRC's expenditure of £345 million is invested in over 50 of
its Institutes and Units, where it employs its own research staff. The
remaining half goes in the form of grant support and training awards to
individuals and teams in universities and medical schools.

The Wellcome Trust is the world's largest medical research charity with
a spend of some £600 million in the current financial year 1999/2000.
The Wellcome Trust supports more than 5,000 researchers, at 400
locations, in 42 different countries to promote and foster research with
the aim of improving human and animal health. As well as funding major
initiatives in the public understanding of science, the Wellcome Trust
is the country's leading supporter of research into the history of medicine.

©2002 Medical Research Council
Data Protection policy | Contact the MRC


Oral transmission and early lymphoid tropism of chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus )
Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3,
Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1

Department of Pathology, College of Veterinary Medicine and Biomedical
Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
Department of Veterinary Sciences, University of Wyoming, 1174 Snowy
Range Road, University of Wyoming, Laramie, WY 82070, USA 2
Colorado Division of Wildlife, Wildlife Research Center, 317 West
Prospect Road, Fort Collins, CO 80526-2097, USA3
Colorado State University Veterinary Diagnostic Laboratory, 300 West
Drake Road, Fort Collins, CO 80523-1671, USA4
Animal Disease Research Unit, Agricultural Research Service, US
Department of Agriculture, 337 Bustad Hall, Washington State University,
Pullman, WA 99164-7030, USA5

Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail

Mule deer fawns (Odocoileus hemionus) were inoculated orally with a
brain homogenate prepared from mule deer with naturally occurring
chronic wasting disease (CWD), a prion-induced transmissible spongiform
encephalopathy. Fawns were necropsied and examined for PrP res, the
abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days
post-inoculation (p.i.) using an immunohistochemistry assay modified to
enhance sensitivity. PrPres was detected in alimentary-tract-associated
lymphoid tissues (one or more of the following: retropharyngeal lymph
node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42
days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No
PrPres staining was detected in lymphoid tissue of three control fawns
receiving a control brain inoculum, nor was PrPres detectable in neural
tissue of any fawn. PrPres-specific staining was markedly enhanced by
sequential tissue treatment with formic acid, proteinase K and hydrated
autoclaving prior to immunohistochemical staining with monoclonal
antibody F89/160.1.5. These results indicate that CWD PrP res can be
detected in lymphoid tissues draining the alimentary tract within a few
weeks after oral exposure to infectious prions and may reflect the
initial pathway of CWD infection in deer. The rapid infection of deer
fawns following exposure by the most plausible natural route is
consistent with the efficient horizontal transmission of CWD in nature
and enables accelerated studies of transmission and pathogenesis in the
native species.


These results indicate that mule deer fawns develop detectable PrP res
after oral exposure to an inoculum containing CWD prions. In the
earliest post-exposure period, CWD PrPres was traced to the lymphoid
tissues draining the oral and intestinal mucosa (i.e. the
retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and
ileocaecal lymph nodes), which probably received the highest initial
exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum
and spleen in a 10-month-old naturally infected lamb by mouse bioassay.
Eight of nine sheep had infectivity in the retropharyngeal lymph node.
He concluded that the tissue distribution suggested primary infection
via the gastrointestinal tract. The tissue distribution of PrPres in the
early stages of infection in the fawns is strikingly similar to that
seen in naturally infected sheep with scrapie. These findings support
oral exposure as a natural route of CWD infection in deer and support
oral inoculation as a reasonable exposure route for experimental studies
of CWD.


now, just what is in that deer feed? _ANIMAL PROTEIN_

Date: Sat, 25 May 2002 18:41:46 -0700
From: "Terry S. Singeltary Sr."
Reply-To: BSE-L

8420-20.5% Antler Developer
For Deer and Game in the wild
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_animal protein_

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Supplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, Calcium
Lignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium Bisulfite
Complex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid,
Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate


Deer Builder Pellets is designed to be fed to deer under range
conditions or deer that require higher levels of protein. Feed to deer
during gestation, fawning, lactation, antler growth and pre-rut, all
phases which require a higher level of nutrition. Provide adequate
amounts of good quality roughage and fresh water at all times.


April 9, 2001 WARNING LETTER


Brian J. Raymond, Owner
Sandy Lake Mills
26 Mill Street
P.O. Box 117
Sandy Lake, PA 16145

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
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...

now, what about those 'deer scents' of 100% urine',
and the prion that is found in urine, why not just
pass the prion with the urine to other deer...

Mrs. Doe Pee Doe in Estrus
Model FDE1 Mrs. Doe Pee's Doe in Estrus is made from Estrus urine
collected at the peak of the rut, blended with Fresh Doe Urine for an
extremely effective buck enticer. Use pre-rut before the does come into
heat. Use during full rut when bucks are most active. Use during
post-rut when bucks are still actively looking for does. 1 oz.

100 % Cow Elk-in-Heat urine (2oz.)

Economical - mix with water in spray mist bottle

Use wind to your advantage

Product Code WP-ESB $9.95

prions in urine?


NOW, what about TSE, urine, and transmission ;

Coincident Scrapie Infection and Nephritis Lead to Urinary Prion Excretion
Harald Seeger,1* Mathias Heikenwalder,1* Nicolas Zeller,1 Jan Kranich,1 Petra Schwarz,1 Ariana Gaspert,2 Burkhardt Seifert,3 Gino Miele,1 Adriano Aguzzi1

Prion infectivity is typically restricted to the central nervous and lymphatic systems of infected hosts, but chronic inflammation can expand the distribution of prions. We tested whether chronic inflammatory kidney disorders would trigger excretion of prion infectivity into urine. Urinary proteins from scrapie-infected mice with lymphocytic nephritis induced scrapie upon inoculation into noninfected indicator mice. Prionuria was found in presymptomatic scrapie-infected and in sick mice, whereas neither prionuria nor urinary PrPSc was detectable in prion-infected wild-type or PrPC-overexpressing mice, or in nephritic mice inoculated with noninfectious brain. Thus, urine may provide a vector for horizontal prion transmission, and inflammation of excretory organs may influence prion spread.

1 Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
2 Institute of Clinical Pathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland.
3 Institute of Biostatistics, University of Zürich, Sumatrastrasse 30, CH-8006 Zürich, Switzerland.

* These authors contributed equally to this work.

To whom correspondence should be addressed. E-mail:

so in short, the statement below is as about as obtuse as the come.

>>>State officials remind hunters and others who enjoy eating venison that CWD is a wildlife management issue, not a public health issue. There is no evidence that CWD is linked to disease in humans. <<<

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

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