Prion Disease: December 1999 news
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'Compelling' evidence that BSE causes nvCJD
Ten new nvCJD cases raise fears of cattle-cull fraud
Rush to stock shelves as beef-on-bone ban ends
More nvCJD cases found in France
EU gives France 5 days to justify UK beef ban
Delays in beef labelling
Lord Phillips: human mad cow deaths `could be tip of iceberg'
Elk game farm shut down
American trafficking in wild elk
81 CWD elk in dumsters awaiting decision
Swiss Browns: accidently bred for BSE?

Data establishes link between "mad cow" disease, human brain disorder

UCSF press release 20 Dec 99.   
"Researchers are reporting what they say is the most compelling evidence, to date, that the infectious proteins called prions that cause bovine spongiform encephalopathy (BSE), or "mad cow" disease, have infected humans, causing fatal brain degeneration.

Recent studies have suggested that the outbreak of mad cow disease in the late 1980s in Great Britain was responsible for the emergence of a new variant of Creutzfeldt-Jakob disease, a fatal brain-degenerative disease in humans also caused by prions. However, the link has been inconclusive. The current study establishes that the particular strain of prions, responsible for mad cow disease, is, in fact, the same strain that causes new variant Creutzfeldt-Jakob disease.

The finding, reported in the December 20 issue of Proceedings of National Academy of Sciences, is particularly unsettling because it undermines the comforting presumption that a "species barrier" dramatically lessened the likelihood that people exposed to "mad cow" disease through meats, cosmetics, and medicinal supplies would be infected. The species barrier refers to the relative lack of susceptibility of one species to prions derived from another species.

While Great Britain took the necessary measures in the late 1980s to limit spread of the disease, the disease is believed to incubate for at least 10 years, making it impossible to predict, the researchers said, how many people have been infected.

More than 175,000 cattle, primarily dairy cows have died of BSE during the past decade. More than 50 teenagers and young adults have died of new-variant Creutzfeldt-Jakob disease (nvCJD) since 1995. Nine new deaths from CJD were reported in the last quarter of 1998.

While the origins of BSE remain obscure, one possibility is that the cattle developed the disease by being fed meat and bone meal contaminated with prions from the sheep with the disease, scrapie. ...

The researchers conducted their study by first creating a line of transgenic mice genetically engineered to contain genes for the bovine prion protein. (Prion proteins are not, in themselves, lethal. They exist in all mammals and birds that have been examined, including humans, and become destructive only when their shape is altered, a change that occurs either through infection by an already infectious protein or through a genetic mutation.) The line of mice was known as Tg(BOPrP).

The researchers then inoculated the mice with prions from diseased cows. And approximately 250 days after being inoculated, all of the transgenic mice developed the neurologic disease.

Next, another group of mice was inoculated with prions from the diseased mice, and this group became sick after a virtually identical period of time, confirming that the transgenic mice transmit mad cow disease prions with no detectable change of strain or species-specific properties attributable to the mice, themselves.

Finally, and most important, transgenic mice inoculated with prions from human cases of new variant Creutzfeldt-Jakob disease produced the same incubation period and pattern of brain damage as had inoculation with prions from diseased cows.

To test their findings, the researchers inoculated transgenic mice with prions from sheep with scrapie, another prion disease causing neurological damage, and determined that these prions have dramatically different biological properties.

"BSE and new variant CJD produce the identical disease pattern of disease in Tg(BOPrP) mice, and those characteristics were those different than that found with inocula from other CJD cases or scrapie from sheep. These findings argue unequivocally that BSE and new variant CJD are the same strain of prion," said senior author DeArmond.

"The fact that the human new-variant CJD prions so precisely duplicate the properties of native bovine BSE prions in their behavior on transmission to the transgenic mice creates a compelling argument for a persuasive link between BSE and nvCJD," said Michael R. Scott, PhD, UCSF associate adjunct professor in the Institute for Neurodegenerative Diseases and the lead author of the study.

Given the enormity of the affected cattle population in Great Britain, a means of assessing risk to the human population is paramount, and more sensitive methods for detection of prions are urgently needed, the researchers said. The newly developed mouse model, Tg (BoPrP), should provide a sensitive test for detecting BSE prions, they said."

Comment (webmaster): This work was presented at the International Virology Congress in Sydney, Australia in August 1999. The full text of the PNAS article became available mid-morning on Tuesday 21 Dec 99.

This is a solid paper and another nail in England's coffin. It is fairly well described by the UCSF press release. In conjunction with earlier work, this paper shows beyond any reasonable doubt that the nvCJD is the same strain of TSE as BSE. A signficant species barrier between cattle and humans is wishful thinking given this paper and earlier data.

The paper is a watershed in that influential scientists at both the Neuropathology Unit and UCSF, while not throwing caution to the winds, are now convinced that BSE is the causal agent of nvCJD and that little by way of species barrier can be expected. It is no coincidence of timing that ten new nvCJD cases were announced yesterday, the first time ante-mortem cases have been added into the total (62 in 3 countries).

The paper is already being criticized in a whisper campaign as 'alarmist and fear-mongering' -- the idea being floated (by those responsible for the epidemic) is that nothing should be revealed because the public might panic. The top scientists are saying with this paper that they no longer can support a cover-up. The hour is late: it is high time to get out of denial and instead really light a burner under the current half-baked research effort. There will not be money for research when the government can say 'Oh, it is only 62 victims so we mustn't alarm beef importing nations unduly.'

The bovine prion gene was inserted in knockout mice that were then challenged by US Suffolk scrapie, BSE, and nvCJD. The final genomic sequence is not described in the paper despite all the problems with doppel in different knockout backgrounds -- the reader is sent off on a wild goose chase in mouse strain nomenclature in older papers. The scrapie genotypes are not provided either, other than Q at 171. Was either really determined?

While it is all well and good to test nvCJD and scrapie in these mice, this is testing the species barrier from human to cow, not really the issue except possibly for animals grazing in cemetaries. Species barriers are not necessarily symmetric, as the UCSF lab well knows from studies of mouse-to-hamster compared to hamster-to-mouse.

To test cow-to-human, a met 129 human prion in transgenic mice is needed. No one will do this, though we have val 129 (these became ill from cow, suggesting valine will prove no protection at codon 129) and chimeric met 129 mouse-human data.

Scale of UK human mad cow epidemic still unknown

21 Dec 99 Reuters World Report By Lyndsay Griffiths
Hundreds of thousands of British meat eaters might eventually die from the human form of mad cow disease but the scale of the epidemic will not be known for years, the government's chief medical officer said on Tuesday.

"There are still a lot of uncertainties about this disease," Professor Liam Donaldson told BBC radio. "We're not going to know for several years whether the size of the epidemic will be a small one, in other words in the hundreds, or a very large one, in the hundreds of thousands."

So far 48 Britons have died from the disease, which is always fatal, and the scare has crippled the British beef trade. Donaldson spoke after British and U.S. scientists said they had come up with very strong evidence that bovine spongiform encephalopathy (BSE, or mad cow disease) and a new form of its human cousin, Creutzfeldt-Jakob disease (CJD), are the same.

BSE swept British herds in the 1980s, killing more than 175,000 cows. Officials were unconcerned until humans started coming down with a new variant of CJD, a brain-destroying disease normally seen in only one in a million people.

BSE and the new CJD have been strongly linked, but there had been little experimental evidence that the two are the same. Now the new research has shown such a link -- mice helped bolster the case that victims' families had been pleading for years -- but the actual scale of the epidemic remains unclear.

The European Commission this month started legal proceedings against France over its continued refusal to import British beef, despite an all-clear from European Union scientists. The executive Commission is also studying Germany's position after some regional governments voiced renewed doubts about Britain's beef.

Britain says it has cleaned up its act and its beef is now safe for export. But past damage is far harder to gauge. A long-running British inquiry into the crisis ended this month with a big question mark when it concluded that it was too soon to say if the 48 victims seen so far were "the tip of an iceberg of infection" yet to spread.

Relatives of the dead are not surprised by the huge numbers being bandied around. "It's a potential time bomb waiting to go off," said Malcolm Tibbert, whose wife Margaret died of CJD. Experts say the disease has such a long incubation period in humans that an epidemic could be seen among people who ate infected British beef decades from now.

"The possibility that a large section of the population is at high risk must be seriously entertained," said the U.S. and British researchers in their report, published in the Proceedings of the National Academy of Sciences.

Donaldson, who advises the government on health issues, concurred, saying: "This latest research is as close as you can get to proof (of a link) and should convince any doubters." Donaldson said eating British beef, which has undergone stringent health checks following the scare, was now safe but there was no protecting meat eaters against old exposure.

"The exposure is a historical one. And so it's very unfortunate but we're just going to have to wait and see what the eventual size of the outcome is," he said.

Ten new nvCJD cases raise fears of cattle-cull fraud

Sunday Times ... Sunday 19 December 1999
Jonathan Leake and Jon Ungoed Thomas
[New data and age distribution for American CJD victims are also available - webmaster]
Up to 10 more people are believed to be suffering from variant CJD, the killer brain disorder thought to be caused by eating beef infected with BSE, known as "mad cow" disease. The victims are all still alive and mostly young, including at least one child , a 13-year-old girl. Variant CJD has already claimed 48 lives in Britain, but the appearance of new cases is particularly serious.

It is more than a decade since the government banned the use of parts of cows thought to present the greatest risk. These new cases imply either a long incubation period for the disease or that infected meat is still entering the food chain.

Dr Richard Knight, a clinical neurologist at the CJD surveillance unit in Edinburgh, confirmed that the unit is dealing with a further 7-10 suspected cases. "There is a long-term rise in the number of cases but the overall numbers are still too small to tell us the eventual size of the epidemic," he said.

The Sunday Times has established that meat banned because it could be infected with BSE is still being sold for human consumption . After the BSE scandal erupted, the government decided to slaughter all cattle over the age of 30 months. By the end of September last year, more than 2.5m cattle had been killed. Last week, however, an official involved with the cull claimed that it has been open to systematic fraud.

"It has not been monitored properly and not nearly enough has been done to stop dishonest practices ," said Graham Bell, who worked at the Intervention Board, a government body. He has sent a file detailing his evidence to the French authorities.

British investigators have confirmed that they are examining more than 50 cases where farmers and cattle dealers have allegedly used bogus identity documents to conceal cows' ages in order to sell them for human consumption . Last week the agriculture ministry admitted that 90,000 cattle have gone missing from its surveillance scheme. About 1,600 cows a year are still being diagnosed with BSE.

Trading standards officers at several county councils, including Gloucestershire, Shropshire and Somerset, said last week that they are involved in dozens of investigations .

"There is a hard core of people who are trying to get animals over 30 months into the human food chain ," said Nigel Durnford, an animal health inspector in Gloucestershire.

A ministry spokesman said there were stringent controls to prevent fraud. "The farming community supports this system and the enforcement of the rules is taken very seriously indeed," he said.

On Friday at the close of the official inquiry into BSE, Lord Phillips, its chairman, warned that the 48 deaths so far could be "just the tip of an iceberg". Three more people, one in Ireland and two in France, have also died from the disease. It emerged last week that a 36-year-old French woman has the disease but is still alive.

Previously, firm evidence of variant CJD could be obtained only through postmortem examinations. New tests, devised by the CJD unit in Edinburgh, now allow diagnoses to be made with some confidence while victims are alive.

The tests include tonsil biopsies and magnetic resonance imaging, which shows victims to have undergone specific changes in a part of the brain called the thalamus. Details of the tests are to be published shortly in a medical journal. Frances Hall, secretary of the Human BSE Foundation which represents families of victims, said: "Ten new cases is truly shocking."

Rush to stock shelves as beef-on-bone ban ends

Thu, Dec 16, 1999 By Karen Edwards, PA News
Butchers and supermarkets were today planning to rush the first consignments of legal beef on the bone on to their shelves in preparation for the lifting of the two-year ban at midnight. Customers will legally be able to buy oxtails, ribs and T-bone steaks tomorrow - exactly two years and a day after it was banned because of links to BSE.

Agriculture Minister Nick Brown announced the lifting of the ban on November 30 - immediately many shops, butchers and restaurants started selling it. Many more had flouted the ban for even longer - running the risk of prosecution.

Now supermarkets admit demand is far outstripping supply as customers queue up to buy the meat they have been denied for two years. Tesco broke the ban and put boned beef back on its shelves on Saturday December 4 - a spokesman predicts they will have sold 250,000 worth by New Year. Now the problem is keeping the prices down as shops struggle to order sufficient beef to meet customer demand, said a Tesco spokesman. The spokesman said: "The laws of supply and demand mean that it is difficult, but we intend to keep prices down because it is what the public wants. "We are struggling to keep up with the demand. "We want to meet it as much as is possible because this is what the British public has been waiting for, for two years."

Safeway was the first supermarket to stock beef on the bone, selling it in the Camden store on December 1 and in all its stores on December 4. A spokeswoman for the supermarket said they had replaced a pre-packed joint of boneless beef with a boned joint and saw sales increase by a third.

Asda will be receiving a special delivery of prime ribs, T-bone steaks, and select T-bone cuts at midnight tonight. Keen customers, the length-and-breadth of the country, will be able to buy boned beef at one of its 80 24-hour stores. Angela Spindler, Asda's director of meat and produce said: "Our range does not represent a `toe in the water' return to beef on the bone. "This move has been a long time coming and, like beef farmers, we are delighted to see the back of this senseless piece of legislation."

A spokesman for the National Federation of Meat Traders said they had advised all their members to obey the ban - but admitted many independent butchers had chosen to break it and stocked beef on the bone during the two years and since Mr Brown's announcement. But he said the federation welcomed the lifting of the ban in time for Christmas, saying: "It is going a big bonus for the independent butcher. Obviously most people are going to want to spend a bit more on a special joint and where better to get it then their local butchers."

The ban on eating beef on the bone was introduced by Jack Cunningham, then Minister of Agriculture, in December 1997. It has proved one of the most unpopular consumer safeguards in recent history.

Even the Government's independent Spongiform Encephalopathies Advisory Committee (Seac) said the chances of contracting BSE from beef on the bone was lower then being struck by lightning. The ban was blamed for refuelling fears abroad about the safety of British beef and is said to have cost the industry 170 million over the two years.

In November 30 1998 Seac reported that the already small risk of contracting BSE from beef on the bone had halved in the past 12 months. On February this year Mr Brown, who had expressed his personal wish to end the ban, said the decision about lifting the ban had been postponed to August.

On March 3 the Prince of Wales showed his opposition to the ban by publicly eating beef cut from the bone, declaring it "absolutely delicious". Alun Michael, then Welsh Secretary, also ate some. On November 30 this year Mr Brown said the ban would be lifted on December 17.

Agriculture Minister Mr Brown will personally celebrate the lifting of the beef-on-the-bone ban on Monday by attending a carvery at the House of Commons. The carving will be hosted by the House of Commons Catering Committee chairman Dennis Turner MP...

Second case of CJD brain disorder found in France

Thu, 16 Dec 1999 Reuters
French doctors have found a second case of the human brain disorder Creutzfeld Jacob Disease (CJD) which they suspect may be linked to beef contaminated with mad cow disease, authorities said on Thursday.

The diagnosis comes a week after France refused to lift a ban on British beef, which French scientists said could still carry mad cow disease that causes Creutzfeld Jacob Disease (CJD).

"This case concerns a person of French nationality, currently being treated and without any particular known risk factor," the government's health authority said in a statement. This was the second case in France of the new variant of CJD, a brain wasting illness, for which there are "strong presumptions of a link with bovine spongiform encephalopathy (BSE)," it added.

The illness is difficult to diagnose and is usually only confirmed with an autopsy. In this case, it was discovered through an exam of a sample taken through a [brain] biopsy, officials said. Doctors in the United Kingdom have detected 48 cases of new variant CJD and one case has been reported in Ireland.

France triggered a new diplomatic row with Britain last week when it opted to maintain a ban on British beef because of fears that it was still at risk from mad cow disease, despite an all-clear from EU scientists.

The European Commission said Tuesday it was giving France five working days to justify its embargo or the matter would be referred to the European Court of Justice.

A French correspondent writes:

"About the 2d vMCJ case, some more information appeared in the press (not necessarily through the official channels) concerning our 2d v-MCJ case:
- a French woman, 36 years old, in the largest hospital of Paris,
- neurologic symptoms began several month ago, looking like psychatric troubles,
- brain biopsy (not said which part) agreed by family, showed lesions specific to v-MCJ : florida spots ("plaques" in French),
- no specific known risk factor , ie no known surgery or treatment that might explain an iatrogenic case, and, I guess we might say she was not practicing body-building,
- although done ante-mortem, the diagnosis is considered definite because of the histological lesions,
- another case suspected of being nvCJD is said to be under investigation.

For those interested on the French position about the precautionary principle, the report ordered by Prime Minister is available online (in French). It is over 250 pages, in French."

France reports 29th case of mad cow disease in 1999

Reuters Fri 17 Dec 1999
France reported a new case of mad cow disease (BSE) on Friday, bringing the number of cattle in the country found with the illness to 29 this year. The disclosure, which came days after France reported the 27th and 28th cases of BSE for the year, follows the government's decision last week to defy the European Commission and maintain a ban on British beef because of fears that it still poses a human health threat.

News of the latest BSE case also came a day after the French government said doctors have found a second case in France of the deadly human brain disorder [new variant] Creutzfeld Jacob Disease (CJD), which is suspected to be linked to beef contaminated with BSE.

The farm ministry said in a statement the infected dairy cow was from the Cantal region in central France, and that authorities had slaughtered it and 138 other cattle in the same herd. The ministry said the latest diseased cow, born in France in September 1994, was most probably infected by consuming feed not destined for cattle.

France banned nerve tissue, ground bone and certain organs from being used in French cattle feed in 1990. The French government has said the number of fresh BSE cases should die down after 2001 as the disease's incubation period is 5 years and even tougher controls were placed on animal feed in late 1996.

France has a total cattle herd of about 21 million. The latest case brought to 78 the total number of reported cases of BSE in France since the first case was dicovered in 1991. All but one of the 29 BSE-affected cows identified this year were born in France. The exception came from Switzerland.

The European Commission earlier this week stepped up its legal battle against France over its refusal to lift the embargo on British beef, giving Paris until 21 Dec 1999 to justify its ban or face swift court action. British beef exports were originally banned in March 1996 after London admitted a possible link between BSE and CJD. The Commission lifted the ban on British beef on 1 Aug 1999, and the EU's top scientists have since judged the meat to be as safe as any in the EU (sic).

Belgium finds 10th case of mad cow disease

Dec 8, 1999 (Reuters)
Belgium said on Wednesday it had discovered a new case of mad cow disease, the third this year and the 10th in total. Agricultural Minister Jaak Gabriels said in a statement that a case of bovine spongiform encephalopathy (BSE), also known as mad cow disease, had been confirmed on Tuesday in a six-year-old cow from a herd in Bievre, in southern Belgium.

EU gives France 5 days to justify UK beef ban

Dec 14  1999 Reuters By Andrew Osborn
The European Commission on Tuesday gave France five working days to justify its ban on British beef or the matter would be referred to the European Court of Justice.

``I am very disappointed we have to go down this road,'' David Byrne, EU Food Safety Commissioner, said in a statement. ``I remain convinced that the system in place ensures the utmost protection of citizens...If France does not comply with Community law within the deadline, I will refer this matter to the ECJ.''

The Commission decision follows France's announcement last week that its embargo would remain in place because of fears that British beef was still at risk from mad cow disease despite an all-clear from EU scientists. The legal action takes the form of a ``reasoned opinion,'' a formal written warning saying France is out of step with EU law.

It is very rare that such a short time period is given to a country to reply. In most cases, countries have two months, but Byrne had indicated that his patience ran out with Paris following weeks of protracted negotiations between both sides.

Britain is resigned to resolve the bitter cross-Channel dispute through legal action, which in the EU is a notoriously lengthy process. It could be two years before the court delivers its final verdict.

However, Byrne has also said he will examine whether it is possible to get the court to issue an interim judgment saying France is in breach of EU law. If a final verdict goes against France, the Commission can ask the court to consider imposing a fine based on the damage done to British farmers. In Strasbourg, British MEPs protested against France's decision by walking out of the official ceremony marking the inauguration of the European Parliament building by French President Jacques Chirac.

``France must be marginalised within the EU until the French learn how to obey the law,'' said Edward McMillan-Scott, leader of the assembly's British Conservative group. ``As long as the French continue to break the law...there must be serious doubts over whether France is suitable to take on the EU presidency in July 2000,'' he said.

MEPs unfurled a huge banner saying: ``Let them eat British beef'' -- a reference to the famous remark by Marie-Antoinette, King Louis XVI's queen consort, who said ``Let them eat cake'' on being told the French people had no bread.

Although EU food experts decided unanimously in August that British beef is now safe, France is adamant that it is justified in keeping British beef out of its market, citing a ruling from its food agency.

The EU's top scientists said in October that British beef is as safe as any in the EU, but France, still traumatized by an AIDS-tainted blood scandal in the 1980s, and Germany are the only EU members not to have followed suit.

The Commission lifted the ban on British beef on August 1, more than three years after it was imposed at the height of the mad cow disease or BSE (bovine spongiform encephalopathy) crisis in March 1996.

The British government admitted a possible link between BSE and a deadly new variant of the human brain-wasting disorder Creutzfeldt-Jakob Disease (nvCJD), which has so far killed more than 50 people.

EU Threatens Germany Over Beef Ban

18 Dec 99 The Associated Press
The European Union Commission issued a warning to the German government to lift its ban of British beef or face legal action. ``The European Commission has learned (Friday) that the German Bundesrat has not advanced as anticipated towards the lifting of the ban on British beef. ... If it is confirmed that the procedure has been stalled, the European Commission would have to open legal proceedings against Germany,'' said a statement released late Friday. The commission is threatening to take legal action, as it has against France for not lifting its own ban on British beef.

Legal action could be launched as soon as Wednesday against Germany at the European Court of Justice in Luxembourg, EU officials said. Such a case could last several years. The commission is currently trying to seek ``urgent clarification'' on the situation from the German government. Apart from France, Germany is the only other EU country which is still not allowing the import of British beef. All member states were legally obliged to lift bans on British beef five months ago.

An EU ban against British beef was enforced in March 1996, because of human health concerns around ``mad cow'' disease. The ban came as a result of a British government study which found that there was a link between mad cow disease, or bovine spongiform encephalopathy, and a fatal brain ailment in humans. France claims that scientific evidence still points to health risks from British beef.

The German Health Ministry has previously said that it intends to lift its ban on imports of British beef by February. The Germans say that their lack to lift the ban is a result of current bureaucratic holdups in the German parliament.

EU agrees to delay in beef labelling scheme

Dec 14, 1999  Reuters
European Union farm ministers on Tuesday decided that a purely voluntary labelling scheme for beef should remain in force for another year, delaying a compulsory system until 2001.

The measure, adopted against the wishes of France, was needed to prevent compulsory labelling coming into force on January 1 under a former Commission proposal.

``The extension of the deadline has been adopted,'' a Commission spokeswoman told reporters. The Commission has argued that a one-year delay was needed because member states did not yet have the procedures in place to enforce the legislation.

French farm minister Jean Glavany told journalists he was astonished the Commission had not done more to enforce some kind of compulsory beef labelling scheme next year.

Ministers also backed a Commission proposal that if ongoing discussions by the European Parliament were not completed by the January 1 deadline, the EU executive could proceed and take the necessary steps itself to implement the legislative delay.

Under the new plan, EU member states will have to enforce limited labels on beef -- showing where the animal was slaughtered and the date -- from 2001. And from 2003 a more comprehensive system giving consumers information on the animal's entire journey from farm to table will come into effect.

Ministers also decided that the Special Committee on Agriculture, made up from national officials, should examine a Commission proposal to trim export refunds on processed goods made from sugar, cereals, rice and milk, by 4.5 percent. The SCA has to report its findings by March 2000, the ministers said in a statement.

The Commission has said it needs to make the savings because of budgetary pressures on farm spending agreed under the Agenda 2000 reforms, but member states have so far objected to the cuts.

Ministers also approved a delay for a deadline for legislation on banning SRMs (Specified Risk Material), the cattle parts deemed most at risk of mad cow disease or BSE (bovine spongiform encephalopathy).

The six month delay was necessary to prevent flawed legislation coming into effect on January 1, which would have had the effect of banning some ingredients used in cosmetics and pharmaceuticals manufacture, and risked sparking a trade battle with the United States.

EU seeks further U.S. hormone-free beef guarantees

Mon, Nov 15, 1999 Reuters North America)
The European Commission is still unhappy with controls on hormone-free beef exports from the United States and may consider imposing a formal ban on the meat later this month, EU officials said Monday. The officials, speaking on condition of anonymity, said a recent EU inspection visit to the United States had concluded that the U.S. authorities had not gone far enough in improving health controls.

"There are still problems that need to be resolved," one said, adding that EU veterinary experts would consider a report by the Commission's inspectors at a two-day meeting Nov. 23 and 24. In August the United States said it had drawn up a plan to allow it to restart hormone-free beef exports after it had itself suspended them over fears sanitary controls were too lax.

EU scientific tests released in April showed that 12 percent of U.S. exports contained hormone residues and gave Washington until Dec. 15 to put new control measures in place to ensure the meat could be certified as hormone-free. This latest hitch may complicate efforts under way to settle a larger dispute between the United States and the EU over the latter's decade-old ban on imports of hormone-treated beef.

EU officials said earlier this month that the United States had expressed interest in negotiating compensation from the EU to replace sanctions it imposed over the ban. The compensation on offer is believed to be increased market access for other U.S. products, including hormone-free beef.

In a separate statement Monday the Commission said the veterinary experts' meeting to review U.S. hormone-free beef exports later this month would also look at compensation. It said the current tariff-rate quota for U.S. exports of hormone-free beef to the EU's 15 member states was 11,500 tons a year, but noted that in 1998 actual exports only totaledbetween 6,000 and 7,000 tons.

Human `mad cow' deaths `could be tip of iceberg'

Fri, Dec 17, 1999 By Sam Greenhill, PA News
The number of people who have died from the human form of mad cow disease could be "just the tip of the iceberg", the chairman of the BSE inquiry warned today. Lord Phillips said that 48 people had died of the disease so far in the United Kingdom but the full extent of the disaster may not become clear for years. He issued his warning in his closing statement on the final day of the marathon inquiry, which has gone on for almost two years.

The inquiry's final report is likely to run to 18 volumes when it is presented to ministers by the end of next March. Lord Phillips began his closing statement by paying tribute to the families of the victims of new variant CJD -- Creutzfeldt-Jakob disease -- the human form of BSE.

Bovine Spongiform Encephalopathy, which was first diagnosed in November 1986, causes a cow's brain to degenerate and become `spongy' in appearance. There is no known cure.

Lord Phillips said today: "When we started, 24 families had seen a loved one struck down by the new variant of CJD. Today that number has doubled. No-one can say whether or not those victims are just the tip of the iceberg of an infection that is still concealed from sight. This is an unusual inquiry in that, while we are investigating events which led to a disaster, the full extent of that disaster may not be clear for many years to come."

He added: "Some of the issues that have emerged from consideration of the period with which we are concerned have not gone away. They continue to face not only the present Government but the new administrations in Scotland, Wales and Northern Ireland."

Lord Phillips paid tribute to the 560 witnesses who submitted written evidence and more than 300 who gave oral evidence, all voluntarily. He said: "There has been a high degree of willingness to help. "I believe that this is quite remarkable and it is, I am sure, attributable in part to the emotion that we all share -- one of the greatest sympathy for the families of victims of variant CJD, who have been following this inquiry so keenly."

The chairman continued: "Hardly a day goes by without BSE being referred to in the media as epitomising maladministration, usually by the use of an epithet such as `the BSE scandal'. "We believe that we have been asked to consider the adequacy of the response to BSE so that these accusations, in so far as they relate to the period with which we are concerned, can receive a fair and dispassionate consideration."

Lord Phillips thanked members of the public who had shown an interest in the inquiry -- including those who have made a million visits to the official Internet website. "Over the past two years the details of the BSE story have been laid bare in this hearing room and on our website for all who wished to follow it," he said.

After Lord Phillips's closing statement, families of victims of nvCJD said they believed the inquiry would expose the lack of communication between government ministers and officials as the BSE disaster unfolded.

Roger Tomkins, of Tonbridge in Kent, who lost his 24-year-old daughter Clare to the disease, said: "What comes over to me is the poor level of communication between government departments when something as serious as the BSE crisis was obviously on the horizon. The apathy was unacceptable."

Frances Hall, who lost her 21-year-old son Peter, said: "In our minds it was apparent all along that our loved ones were dying through some contact with bovine products."

Most of the 48 people in Britain who have died from nvCJD were represented by relatives at today's hearing. Summing up his feelings at the end of the inquiry, David Churchill, of Devizes in Wiltshire, whose son Stephen, 19, was the first confirmed victim of nvCJD in Britain, said: "There are a number of people we may wish to blame, but we have to wait for the inquiry's findings. It may find there is no one to blame as such, but find many people, or their actions, responsible."

Swiss Browns: bred for BSE?

Tue Dec 14, 1999 Medline

A new allelic variant in the bovine prion protein gene (PRNP) coding egion

Schlapfer,I., Saitbekova,N., Gaillard,C. and Dolf,G.
Animal Genetics 30 386-7 1999
Comment (webmaster): In a panel of 154 panel of cows (72 Swiss Brown, 46 Heren, 18 Simmental, 18 in various breeds), the article below finds the 7x prion repeat allele exclusively in the Swiss Brown breed. The Oklahoma State University cattle breed site states that the Brown Swiss breed is one of the oldest of the dairy breeds in the world and that importation to the US began in the nineteenth century [see below] The prion protein allele frequencies for Swiss Browns were:
5x 15.3%
6x 69.4%
7x 15.3%
At least 2 cows were homozygous for each of the alleles (Fig.2 of paper). Given that extra repeats in humans cause CJD in the heterozygote, this is potentially a very bad situation showing the dangers of livestock highly inbred for a narrow range of properties (milk/high elevation). 7x or higher repeats are never seen in wild animals. One has to wonder about what causes sporadic CJD in Switzerland.

Two extra repeats in a homozygote would be a worrisome situation in a human. In a breed of cattle (ie, a huge kindred), it strongly reinforces the liklihood of 'anticipation', a genetic situation where the 7x is a high probability springboard for even more replication slippage (more repeats to slip on). In polyglutamine amyloidoses, anticipation is so fast as to be seen within a generation and even somatically. Anticipation will be worse in bulls.

Familial BSE and familial scrapie (pt mutation) are prime candidates for the ultimate initiating events in the BSE epidemic. The sheep inbreeding establishing high risk prion alleles took place centuries ago. Highly intensive and widespread amplification through feedback loops is limited to the latter decades of this century.

CWD is probably just originated as a horizontal transfer in confined animals from sheep scrapie. While affected animals do not favor any allele, it is important to keep in mind that 90% of all male deer and elk are slaughtered by hunters, ie, a CWD allele in a surviving buck could spread very quickly and even become homozygous, rather like a bad sheep allele in a ram.

This breed has been imported to the US in large numbers, beginning in 1869. If the first bull in that group, William Tell, was 7x homozygous...

The authors note wryly that "the few genetic studies in connection with BSE in cattle were restricted to the coding region of Prnp."

"The Brown Swiss breed is one of the oldest of the dairy breeds in the world. It is descended from cattle used in the valleys and mountain slopes of Switzerland since before historic records began. It was here that certain breed characteristics became so firmly established that they are still evident to this day. The origins still farther back are somewhat doubtful. Some think that the breed goes back eventually to Oriental origins, having been introduced into Central Europe from the steppes and valleys of Western Asia. On the other hand, cattle bones found in the ruins of the Swiss Lake Dwellers are very similar to the bones of present Brown Swiss cattle. Such evidence indicates that a type of cattle apparently closely related to Brown Swiss of today existed during the Bronze Age in the area now known as Switzerland.

The Swiss nation is made up of twenty-two cantons (similar to our states). From remote time the Canton of Schwyz had the reputation of possessing the best Brown Cattle, and thus the name Schwyz cattle, or Brown Swiss as known in America, was derived.

Brown Swiss in the United States are probably the purest of all recognized breeds of dairy cattle. It is definately believed that there has been little or no infusion of foreign blood, no apparent crossing with other cattle throughout the establishment of the breed. In Switzerland so well fixed did the characteristics of the breed become, that cattle raisers from Germany, Italy and other surrounding domains made regular importations of Swiss stock to strengthen the productive quality of their herds.

Early Swiss farmers gave great attention to cattle breeding. Cattle raising received special encouragement of awards and premiums made through action of the confederacy and cantons. The aim of these awards was primarily to locate for extended use the best females and breeding bulls in the country.

Swiss cattle were grazed throughout the summer at 3,000 to 8,500 feet above sea level. The unusual physical exertions and high altitude under which generation after generation of these cattle were developed has played an important part in the selection for strength and ruggedness as found in the Brown Swiss breed today.

It is from the great cow families of these herds developed generation after generation under these conditions that selections were made for importations as foundation animals of the Brown Swiss breed in America.

The first recorded Brown Swiss cattle to be introduced into America were brought in by Henry M. Clark, of Belmont, Massachusetts, in the winter of 1869 and 1870. This first importation consisted of one bull and seven females. The bull, William Tell, is the No. 1 bull listed in the Brown Swiss Record, and the seven females Zurich, Lucerne, Gretchen, Brinlie, Lissa, Christine and Geneva are the first seven female listed therein. All eight of these animals came from the Canton of Schwyz, Switzerland. This first shipment also included Albert Tell, imported in dam Zurich No. 1, and recorded as No. 2 bull in the Brown Swiss Record; and also a female, Verona, imported in dam Brinlie No. 4, and recorded as female No. 8.

From this first importation made by Clark, there were 251 descendants recorded before the next importation of 10 head (one bull and nine females) was made in 1882 by Messrs. George W. Harris of Wethersfield, Connecticut and Nelson B. Scott of Worcester, Massachusetts.

The cattle imported by Scott and Harris in 1882 came by boat from New York to the Wethersfield wharf where they were unloaded and driven to the farm. The second importation came in March of the next year. The Connecticut River was frozen so these ten heifers were taken from the boat at Saybrook, at the mouth of the river, and shipped by train to Wethersfield, walking from the station to the farm where they were quarantined. They had had a long passage and showed the effects when they arrived. Later a quarantine station was established at Garfield, New Jersey, and imported animals spent the period of quarantine there. ...

In 1889 Enos M. Barton of Hinsdale, Illinois, imported 14 head and in 1904 he imported his second load of five bulls, and 34 females, making him the importer of 53 head. Shortly afterwards a bull and seven females were imported by C.D. McLaury and Frank Freemyer of Breakabeen, New York.

After the last importation by Barton, the United States government prohibited any further importations of cattle from Switzerland because of the spread of foot-and-mouth disease in Europe. In 1931 imports were again allowed.

... As the breed was expanding across the country and world and growing in numbers, the cow herself was also improving. At the 1898 Annual Meeting of the Association in Chicago Brown Swiss was declared a dairy breed and the wheels were turning for breed progress. The pathway of progress was not an easy one, however. Tragic events caused numerous setbacks. Many herds in the early 1900's had to be destroyed due to an outbreak of tuberculosis or foot-and-mouth disease. The E.M. Barton herd which played an important role in the breed's early history had one of the most tragic stories.

In the fall of 1914 the Barton show herd was at the National Dairy Show in Chicago. Excerpts from an article in the May 1923 Bulletin tell the story best.

"Foot and mouth disease on its sweep through the country in 1914 found a foothold at Sedgeley Farm and marked the beginning of its end. About nine o'clock on the morning of November 9, two of our Brown Swiss were noticed to be affected by the disease, at noon 35 were affected, and in 24 hours they were all showing the disease. Only those who cared for the cattle during that 60 day siege that they were in quarantine can appreciate the distress for both men and cattle. The cattle all recovered and regained their normal flow of milk, but were condemned by government officials. Only four calves had been lost during the siege, these due to complications of pneumonia.

Every effort was made to save the herd. Petitions signed by all of the neighbors were sent to the Secretary of Agriculture and offers were made to pay all salaries and expenses of officials to keep the cattle in quarantine. But the department declared that it was its policy to kill all affected animals regardless of kind.

Fate was cruel to the breed, when on January 7, 1915, 215 head of Brown Swiss, looking fine as ever, were turned out of the lots, driven across the pastures into a large hole, shot down and covered with quick lime and dirt. They were appraised by the officials at $845 per head.

The barns were disinfected by the officials and made ready for the return of the show herd, which had been away at the National Dairy Show and there held in quarantine during the siege at the farm. Upon testing a few of these were found to have contracted tuberculosis while in quarantine, and again the herd was decreased by a few, leaving only a small herd of about 50 head. Mr. Barton's health was such that he had little opportunity to rebuild the herd. He died May 13, 1916. The herd was dispersed in 1921.

The H. Ayers herd was completely wiped out by T.B. in 1920 and had to be rebuilt. A good portion of the Harris herd also had to be destroyed that same year. Records show many other herds throughout the country as having suffered the same fate. The Swiss population in the United States suffered a severe setback. The depression followed putting a strain on registration.

The 40's and 50's were golden decades for Swiss with number of breeders, number of animals and type and production on the upswing. While numbers have leveled off, production and type has shown marked improvement. Brown Swiss in the show ring are cows to be respected and there is a new Breed Champion in Milk and Fat to launch us into the new century.

In closing perhaps the prime example of where we have gone in 100 years is illustrated by comparing Brienz 168 and Century Acres Liz C 619223. Brienz 168 came to the U.S. in Scott and Harris' second importation in 1883. At the 1893 World's Fair in Chicago Brienz was the Champion Over All Breeds with a three day record of 245 pounds milk and 11.66 pounds butter. This past January Century Acres Liz C completed a record of 37,846 pounds milk and 1667 pounds fat in 365 days. The Brown Swiss breed has made considerable progress during the past 100 years.

Depopulation of Alternative Livestock Facility

  6 Dec 1999 Department of Livestock to Depopulate Alternative Livestock Facility
Montana Dept of Livestock press release
Comment (webmaster):
There are quite a few elk at this Montana game farm. It has been under quarantine for a few years because of tuberculosis; in late October, CWD was confirmed. Elk have been moved recently to another game farm in Idaho under the same owner and also sold to a game farm in Oklahoma, where 2 developed CWD. The Idaho facility is apparently still operational.

Transportation by truck and burial at a landfill are not preferred alternatives for disposal. This may simply mean burial of the elk under mounds of garbage, where they would still be accessible to scavangers and subject to rainwater elution to streams or groundwater.

The Dept of Livestock does not spell out what it means for the fence line to be decontaminated nor does it describe allowed uses for the facility in the future, which is evidently too large to be decontaminated. (Cattle? Sheep? Fresh elk?). There has also been prior discussion of killing deer in the watershed surrounding the facility.

Import restrictions of captive elk from CWD states and facilities remain very lax (shorter quarintines than shortest known onset).

Montana Dept of Livestock press release

Tomorrow, the Montana Department of Livestock will depopulate an alternative livestock facility near Philipsburg where chronic wasting disease was recently discovered, the Department announced today.

Dr. Arnold Gertonson, State Veterinarian, said elk at the facility will be euthanized, and that a team of pathologists supervised by the U.S. Department of Agriculture will take tissue samples from all of the animals for testing and research purposes.

Gertonson said the animals will be transported to the High Plains Sanitary Landfill north of Great Falls, where they will be buried.

The Department had previously announced plans to burn the dead animals, but Gertonson said that method of disposal proved to be "prohibitively expensive and complicated. Burying is a method recognized by APHIS under certain conditions in accordance with Sub Title D regulations."

Gertonson said equipment used to feed, water and care for the animals will similarly be transported for disposal at the landfill, and that the fence lines at the alternative livestock facility will be disinfected once the operation has been completed.

He reiterated that the Department of Livestock will be working with the Departments of Fish, Wildlife and Parks, and Health and Human Services, to review the state's rules and regulations governing chronic wasting disease and alternative livestock facilities. The Department's rules already are among the strictest in the nation, stricter than the rules of other states where chronic wasting disease has long been known to exist, Gertonson noted.

When chronic wasting disease was first documented to have occurred at the Philipsburg facility, Gertonson issued an emergency order to make Montana's rules for importation of alternative livestock tougher than they already were.

American trafficking in ekl

Mon, 13 Dec 1999  By JOE  KOLMAN Gazette Bozeman Bureau
CORWIN SPRINGS - At 91, Welch Brogan is a granddaddy, no matter how you cut it. But more than anything, Brogan is probably best known for being a forefather of the modern-day elk farm. Welch Brogan, seen here on his ranch at Corwin Springs where he used to raise elk, has been called the "granddaddy" of elk farmers.

By the time game farming became all the rage in the late 1970s and early '80s, Brogan had been at it for about 30 years. He estimates he sold more than 2,500 elk all over the world, sometimes fetching a top price of $5,000 each. Industry experts have said that herds at many of today's commercial elk operations can trace their lineage to Brogan's Cinnabar Game Farm.

"I probably sold more elk internationally than anyone in the United States," says Brogan, a wiry man who is always busy and whose age is betrayed only by a slight slouch. In 1946, Brogan had no specific plans for the small herd of wild elk that he bought from his neighbor to the south, Yellowstone National Park. The park was selling "surplus" elk for $20 each. Brogan, an enterprising businessman, bought 12 wapiti - although he actually got a baker's dozen because culling the extra animal out of the stock truck proved too difficult.

Brogan asked state wildlife officials what he had to do. Put up a fence, they said. "I think the way things were operated back then was a handshake, and that was about it," says Tim Feldner of the state Fish, Wildlife and Parks Department, which shares regulation game farms with the Livestock Department.

Brogan was not the first Montanan to have elk on his ranch and he got some prophetic words from Courtland Durand, a Martinsdale-area entrepreneur who counted elk as well as bison, mountain goats and antelope among his stock.

Durand, whose game farm had recently gone belly up, predicted that domesticating wild animals would not be a popular practice. "'Brogan, you're going to run into all kinds of trouble,'" Durand said.

Nevertheless, Brogan forged ahead. A math whiz who never attained a high school diploma, Brogan had been earning $12 a day as an outfitter in the early 1940s after being discharged from the Navy. He also owned the restaurant at Corwin Springs. The elk roamed 14 acres west of Highway 89.

Brogan's elk were never domesticated targets for paid trophy hunters, as is now common. He never butchered his elk on site, preferring to travel to various surplus game sales - when they were still legal - buying venison and then reselling it.

A Korean businessman who bought a few head of elk persuaded Brogan to go into the antler business. For a time, Brogan bought shed horns for $1 a pound from area collectors and sold them for $2 a pound, at times amassing tons of horns. He was leery at first about cutting antlers in velvet from his elk but embraced the practice as demand increased in the Orient, where antlers are made into folk medicines and aphrodisiacs.

But, as Durand predicted, there was trouble. In the 1950s, Brogan was fined for game violations in Wisconsin. He says there was confusion over a 60-cent stamp to transport meat.

Montana wildlife officials, Brogan says, urged him to set up a grandstand to make it easier for the public to see his elk. He never did that, but he did make a parking area and sold film and soda at the site. But then Brogan was ticketed in 1976 for operating a roadside zoo and menagerie without a permit.

In 1981, he was targeted as part of a sting operation by the National Park Service to apprehend illegal horn hunters in Yellowstone. Brogan was cleared of any wrongdoing. Inaccurate record-keeping led to a misdemeanor violation in 1988. It was also during that year that Brogan allegedly sold elk infected with tuberculosis to a Canadian game farm. Brogan's elk were quarantined and he was ordered to pay $100,000 to the business.

"If you have a game farm as long as I did, you are bound to have conflicts somewhere along the line," Brogan says. But in 1989, a battle started that would go to the state Supreme Court and eventually result in the demise of his game farm.

In February of that year, state officials charged that Brogan illegally captured 80 wild elk. As that case worked its way through the courts, Brogan was charged in 1992 with capturing three wild elk, a felony crime for which he was convicted. In 1997, the Supreme Court agreed that Brogan's license should be revoked.

To this day, Brogan says his troubles came from a shift in philosophies about game farms at the state level. Tuberculosis testing was shoddy and inaccurate, he says. And, as for the wild elk found in his pastures, Brogan charges it was part of a conspiracy among state wildlife officials to put him out of business.

"Fish and Game will say, 'Oh no, we never had anything against game farms,'" Brogan says. "Well, the hell they don't." They figured if they can get rid of me, some of the rest of them will figure it isn't worth it. At the center of the battles over wild elk on Brogan's property was Jim Kropp, the FWP warden captain in Bozeman who Brogan refers to as "that kid."

At its peak, the Cinnabar Game Farm had 400 elk on about 400 acres of rugged terrain to the east of Highway 89. Brogan's ranch backs up to the Absaroka-Beartooth Wilderness, which provides habitat for thousands of wild elk in the Greater Yellowstone area.

"It grew so big, so fast," recalls Kropp, who assumed his job in 1988. "All of a sudden ... the potential for disaster became huge." The location itself was a big worry when it came to disease control, Kropp says, adding that "wild and domestic animals could literally rub noses through the fence." Added to that was Kropp's firm belief - which was also held by many other officials - that Brogan was cooking up false records and intentionally stealing wild elk.

Ever the entrepreneur, Brogan took over operation of a reindeer processing plant in Nome, Alaska, in the mid-1970s, after it became illegal to resell wild-game meat. The plant, owned by the University of Alaska, was managed by Mike Brogan, the only child born to Brogan and his wife of 59 years.

On New Year's Day, 1995, the plant burned down. Mike Brogan reportedly died after running into the burning Quonset hut in an attempt to rescue another man, who also died in the blaze.

Brogan says the turmoil surrounding the death of his son is the main reason he ended his fight to save the game farm. The processing plant is just now nearly finished being rebuilt, and Brogan is still deeply involved in its operation. He also runs sand and gravel operations that supply material for many of the roads in the area.

He says some elk farmers have been interested in leasing his property, but for now it is the rented home of six bison. Elk farmers from around the world regularly ask him for advice.

Elk farming, Brogan says, is still a good business. But those entering the industry must realize that it is different from raising cattle or sheep. Even though the elk might eat out of hand, they are still wild animals. They must be treated as such and be given enough room to roam.

CWD: State uncertain how to dispose of game farm elk carcasses

By Duncan Adams The Montana Standard
...At this writing, the 81 dead elk stored in plastic-lined Dumpsters at the David Kesler game farm near Philipsburg are akin to carcasses without a country. State officials face a situation reminiscent of the infamous wandering garbage barge that returned forlornly to anchor in New York Bay in May 1987 after its cargo was rejected by six states and three countries.

Nobody wants the elk. Nobody wants them because they could be infected with chronic wasting disease - a malady that is fatal to deer and elk and could present a serious health risk to other species, including cattle and humans.

Chronic wasting disease, like "mad cow" disease in cattle or Creutzfeldt-Jakob disease in humans, is a transmissible spongiform encephalopathy (TSE). The TSEs produce tiny, sponge-like holes in the nerve cells of brain and upper spinal cord tissue.

During an interview Friday, Dr. Arnold Gertonson, state veterinarian, shared his frustration about finding a disposal method or site that is both politically palatable and scientifically prudent.

"In the last few days, people have been saying, 'We don't want them here, we don't want them there,'" Gertonson said. "But they're not telling me where they want the elk or how they want them handled or at what cost."

Ultimately, the barge garbage was incinerated. Gertonson and other Department of Livestock officials are considering a similar solution for the disposal of the elk after many residents of Cascade County expressed opposition to burying the animals in a landfill north of Great Falls. But incineration is expensive. Gertonson said research suggests burial can be a safe and reasonable alternative.

Livestock officials began killing the elk Dec. 7. They used a series of three injections. The first contained a muscle relaxant; the second was analgesic; and the third, containing potassium chloride, stopped the animal's heart.

"It was a very humane operation," said Tim Feldner, commercial wildlife permitting program manager for the Montana Department of Fish, Wildlife and Parks. "The animals didn't feel a thing."

But strong feelings have been stirred among Montanans in recent weeks about the state's handling of the Kesler herd, specifically, and about its response more generally to the threat of chronic wasting disease.

Stan Frasier, past president of the Montana Wildlife Federation, said wildlife advocates have warned state officials repeatedly that game farms could breed disease.

"For years we've been trying to tell the Legislature and the livestock industry that game farming is a disease threat. And they have completely ignored us," Frasier said. "They have ignored or tried to downplay this disease problem for years and now it's come back to bite them in the butt."

But Frasier and other wildlife federation officials - including John Kober, director of field operations, and Craig Sharpe, interim director - said Friday they find no satisfaction in being prophetic. The stakes are too high, Sharpe said.

"If you look at our heritage and at the importance of wildlife to Montana, if you look at the history of Montana and the role that wildlife plays here, the stakes are substantial," Sharpe said.

Kober agreed. He said if chronic wasting disease is detected in big-game populations, Montana's hunters will face potentially discomfiting decisions.

"If CWD gets into elk and deer populations, every Montanan should ask themselves the questions: 'Will I go hunting? Is the risk worth the pursuit of the sport and the sense of heritage that accompanies that pursuit?'" Kober said. "My guess is that if every hunter has to ask themselves that question, we're going to lose some hunters, we're going to lose some activists and some advocacy."

Chronic wasting disease has been detected in wild deer and elk in northeastern Colorado and southeastern Wyoming. In a September 1998 memo to Racicot, Pat Graham, director of FWP, described precautions hunters in those states have been advised to heed.

"Although there is no clear evidence that CWD affects people, Wyoming and Colorado are recommending that hunters not shoot animals that look sick," Graham wrote. "They are further suggesting that hunters wear rubber gloves when field dressing game; minimize contact with brain and spinal cord tissue in areas where CWD is endemic; don't eat brains or spinal cord tissues; and that they bone out the meat and discard the brain, spinal cord, eyes, spleen and lymph nodes to minimize exposure."

But Dr. Bruce Chesebro, a researcher with years of experience studying spongiform encephalopathies, scoffs at these precautions. At Chesebro's lab in Hamilton researchers use autoclaves and lye to sterilize their instruments, something he imagines hunters aren't likely to do with hunting knives.

Meanwhile, although no one can say for sure whether CWD spreads from game farms to wild animals or from wildlife to "alternative livestock," the Kesler experience has increased scrutiny of this unique livestock industry.

The Department of Livestock decided to "depopulate" the Kesler herd after an elk that died in October was later diagnosed with CWD. The Kesler game farm had been under quarantine since June 1998 after a [second] dead elk at an Oklahoma game farm was diagnosed with CWD. Officials believed [State records obtained by this web site show] that elk had been transported to Oklahoma from the Kesler farm. A second elk, also traced to the Kesler farm and similarly posthumously diagnosed with CWD, died in June 1999 in Oklahoma.

Elk from the Kesler farm also have been shipped elsewhere. "Some went to (a game farm in) Hardin," Gertonson said. "Some went to Oklahoma. And one animal went to Missouri and is now located in a game farm in Iowa."

The Great Falls Tribune reported Saturday that the state is considering slaughtering the Hardin herd because of its potential exposure to chronic wasting disease. Kober said the Montana Wildlife Federation is concerned about the whereabouts of other animals that might have come from the Kesler herd.

"We know this has been a closed herd since 1990. But where did Kesler sell animals prior to 1990?" Kober asked. "How many other game farms in this state have Kesler animals? We want the Department of Livestock to answer that question, and we haven't heard anything back yet."

The Kesler family has not responded to requests for interviews. On Saturday, when a reporter telephoned the Kesler home, a man who described himself as David Kesler's son said his father wasn't home and then hung up.

Graham, too, acknowledged that the stakes are high for Montana's wildlife and sportsmen when the potential threat of chronic wasting disease is considered.

"A disease like CWD is certainly more troubling than some of the other problems we've had to address simply because of the difficulty to detect it, the lack of knowledge about how it is transmitted, and all the other uncertainties about the disease," he said.

Fish, Wildlife and Parks will continue to monitor game farms, he said, and to evaluate safeguards designed to lessen the risk of disease transmission >from captive animals to wildlife.

"If we decide the safeguards aren't adequate then, in my opinion, we'll have to do what is necessary. Because I don't believe the public is going to tolerate a diminishment of their wildlife populations as a result of something like this," he said.

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