Prion Disease
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USDA rejects Belgian request to return Vermont sheep
APHIS: still chasing virinos after all these years
CWD to cattle, CWD to human: update
Game farmers fight Montana initiative to outlaw
Disease may wipe out elk industry
More mad cow cases in France this year than ever
Health care workers: 7% of Slovakian sporadic CJD
England worried briefly about infecting other countries
Damning BSE Inquiry report adds to Tory woe

USDA rejects Belgian request to return Vermont sheep

Reuters World Report Fri, Aug 25, 2000 
U.S. Agriculture Secretary Dan Glickman on Friday rejected a request by the Belgium agriculture minister to return the remaining Vermont sheep, which are suspected of carrying a neurological ailment similar to mad cow disease.

Glickman said in a letter to Belgium's Agriculture Minister Jaak Gabriels that returning the 350 imported East Fresian sheep "could undermine confidence in the integrity of our animal health system."

Earlier this month, Gabriels asked the USDA to return the sheep alive after four of the animals tested positive for a -- still unidentified -- form of transmissible spongiform encephalopathy or TSE. One form of TSE is scrapie, a disease fatal to sheep but no threat to people. Another form, however, is bovine spongiform encephalopathy or mad cow disease, which has been linked to a fatal human disorder.

"We must consider all of the animals to be diseased or exposed to the foreign TSE and handle their disposition here in the United States," Glickman said in the letter. The USDA, which fears that the European sheep were exposed to feed contaminated with mad cow disease, says it will take years of testing to determine what form of TSE the Vermont sheep had.

The Vermont sheep farmers, who imported breeding pairs of the dairy sheep from Belgium and the Netherlands in 1996, said they would rather send them back to Belgium than allow the USDA to purchase and slaughter them.

U.S. District Judge Garvan Murtha ruled earlier this month the USDA could carry out its plan to slaughter the sheep. The USDA has agreed with the farmers' lawyers not to acquire the sheep until after a fuller court hearing was held.

No cases of BSE have been found in the United States, but the human form of the disease has been blamed for 81 deaths in Britain since the 1980s.

BSE-suspected Vermont sheep appear destined for slaughter

August 26, 2000 United Press International
The U.S. government has refused the request of the Belgian government to take back two flocks of East Friesian sheep that appear to have a form of mad cow disease, one of the owners told United Press International Friday.

Linda Faillace, who owns one of the flocks in Vermont, said further appeals to save the sheep from slaughter under orders of the U.S. Department of Agriculture are being considered.

U.S. Secretary of Agriculture Dan Glickman cited "conclusive diagnosis" of a form of bovine spongiform encephalopathy (BSE), commonly referred to as mad cow disease, for the drastic action, Faillace said.

"We must consider all of the animals to be diseased or exposed to the foreign TSE (transmissible spongiform encephalopathy) and handle their disposition here in the United States, in accordance with the U.S.'s animal health regulatory system," Glickman informed the Belgian Embassy in Washington.

Linda and Larry Faillace of Warren, Vt., and Houghton Freeman of Stowe, Vt., who together own some 350 sheep, have been battling the United States for more than two years to spare their animals' lives. During this time the sheep have been under quarantine with no sign of any problem.

Linda Faillace said the owners had counted on a reprieve when the Belgian government agreed to accept the sheep. Now that the U.S. government has refused that request, Faillace said she would be conferring with the family's lawyer on what, if any, steps they might yet take to save their flocks. The two flocks include 50 imported from Belgium in 1996 and their 300 offspring born in Vermont.

The government has claimed four animals tested positive for a form of mad cow disease. Although government experts said they could not be certain the infection was the deadly disease or a more common sheep ailment, scrapie, they preferred to err on the side of caution.

After the government's announcement, Vermont Health Commissioner Dr. Jan Carney issued a warning against eating cheese made from the flocks, namely the Faillaces' Three Shepherds of Mad River Valley and Freeman's Northeast Kingdom Sheep Milk Cheese.

Fearful of a backlash against all the state's cheeses, the Vermont Cheese Council has requested the owners to surrender their sheep and take up the government's offer of reimbursement.

U.S. District Judge J. Garvan Murtha ruled against the owners on August 1. They had presented witnesses who argued the government's tests were scientifically flawed. Later the judge gave the owners' permission to appeal his ruling before the U.S. Court of Appeals.

Meanwhile, the owners sought help from the Belgian government, which subsequently informed the USDA of its willingness to take back the 50 sheep born in Belgium.

A recent analysis in the journal Nature extrapolated that nearly 140,000 cases of Creutzfeldt-Jakob disease, the human form of the disease, could eventually appear in Britain among those who ate infected beef. Mad cow disease first appeared in Britain in the mid-1990s, killing 81 persons and devastating the beef industry. The European Union banned imports of British beef at that time.

The USDA fears that the sheep exported to Vermont may have eaten some contaminated feed in Belgium and wants to take all precautions to keep mad cow disease out of the United States.

Opinion (John C. Stauber, author of Mad Cow USA):

"According to USDA Secretary Glickman quoted in the story above, the US refuses to return the Vermont TSE-exposed sheep to Belgium because doing so "could undermine confidence in the integrity of our animal health system." Anyone who is familiar with the information in our book Mad Cow USA will have already have zero confidence in the USDA and FDA handling of mad cow-type disea ses in the US, and this is one more reason why.

If these sheep do have BSE, that information is critical to the Europeans because no doubt other sheep would have it too throughout the UK and the EU countries. The US owes it to Europe to let them have these sheep for research purposes.

Do the Vermont sheep have BSE? In my opinion it's just as likely that these sheep have a US TSE picked up in Vermont where US sheep are infected with scrapie.

This USDA decision is further proof that the administration is simply grandstanding to protect the image of meat exports, desperately hoping no one notices the TSE epidemic spreading in deer and elk in the wild in Colorado and Wyoming through game farms, and already suspected in the deaths of at least two young hunters.

Opinion (webmaster):

There is no real evidence presented so far for any TSE in these sheep. True, an unrepeatable positive western blot was seen on unencoded samples on an overloaded gel under-treated with protease K lacking any negative control or molecular weight marker by an eminent hay mite/virino laboratory, but is a prescription for generating a false positive.

How is it possible that 13 month healthy culls have TSE when their 65 month old parents (and progenitors back in Belgium) remain healthy? How is it possible for IHCs to be negative and for histopathology to only show signs "common in adult sheep"? What we have here is not science but a decision (made years before any tests were done) to get rid of the sheep so that cattle exports to Europe might resume.

Why not send the sheep back to Belgium for further observation and testing -- because USDA fears independent testing.

USDA: still chasing virinos after all these years

APHIS fact sheet pdf on Vermont sheep
Q: What causes BSE?

A: Little is known about the actual agent that causes BSE and other TSEs. So far, scientists know that the TSE agent is smaller than most viral particles. It is highly resistant to heat, ultraviolet light, ionizing radiation, and common disinfectants that normally stop viruses or kill bacteria. Also, the agent does not cause the host's immune system to create detectable antibodies. The TSE agent has not yet been observed under a microscope.

Three main theories on the nature of the agent have been proposed:

1) An unconventional virus.

2) A prion or a partially protease-resistant protein that is rebuilt into an abnormal prion.

3) An incomplete virus (i.e., a small piece of DNA) that protects itself by using a host protein. This is called a virino."

Opinion (webmaster): Here is the heart of the problem with USDA -- clueless after all these years. Scrapie-associated fibrils were first observed under the microscope in the early 1970's. The kooky models above were ruled 18 years ago, so what public purpose is served by resurrecting them now? USDA has no business in TSE regulation if they are this ignorant of the science.

Virinos? The first of 3 speculative papers on virinos came in 1982 (note 3414 publication exist on prions), the second was from Staten Island (surprise!) and the last in 1994. Virinos never had supporting data and no more than a handful of true believers: Kimberlin-Dickinson-Hope, Dormont, Staten Island, and a Polish clinician.

Medline has the real story, which is that of a conventional amyloidosis. The mystery was all over by the time of Glenner's 1983 paper, considering this was something like the eighth known amyloid at the time, that review articles on familial CJD had been written and so on. By the time of the great flurry of sequencing articles in the summer of 1986 and infectious insulin protein demonstrated in 1988, there were no real outstanding issues about the causative agent and no great surprises either:

Molecular cloning and complete sequence of prion protein cDNA from mouse
brain infected with the scrapie agent.
Locht C, Chesebro B, Race R, Keith JM.
Proc Natl Acad Sci U S A. 1986 Sep;83(17):6372-6.

Isolation of a cDNA clone encoding the leader peptide of prion protein and
expression of the homologous gene in various tissues. Proc Natl Acad Sci U
S A 1986 Sep;83(17):6377-81
Robakis NK, Sawh PR, Wolfe GC, Rubenstein R, Carp RI, Innis MA
We have isolated a hamster cDNA clone representing the coding sequences for the entire precursor of prion protein (PrP) 27-30....Our data suggest that PrP is not a necessary component of the infectious agent.
Molecular cloning and complete sequence of prion protein cDNA from mouse
brain infected with the scrapie agent.
Proc Natl Acad Sci U S A 1986 Sep;83(17):6372-6
Locht C, Chesebro B, Race R, Keith JM

Molecular cloning of a human prion protein cDNA.
Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB,
Dearmond SJ.
DNA. 1986 Aug;5(4):315-24.

Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies.
Kitamoto T, Tateishi J, Tashima T, Takeshita I, Barry RA, DeArmond SJ, Prusiner SB.
Ann Neurol. 1986 Aug;20(2):204-8.

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene.
Cell 1986 Aug 1;46(3):417-28
Basler K, Oesch B, Scott M, Westaway D, ... Prusiner SB,  Weissmann C

Molecular cloning of a human prion protein cDNA.
DNA 1986 Aug;5(4):315-24
Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene.
Cell. 1986 Aug 1;46(3):417-28.

Human prion protein cDNA: molecular cloning, chromosomal mapping, and
biological implications.
Liao YC, Lebo RV, Clawson GA, Smuckler EA.
Science. 1986 Jul 18;233(4761):364-7.

Conservation of the cellular gene encoding the scrapie prion protein.
Nucleic Acids Res 1986 Mar 11;14(5):2035-44
Westaway D, Prusiner SB
The major protein, PrP 27-30, in purified preparations of hamster scrapie prions is encoded within the genome of the experimental host. DNA sequences related to a PrP cDNA clone can be detected in a wide variety of organisms under relatively stringent conditions...
Purification and structural studies of a major scrapie prion protein.
Cell 1984 Aug;38(1):127-34
Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE
...A single N-terminal amino acid sequence for PrP 27-30 was obtained; the sequence is N-Gly-Gln-Gly-Gly-Gly-Thr-His-Asn-Gln-Trp-Asn-Lys-Pro-Ser-Lys and it does not share homology with any known proteins. The same amino acid sequence was found when an extensively purified preparation of prions aggregated into rods and containing approximately 10(9.5) ID50 U/ml was sequenced directly. Knowledge of the amino acid sequence should permit determination of the genetic origin and replication mechanism of prions.
Molecular characteristics of the major scrapie prion protein.
Biochemistry 1984 Dec 4;23(25):5898-906
Bolton DC, McKinley MP, Prusiner SB

Purification of the scrapie agent by density gradient centrifugation.
J Gen Virol 1984 Feb;65 ( Pt 2):415-21
Marsh RF, Dees C, Castle BE, Wade WF, German TL

Scrapie prions aggregate to form amyloid-like birefringent rods.
Cell 1983 Dec;35(2 Pt 1):349-58
Prusiner SB, McKinley MP, Bowman KA, Bolton DC, Bendheim PE, Groth DF, Glenner GG
A large scale purification protocol employing zonal rotor centrifugation has been developed for scrapie prions. The extensively purified fractions derived using this protocol contained only one major protein, designated PrP, and rod-shaped particles. The rods measured 10 to 20 nm in diameter and 100 to 200 nm in length by negative staining; no other particles were consistently observed. SDS denaturation caused the rods to disappear, prion infectivity to diminish, and PrP to become sensitive to protease digestion. Arrays of prion rods ultrastructurally resembled purified amyloid and showed green birefringence by polarization microscopy after staining with Congo red dye. The rods appear to represent a polymeric form of the scrapie prion; each rod may contain as many as 1,000 PrP molecules. Our findings raise the possibility that the amyloid plaques observed in transmissible, degenerative neurological diseases might consist of prions.
Familial spongiform encephalopathies.
Res Publ Assoc Res Nerv Ment Dis 1983;60:273-91
Asher DM, Masters CL, Gajdusek DC, Gibbs CJ Jr

Identification of a protein that purifies with the scrapie prion.
Science 1982 Dec 24;218(4579):1309-11
Bolton DC, McKinley MP, Prusiner SB

Further purification and characterization of scrapie prions.
Biochemistry 1982 Dec 21;21(26):6942-50
Prusiner SB, Bolton DC, Groth DF, Bowman KA, Cochran SP, McKinley MP

Viroids and prions.
Proc Natl Acad Sci U S A 1982 Sep;79(17):5220-4
Diener TO, McKinley MP, Prusiner SB
Viroids are small "naked" infectious RNA molecules that are pathogens of higher plants. The potato spindle tuber viroid (PSTV) is composed of a covalently closed circular RNA molecule containing 359 ribonucleotides. The properties of PSTV were compared with those of the scrapie agent, which causes a degenerative neurological disease in animals. PSTV was inactivated by ribonuclease digestion, psoralen photoadduct formation, Zn2+ -catalyzed hydrolysis, and chemical modification with NH2OH. The scrapie agent resisted inactivation by these procedures, which modify nucleic acids. The scrapie agent was inactivated by proteinase K and trypsin digestion, or urea. PSTV resisted inactivation by these procedures, which modify proteins.
Novel proteinaceous infectious particles cause scrapie.
Science 1982 Apr 9;216(4542):136-44
Prusiner SB

Scrapie agent contains a hydrophobic protein.
Proc Natl Acad Sci U S A 1981 Nov;78(11):6675-9
Prusiner SB, McKinley MP, Groth DF, Bowman KA, Mock NI, Cochran SP, Masiarz FR

... Employing preparations of the scrapie agent enriched 100- to 1000-fold
with respect to protein, we found that digestion by proteinase K destroyed more 
than 99.9% of the infectivity.

Thiocyanate and hydroxyl ions inactivate the scrapie agent.
Proc Natl Acad Sci U S A 1981 Jul;78(7):4606-10
Prusiner SB, Groth DF, McKinley MP, Cochran SP, Bowman KA, Kasper KC

..Thus, it is unlikely that the agent is  composed only of a "naked"
nucleic acid. Certainly, if the agent were a naked nucleic acid, its
lability in alkali virtually eliminates the possibility that it is composed
of a single-stranded molecule of DNA.

Molecular properties, partial purification, and assay by incubation period
measurements of the hamster scrapie agent.
Biochemistry 1980 Oct 14;19(21):4883-91
Prusiner SB, Groth DF, Cochran SP, Masiarz FR, McKinley MP, Martinez HM

...These data provide evidence in support of a protein component within the
scrapie agent which is essential for maintenance of infectivity. Thus, it
is unlikely that the scrapie agent is composed only of a "naked" nucleic
acid as is the case for  the plant viroids.

Requirement of a protein component for scrapie infectivity.
Intervirology 1980;14(3-4):213-6
Cho HJ

Scrapie infectivity was not affected by RNase A treatment and was only
slightly reduced by DNase I treatment. These results indicate that a
protein component is required for the transfection of scrapie  nucleic acid
and that free scrapie nucleic acid is either not infectious or its
infectivity is extremely low.

More Mad Cow Cases In France This Year Than Ever Before - Ag Minister

 August 24, 2000 Deutsche Press-Agentur 
The number of cases of bovine spongiform encephalopathy (BSE), or "mad cow" disease, will be found in France this year than ever before, Minister of Agriculture Jean Glavany said on Thursday. Speaking on the radio station Europe 1, Glavany said that the disease will be found to be far more prevalent "because we are looking for it."

"I say this with a kind of serenity," Glavany said, "because France is at the forefront" of researching the prevalence of the epidemic. "The more one looks, the more one finds," Glavany said. "But the more one finds, the more one knows."

After Wednesday's announcement, by a local agricultural union, that another case of BSE had been detected in western France, the number of stricken animals found in the country this year has risen to 37.

"Today, we are at 37" cases for 2000, Glavany said. "And in the coming days we will no doubt reach 38, 39." In 1999, 29 cases of "mad cow disease" were detected in France.

In June, the government began a programme of testing 48,000 dead animals for BSE using a new test developed by the Swiss firm Prionics. Use of the test in Switzerland led to the discovery that BSE was far more prevalent in the country than had been believed.

Glavany also noted that, while France continues to follow the advice of scientists who advise slaughtering an entire herd if one sick animal is found in it, the government was ready to employ less drastic measures, if they were found to be scientifically feasible.

Since 1991, when BSE first broke out in France, 117 cases of the disease have been detected in the country.

France finds 3 more cases of mad cow disease

Dow Jones Mon, Aug 28, 2000
French officials have discovered three new cases of mad cow disease, the country's Agriculture Ministry said in a statement Monday. The infected livestock were detected as part of a program launched in June to screen animals considered to be at high risk for mad cow disease, or bovine spongiform encephalopathy. Until then, France only tested cows suspected of carrying the disease. [This is significant: it shows the Prionics test is working to find preclinical BSE and implies other countries should do the same. -- webmaster.]

More than 40 cases have been discovered in France this year. Last year, officials reported 31 infected animals. The infected animals' herds were slaughtered, in keeping with French regulation.

New cases of the ailment are expected to appear in France until 2002, five years after authorities first took rigorous measures to prevent more outbreaks. Europe's beef scare was triggered in 1996, when the European Union imposed a ban on British beef after a link was established between the disease and Creutzfeldt-Jacob disease, a fatal brain-wasting condition in humans.

France rules out lifting beef ban

Sun, Aug 27, 2000 By Bob Roberts, Deputy Political Editor, PA News
The Ministry of Agriculture has shrugged off reports published today that French farms minister Jean Glavany had ruled out an early lifting off the beef ban. In a French newspaper interview Mr Glavany said it was "out of the question" that the beef ban would be lifted at the moment.

In a reference to a recent report claiming that the death toll of the human form of mad cow disease could be as high as 136,000 or as low as 63, Mr Glavany told Le Journal du Dimanche: "We consider that news coming from the United Kingdom does not go in the right direction." However, a MAFF spokesman said the report showed the human consequences of BSE were not yet known.

He added: "What is important for our partners in Europe is that the trend in the incidence of BSE in cows remains firmly downwards and that the public safety provisions we have in place ensures that no material likely to be carrying infection passes into the food chain."

The European Commission has started court action against France over its refusal to lift its ban on British beef and the spokesman said the Government was not expecting France to change its position.

Comment (webmaster):

There are many, many stories about the odd case of BSE in France but none about continuing BSE in England which is at twenty times higher the French incidence. Why s a big news release when France finds a mad cow when we could be having 3-4 press releases every day for the UK?

On 11 August 2000, the cumulative UK total was 177,122 confirmed cases of BSE on 35,074 farms.

For 2000, there have been 781 UK BSE cases confirmed, or 100 cases per month or 3.3 cases per day, so 21.1 x the rate in France, though one could ask, how many BSE cases does the UK actually have, since only France is doing real random testing with the sensitive Prionics assay, something that England has never considered.

UK farms by type and BSE incidence:

Dairy farms    22104  63.0%
Suckler farms   9535  27.2
Mixed farms     2094   5.97
Not recorded    1336   3.81

Dairy cases   143501 81.03
Suckler cases  20859 11.78
Mixed cases    10467  5.91
Not recorded     2261 1.28 

Over Thirty Month Scheme (OTMS) - cull totals for week ending 23/07/00

Regions     Abattoirs     Incinerators     Total           
England     2,416,074     212,120        2,628,194           
Wales         393,382      70,457          463,839           
Scotland      575,947      33,892          609,839           
N. Ireland    628,657           0          628,657           
UK          4,014,060     316,469        4,330,529     

Change in Disease Status in Denmark Because of BSE

Federal Register: August 24, 2000 (Volume 65, Number 165) [Page 51518-51519]
 From the Federal Register Online via GPO Access [] [DOCID:fr24au00-5]
DEPARTMENT OF AGRICULTURE Animal and Plant Health Inspection Service
9 CFR Part 94 [Docket No. 00-030-2]

AGENCY: Animal and Plant Health Inspection Service, USDA.
ACTION: Final rule.
SUMMARY: We are amending the regulations by adding Denmark to the list
of regions where bovine spongiform encephalopathy exists because the
disease has been detected in a native-born animal in that region.
Denmark has been listed among the regions that present an undue risk of
introducing bovine spongiform encephalopathy into

[[Page 51519]]

the United States. Therefore, the effect of this final rule is a
continued restriction on the importation of ruminants that have been in
Denmark and meat, meat products, and certain other products of
ruminants that have been in Denmark. This final rule is necessary in
order to update Denmark's disease status regarding bovine spongiform

EFFECTIVE DATE: September 25, 2000.

Veterinarian, National Center for Import and Export, Products Program,
VS, APHIS, 4700 River Road Unit 40, Riverdale, MD 20737-1231; (301)

Health care workers: 7% of Slovakian sporadic CJD

Eur J Epidemiol 2000 Apr;16(4):353-5
Mitrova E, Belay G    [not Belay ED of CDC]
"Creutzfeldt-Jakob disease (CJD) is the most important human transmissible spongiform encephalopathy (prion disease), recognised in sporadic, genetic but also iatrogenic forms. The identification of 8 health care workers in a group of 114 definitive CJD patients in Slovakia suggested the possibility of professionaly acquired CJD and induced the investigation of potential endo- and exogenous risk factors.

In CJD-affected health professionals special attention was paid to a detailed occupational history, including a possible professional contact with CJD patient and to the findings characteristic for iatrogenic CJD: early cerebellar symptomatology, long duration of the disease, absence of typical EEG finding and homozygosity of PRNP gene at codon 129. Analysis of epidemiological, clinical and molecular biological data in investigated group of CJD-affected health professionals gave no evidence of an occupational risk for CJD."

Comment (webmaster): 7% of sporadic CJD in health professionals is way too high to occur by chance, despite the tone of the abstract. Now the first thing Dr. Mitrova would have done is rule out familial CJD of the form E200K, which has a very extensive cluster in Slovakia, though the abstract does not really say what was sequenced besides codon 129 of the prion gene. Little credence can be given to the idea that a characteristic pattern exists for iatrogenic CJD, which is a total hodge-podge after the dominant forms, growth hormone and dura mater, are subtracted.

One very real possibility in Slovakia is that the high numbers of E200K cases, prior to diagnosis, has led to a secondary epidemic in health care workers. This source would be missed by epidemiology because only exposure to clinical E200K (hospice workers) would be considered.

One clear-cut case of a health worker at occupational risk is the orthopedic surgeon who removed dura mater from sheep brain for the company in Germany selling dura mater for human transplants.

Until Germany conducts a Prionics-type survey of its sheep, we will have no real idea of the prevalence of scrapie there. The whole dura mater tragedy unfolding in Japan may have resulted from sheep dura mater being used instead of human cadaver. If so, this unfortunate 'experiment' would prove transmissibility of scrapie to humans (though who would doubt this in a scrapie dura mater brain transplant?).

Spinal dura contains elastin and collagen gene products, which would allow the species origin of the transplant to be determined even decades later (if it is not resorbed). Thus it is still possible to determine is causing the Japanese dura mater outbreak.

The other related case of a possibly affected caregiver is a husband-and-wife pair who contracted sporadic CJD within a few years of each other:

Creutzfeldt-Jakob disease in a husband and wife.

Neurology. 1998 Mar;50(3):684-8.
Brown P, Cervenakova L, McShane L, ... Piccardo P, Ghetti   B, Gajdusek DC.
A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.

CWD to cattle transmission

27 Aug 2000 adapted from private lab correspondence 
The question of CWD and its potential for transmission to cattleis being actively investigated by a number of scientists in different agencies, who take this potential seriously. There is currently no evidence that cattle are naturally susceptible to CWD (pasture transmission); however, we are looking at this potential by several different approaches.

Some cattle (3 of 13) indeed contracted CWD from mule deer when inoculated intracerebrally (in other words, injections directly into the brain). This, obviously, is not a natural mode of transmission. Almost all species are susceptible to prions (or transmissible spongiform encephalopathy agents) by intracerebral inoculation except when a species barrier exists. There is no species barrier for deer CWD to cattle by the route studied.

These kinds of studies allow researchers to compare what CWD looks like in cattle as compared to BSE -- and CWD in cattle is in fact distinguishable from UK BSE. These experimentally infected cattle allow testing of diagnostics used in cattle surveillance. These tests indeed are adequate to detect CWD in cattle.

Other studies are underway to see if cattle are susceptible by oral exposure (so far all the cattle are normal - almost 3 years post inoculation); by paddock contact (all normal after 3 years), and by surveillance of suspect and cull cattle from the areas where CWD occurs (no indication for CWD in cattle in these areas at this time). However, these studies are not yet completed and are planned to be 10 year studies.

A goat has also been infected by ic injection in a published study but only as an aside. Goat and sheep will be the very similar, being very recently diverged.

On Sept 1, the EMBO Journal will publishe evidence that CWD and scrapie both transmit to human using an in vitro proxy test that has been reliable in the past. [The title is misleading (the authors did not want to create a furor) -- full text of the article confirms that definite conversion occurs, not that much less efficiently than human by BSE. So if you have a problem with nvCJD, then you have a similar problem with cwdCJD and scrapieCJD. -- webmaster]

Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease

EMBO Journal September 1, 2000; 19 (17)
G.J.Raymond, A.Bossers, L.D.Raymond, K.I.O'Rourke, L.E.McHolland, P.K.Bryant III, M.W.Miller, E.S.Williams, M.Smits and B.Caughey 
Opinion (webmaster):

The public policy implications of this article would seem to be:

1. No more hunting for meat or trophies in Larimer County, Colorado, other affected units, and affected and trace-forward game farms.

2. Counseling, monitoring, exclusion from blood donation, and possibly tonsil testing of hunters who have eaten venison from affected regions in Colorado and Wyoming.

3. Scrapie meat is no longer acceptable in the US human food supply based on the best available science concerning in vitro transformation of human prion.

England worried briefly about infecting other countries

27 Aug 00 confidential correspondence obtained by Terry S. Singeltary Sr.
BSE11/2 020;


Richmond House, 79 Whitehall, London SWIA 2NS
Telephone 01-210 3000
From the Chief Medical Officer

Mr K C Meldrum
Chief Veterinary Officer
Ministry of Agriculture, Fisheries and Food
Government Buildings
Hook Rise South
KT6 7NG    3 January 1990

Dear Mr Meldrum


You will recall that we have previously discussed the potential risks of
BSE occurring in other countries as a result of the continuing export
from the UK of meat and bone that may be contaminated by scrapie or
possibly BSE.

I remain concerned that we are not being consistent in our attempts to
contain the risks of BSE. Having banned the feeding of meat and bone
meal to ruminamts in 1988, we should take steps to prevent these UK
products being fed to ruminants in other countries. This could be
achieved either through a ban on the export of meat and bone meal,
or at least by the proper labelling of these products to make it
absolutely clear they should not be fed to ruminants 
[or zoo animals, including rare and endangered primates -- webmaster]. 
Unless some such action is taken the difficult problems we have faced with BSE
may well occur in other countries who import UK meat and bone meal.
Surely it is short sighted for us to risk being seen in future as
having been responsible for the introduction of BSE to the food chain
in other countries.

I would be very interested to hear how you feel this gap in the present
precautionary measures to eliminate BSE should be closed. We should be
aiming at the global elimination of this new bovine disense. The export
of our meat and bone meal is a continuing risk to other countries.

Yours Sincerely
Donald Acheson

Dr Metters
Dr Pickles

BSE13/3 0083

Dr Pickles	                  
From: Dr J S Metters DCM0
 International, Prevention and Community Services
7 June 1990
 Copies to: Dr McInnes  Miss Pease  Mr Otley


1. I spoke to Mr Capstick yesterday. Among other things, he told me
that MAFF are now considering the labelling of animal foodstuffs, and
in particular what detail would be required if such labelling was made
compulsory. Apparently our freedom of action is constrained by EC
Directives [total garbage, MAFF wants to keep exporting -- webmaster], and there is also concern about the level of detail that
should be included in any foodstuff labels.

2. Mr Capstick suggested that this was not an area that DH had a
particular interest. I countered by saying that we supported the
principle of labelling of animal foodstuffs, particularly when these
were going for export.

3. I also thanked him for keeping us informed, in a way that I hope
encourage further communication of MAFF's internal deliberations.

Room 509
Richmond House
Ext. 5591 92/YdeS 90/6.7/5.1

Damning BSE Inquiry report adds to Tory woe

Sunday's Independent 27 August 2000 By Colin Brown, Political Editor
Tory leader William Hague is preparing for a fresh blow to the Conservatives' election hopes with the publication of the long-awaited report into the BSE scandal.

Lord Phillips, who chaired the two-year long investigation, is planning to deliver his findings to the Government by the end of next month, say sources close to the inquiry. And one member of Mr Hague's shadow cabinet admitted that the report would be highly critical of the previous Tory government: "We are going to have to take it on the chin. There's nothing else we can do," he said.

Conservative Party sources are calculating that the main criticism will be directed away from Mr Hague's existing front-line team. "Angela Browning is the only one who is in the shadow cabinet now and she won't take the blame," said one Conservative source. Mr Hague, who saw his hopes of a Tory revival dashed in the opinion polls last week, was Welsh Secretary at the height of the BSE controversy and a member of John Major's Cabinet.

The Phillips investigation uncovered a trail of incompetency under the last Tory administration that allowed potentially dangerous offal to get into the food chain after it had been banned. Lord Phillips is expected to highlight the buck-passing which was evident in the public hearings as ex-ministers and officials sought to shift the blame to local authorities for allowing lax controls on slaughter houses.

The line-up of top former Tory ministers who were questioned by the inquiry included former agriculture ministers, John MacGregor, William Waldegrave, John Gummer and Douglas Hogg. The handling of the inquiry has angered some former Tory ministers. "There was information on the inquiry website that was inaccurate that had to be completely rewritten. The way it was handled was appalling," said one ex-minister.

The Phillips inquiry has warned Commons officials that the report will be "the size of packing case" when it is published in October after the Commons returns from the summer break.

Mr Hague, who is certain to face renewed speculation about his leadership unless the Tories narrow Labour's lead soon, is about to launch his own ballot of the party to support key policy proposals including rejecting the euro, 5 pound per week on the state pension and cutting taxes on business. But there are fears of a low turn-out.

The Tory leader this week will call for more protection for children in care and measures to end "political correctness" in adoption, creating a national register and removing county boundaries. John Prescott, the Deputy Prime Minister, revealed Labour ministers are counting on the BSE inquiry to remind voters of the "total incompetence of Tory government".

Mr Prescott, who has been running the Government's day-to-day operations while Mr Blair has been on holiday, ridiculed the Tory summer offensive. Speaking to the Independent on Sunday, the Deputy Prime Minister said the failure of the Tories to press home their attacks and the embarrassment caused by Mr Hague's claim to drink 14 pints of beer a day as a teenager will have unnerved the Conservatives.

"Whoever organised the Tory summer campaign must have been drinking 14 pints a day. I'd be happy to pay their drinks bill. The Tory offensive was based on fear and prejudice over asylum seekers, paedophiles and now travellers but it has been a total flop," said Mr Prescott. He said: "Our people got a few jolts about how the Tories play all these fear and prejudice issues. It must be the first example of the failure of negative campaigning in Britain. "I think now our people are fired up for the election. A lot of people were nervous and apprehensive about the Tory attacks. But it was the best thing they could have done to us.

Game farmers fight Montana initiative to outlaw

 Associated Press  8/20/00
The Montana Alternative Livestock Producers have filed a lawsuit asking the district court to strip from the Nov. 7 ballot an initiative that would ban new game farms.

I-143, advocated by the Montana Wildlife Federation and other groups, seeks to indefinitely block issuance of new game farm licenses, stop expansion of operations, prohibit the transfer of existing licenses and halt captive shooting of game farm animals.

The Alternative Livestock Producers, include 92 Montana ranching families who have diversified their existing operations to include raising elk, say the initiative was improperly filed. I-143 opponents also condemned the measure as "a frontal assault on private property rights" by removing any financial benefit for a rancher to continue in this business.

Stan Frasier, a Wildlife Federation leader and secretary-treasurer of Sportsmen for I-143, responded by comparing it to cockfighting.

"People are still allowed to own chickens, but they can't do cockfighting because society determined it was unethical, inhumane," Frasier said. "This initiative does not prevent any existing livestock producers from owning elk. It merely says they can't charge people to shoot them and call it hunting."

Advanced method more sensitive in testing for CWD

July 14, 2000                                                      
Karen R. Cooper, APR 
Public Information Officer
Department of Livestock
By using a more sensitive test than was available during the Philipsburg alternative livestock facility depopulation at the end of last year, tests recently performed on tissues collected from the herd have shown an additional five elk had Chronic Wasting Disease (CWD), according to Dr. Arnold Gertonson, Montana State Veterinarian.

A total of nine of the 81 elk tested from that alternative livestock facility were positive for CWD. CWD is deadly to deer and elk and causes a slow wasting away of the animal, as the disease's name implies.

"The second generation immunohistochemical monoclonal test is more sensitive than the previous testing method available to diagnose CWD," Gertonson said. The newer version was used to test the elk recently depopulated by the Montana Department of Fish, Wildlife and Parks (FWP) at the Elk Valley Game Ranch near Hardin, a herd that had been under quarantine since June of 1998.

No cases of CWD were found in the Elk Valley herd, even though four of the 29 adult elk at the site originated from the Kesler alternative livestock facility in Philipsburg. The depopulation of the Elk Valley alternative livestock farm took place because the license had not been renewed and the alternative livestock facility was closing.

The Montana Department of Livestock conducts a surveillance program for CWD in alternative livestock. Since April of 1999, a total of 560 samples have been submitted from 41 alternative livestock farms. Montana's rules and regulations for alternative livestock facilities require that whenever any animal 16 months of age or older dies at a facility, it must immediately be tested for CWD.

"No elk other than the nine from the one facility in Philipsburg have been diagnosed with CWD," Gertonson said. "The Kesler facility in Philipsburg did not provide elk to any other Montana alternative livestock facility except the Elk Valley Game Ranch, where all elk recently tested negative for the disease; therefore it appears that the Kesler facility did not spread the disease to any other facility in Montana."

In February, the Montana State Veterinarian mandated a five-year CWD surveillance for cervidae being imported into Montana. With this order, no deer or elk can be imported into Montana without first having been under continuous CWD surveillance for five years and having lived within a captive herd where no CWD has been diagnosed for five years.

"Montana's regulations on alternative livestock facilities are the strongest in the nation, and other states are currently reviewing the regulations and considering adopting the same for their states," Gertonson said. The Montana Legislature recently enacted a moratorium on the licensing of any new alternative livestock facilities in Montana until a live animal test for CWD can be developed.

Disease may wipe out elk industry

10 Aug 2000 Calgary Herald Colette Derworiz
The discovery of Saskatchewan's sixth case of a deadly disease in elk may trigger an end to the elk industry in Alberta and the rest of Canada, says one of the country's leading experts on the disease.

Val Geist, former head of the environmental science department at the University of Calgary, said it is inevitable elk farmers will see more cases of transmissible spongiform encephalopathy (TSE), related to mad cow disease in cattle.

"It may lead to the demise of the elk ranching industry," he said in an e-mail interview from British Columbia. "And good riddance it would be, as game ranching is based on policies diametrically opposed to those that have aided conservation and returned wildlife to some abundance over the last century." He said as long as free-range elk are caught and sold, the disease will continue to appear in captured livestock.

John Knapp, director of of Alberta Agriculture's animal industry division, disagreed, saying there is only one, possibly two, cases of disease in the wild -- in Colorado. [Actually, 1-2% of the elk in NE Colorado are affected in the wild -- webmaster]

"We certainly do not and specifically prohibit any importation from that area," he said, noting the Alberta borders remain closed to importation of elk and it is illegal to capture wild elk. He said the case of the diseased animal in Saskatchewan -- leading Agriculture Canada to destroy an entire herd of 64 animals and plans to kill another 35 related elk -- will have little effect on the Alberta industry.

The province, said Knapp, has dealt with its own depopulation of diseased livestock in the past. "That has happened in cattle, in pigs, in sheep, in horses, in poultry, it happened with elk in the early nineties with tuberculosis," said Knapp. "In every case, that depopulation has raised the health standard and the industry has rebounded and reached greater heights."

Minister of Agriculture Ty Lund said it is unlikely the elk industry will be outlawed, since it is an important part of diversified agriculture in the province. The antlers and its velvet -- used to make pain remedies for arthritis and aphrodisiacs -- are a $52.5-million industry in Alberta. The herd value on Alberta farms is worth another $101 million.
"But at the same time, we are not prepared to jeopardize livestock," he said. "The health of the animals is one of the primary interests we have in the livestock industry." He said, however, more study on the disease is needed before commenting on its long term effect to the elk industry.
Producers say the destruction of the entire herd was the proper channel for the federal government to take, said Ian Thorleifson, executive director of the Canadian Venison Council, based in Edmonton. He said the disease is a threat to other elk and deer raised on game farms, but was caught before it infected other animals. "It was one source and all the animals at risk are quarantined, all the product at risk is quarantined," said Thorleifson.
Elk in Alberta are harvested primarily for their velvet and antlers, he said. "I don't think we will see any effect on the Alberta industry," Thorleifson said. "All industries, whether it is the oil industry or any other industry, have problems they have to deal with. We are no different." Elk killed to halt disease 64 animals slaughtered, 35 more will also die for study Agriculture Canada will destroy up to 100 elk in Saskatchewan in a bid to control the spread of a deadly infection related to mad cow disease.

A herd of 64 elk on a Saskatchewan game farm near the Alberta border has already been euthanized, and another 35 animals will be killed, after the provinces fourth case of chronic wasting disease in the past year.

"This disease is not only a threat to our industry, it is a threat to all other types of livestock and wildlife as well. It is in the best interest of everyone in Canada that these animals are removed," said Ian Thorleifson, executive director of the Canadian Venison Council in Edmonton.

The slaughtered animals will be tested for chronic wasting disease, a serious ailment that attacks the nervous system of mule deer, whitetail deer, and elk. Chronic wasting disease belongs to a group of diseases known as transmissible spongiform encephalopathy (TSE).

"This is a very difficult disease to deal with," said Thorleifson. "In Britain, they have been working on scrapie and (mad cow disease) for a large number of years. Chronic wasting disease is different but it belongs to the same group." He said the death of four elk in the last year raised fears the disease may be spreading through the herd.

Six animals on four different Saskatchewan farms have died as a result of the disease since 1996. Two of the elk, including the most recent this spring, were raised on the same farm north of Swift Current, Sask.

The owners of the elk will be compensated for about half the cost of the animals. Elk range in price from $8,000 for a bread cow to more than $20,000 for a good bull, said Thorleifson.

"We as and industry, have realized we have to deal with the situation," Thorleifson said. "We sat down with the CFIA (Canadian Food Inspection Agency, a division of Agriculture Canada) and discussed it. We decided the best approach to take is to depopulate all the animals as expediently as possible."

Dr. George Lutterbach, CFIA’s program manager of animal health, midwest region, said the animals will be used to learn more about the relatively unknown disease.

"We have ordered the destruction of al 64 elk on that farm to participate in a research project to determine the level of infection and to help advance the science around the transmission of the disease," he said.

He said researchers are in the process of selecting 35 animals from another farm near North Battleford to be included in the study. The animals were related to the herd that was killed. "The tissues are sent to an Ottawa laboratory," said Lutterbach.

Some of the symptoms of chronic wasting disease include: lack of co-ordination, unusual behaviour, weight loss, difficulty swallowing, and increased thirst. Signs may last for months before an animal dies. Often, he said, it is unknown if the animal is infected until it is destroyed.

"The disease is not known to exist in the wild in Canada," said Lutterbach, noting it is believed the disease is only spread from mother to offspring or between animals in a herd. There are about 26,000 elk in Saskatchewan and another 30,000 in Alberta.

Disease farm elk may harm trade

Friday, August 11, 2000 Colette Derworiz Calgary Herald
The destruction of almost 100 elk on two Saskatchewan farms as a result of a deadly disease may hurt Alberta's multimillion-dollar cross-border animal trade, says the provincial department of agriculture.

But an unblemished record among Alberta elk producers since the province implemented a monitoring program for chronic wasting disease (CSD) should ensure its continued success.

"We have very strict regulation, legislation, routine inspection programs and voluntary surveillance on top of it," said John Knapp, director of Alberta Agriculture’s animal industry division.

"The rest of the world looks at us and says, Well you guys in Alberta are running a tight ship. You've got a good program. We are interested in buying your animals because we can buy them with confidence." In the last four years, he said, the department examined as many as 1,000 animals under the surveillance program without finding a case of the disease.

Alberta's monitoring program began in 1996, the same year Saskatchewan saw its first of six animals die of the CWD. The death of four elk in the past year, including two on the same farm, led the Canadian Food Inspection Agency, a division of Agriculture Canada, to order the destruction of the entire herd of 64 animals. Another 35 related elk will be killed in the next two weeks.

The destroyed animals will be tested and researched to learn more about CWD, which affects the nervous system of elk, mule deer and whitetailed deer. It belongs to a group of diseases known as transmissible spongiform encehpalopathy, or TSE.

Dr Gerald Ollis, chief provincial veterinarian with Alberta Agriculture, said there is no evidence to suggest the disease can be transmitted to humans or other animals. [Wrong. See Sept 1, 2000 EMBO Journal -- webmaster]

"We don't eat elk brains or the spinal chord," he said. "That would be the high-risk tissue." [We heard this one before, from England. -- webmaster]

He said the surveillance program, which sees Albertans bring in the heads or carcasses of elk and deer that die on farms or roads for examination, suggests there are no problems.

"I don't think we will ever be able to say we are 100-percent positive we do not have CWD," said Ollis. "But as time goes on and the more carcasses we look at, the more confident we are that we don't have it."

Besides the six elk in Saskatchewan, there was only one other case of CWD found in a mule deer at the Metro Toronto Zoo. Both the deer and the first elk to die in Saskatchewan were imported from a game farm in South Dakota in 1989 a year before a ban was placed on transporting elk across the border.

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