Prion Disease: Mad sheep seizure in Vermont
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Mad sheep seizure in Vermont
BSE sheep: a rush to judgement?
Mad sheep: send 'em back where they came from
The Queniborough cluster
American nvCJD involved in Queniborough cluster?
10,000 tonsils in Leicestershire to be checked
School meals linked to CJD deaths
Stop the madness: CWD threatens Wisconsin's elk and ultimately people
How CWD spreads from Colorado
Montana to ban new game farms
CWD research update

Mad sheep seizure in Vermont

USDA Release No. 0235.00 Andy Solomon (202) 720_4623
USDA to destroy three vermont sheep flocks quarantined for TSE
The U.S. Department of Agriculture today announced that it is acquiring 376 sheep from three Vermont flocks after four sheep were confirmed positive on July 10 for a transmissible spongiform encephalopathy (TSE). USDA is purchasing one flock of 21 sheep and has issued an order to seize two other flocks of 355 sheep.

The owners of the sheep will receive fair market value for their animals, which USDA will then destroy to prevent the possible contamination of other livestock. [These are a unique breed of high milk producers but 'fair market value' will be as slaughter sheep. -- webmaster]

TSE is a class of degenerative neurological diseases that are characterized by a very long incubation period and a one hundred percent mortality rate. TSE's are not known to be very contagious. [??? -- webmaster]

Two of the better known varieties of TSE are bovine spongiform encephalopathy (BSE) in cattle and scrapie in sheep. Unlike BSE, there is no evidence that scrapie poses a risk to human health. Further testing, which will take several years, is required to determine which type of TSE has infected these sheep.

The original sheep, imported from Belgium and the Netherlands in 1996, were placed under limited federal restrictions when they entered the country, as part of USDA's voluntary scrapie eradication efforts. In 1998, USDA learned that it was likely that European sheep were exposed to feed contaminated with BSE.

At USDA's request, the state of Vermont imposed a quarantine on these flocks, prohibiting their slaughter or sale for breeding purposes. Since 1996, USDA has been actively monitoring these flocks for any evidence of TSE.

Milk from these sheep was sold and used to produce cheese that also was sold. While none of the original imported sheep were slaughtered for human consumption, prior to imposition of the quarantine and detection of TSE, some offspring of these animals were slaughtered for human consumption. USDA is working with other federal agencies and the state of Vermont to determine if there are any associated human health concerns.

Opinion (webmaster): The cattle industry has long wanted to slaughter these 3 particular sheep flocks, because development of BSE in them could jeoparize cattle, cosmetic, and pharmaceutical exports to the EU. BSE has never been seen in sheep except from laboratory-induced infections, though farmed sheep remain an active area of investigation in England. While contaminated bone meal was sent to Europe, little gets fed to sheep and relatively few cases of BSE in cattle have been seen there.

Nevertheless, these Vermont sheep did pose a special risk. They are a special breed that produces some 10 times as much milk as conventional sheep breeds. The owners, who managed to import them legally during a brief window of opportunity, intended to sell them nationwide to farmers who wanted to establish their own flocks. If these sheep had a new strain of TSE, be it a novel form of scrapie or BSE itself, this would have amounted to distribution of the disease across the entire US in a matter of years, making eradication vastly more difficult.

Yet why focus on shipments of sheep? The USDA allows unregulated shipment of deer and elk from herds contaminated with chronic wasting disease. CWD has been shown to be just as efficient as BSE in converting human prions to a proxy of the infectious form seen in CJD, whereas no data is available on the efficiency of the Vermont sheep.

Mad Sheep

Fri, Jul 14, 2000 e Associated Press By WILSON RING
EAST WARREN, Vt. The Agriculture Department on Friday ordered 376 imported sheep to be destroyed after tests showed they might be infected with a sheep equivalent of mad cow disease.

Tests of four slaughtered sheep found evidence of a version of bovine spongiform encephalopathy, the scientific name for the always-fatal mad cow disease, officials said. The tests mean the animals could have had the same disease that killed 77 people and devastated the beef industry in Great Britain in 1995.

"Even the remotest possibility that it could be that, I think that we have to take all precautions," said Linda Detwiler, the department's senior staff veterinarian.

Agriculture Secretary Dan Glickman signed an order Friday to destroy two flocks of sheep in Greensboro and East Warren. The owners of a smaller flock in Lyndonville agreed to sell their 21 sheep to the government for destruction.

More tests will be done to see if the sheep had the disease or another less-serious ailment, but the results will not be available for several years, Detwiler said.

The sheep, which have been quarantined since 1998, came from an area of Belgium where mad cow disease was found. They are the only such flocks in the United States.

Officials said sheep have not gotten mad cow disease anywhere in the world, but acknowledged that scientists have been able to infect sheep with the disease in laboratories.

Americans may be exposed to nvCJD through sheep meat, milk, and cheese

Sunday Times of London 16 July 00 Jonathan Leake Science Editor
Vets have found the first evidence that sheep may have become infected with BSE. Three flocks in America were this weekend being forcibly taken from their owners to high security laboratories for slaughter and testing after tests showed clear signs of the disease.

The flocks of Friesian milking sheep were imported from Europe to Vermont within the last four years. Checks have shown that their parent flocks had eaten British-bought feed that was likely to contain material from BSE-infected cows.

Linda Detwiler, the United States Department of Agriculture vet responsible for monitoring BSE and related diseases in America said they would be taken to Plum Island near New York, America's most secure biological facility.

She said: "Four sheep were confirmed positive on July 10 for a transmissible spongiform encephalopathy (TSE) - the class of diseases that includes BSE. Specific tests for scrapie, the TSE normally found in sheep, have proved negative so there is a distinct possibility they have BSE instead." [No such test exists. Scrapie is a collection of many dozens of different strains, each with slightly different properties. Indeed, BSE transmitted to sheep is just another strain of scrapie. Some common strains could be ruled out but not all. It is unclear what Dr. Detwiler means here. -- webmaster.]

Scientists have known for some years that, under laboratory conditions, sheep can catch BSE from eating infected bovine material. Until now there has never been any clear evidence of this happening on farms. Some experts believe, however, that sheep with BSE would have been misdiagnosed as suffering from scrapie which has identical symptoms.

Four scientific tests were used on the Vermont sheep. The first looked at the brains of culled animals and found clear signs of the lesions seen in sheep with BSE-like diseases.

When tests designed specifically for scrapie proved negative the samples were sent to other scientists to see if they could find prions - the protein particles that are thought to cause and transmit TSEs.

A USDA scientist who carried out one set of tests, said several of the animals had proved positive - and her results had been independently confirmed by another scientist using a different technique. "The sheep are negative for scrapie but they definitely have some kind of prion disease. it could be a new form of scrapie - or BSE," she said.

The evidence in favour of BSE is strengthened by the animals European parent flocks having been certified completely free of scrapie. [If such certification were truly possible, scrapie could be easily eradicated. Dairy sheep are kept to far more advanced age than meat sheep and may receive intense feed supplementation. If sheep with scrapie had been grazed previously on Belgium or Vermont pastures, a contaminated environment could account for transmission. -- webmaster]

Despite the fact that infective feed is now banned, if BSE had once entered the sheep population, it could still persist. In cattle BSE appears to pass from cows to calves and in sheep scrapie is renowned for its ability to transmit between generations and across flocks, although the mechanism is not known.

Professor Peter Smith, acting chair of the Spongiform Encephalopathy Advisory Committee which advises the government on safety issues, greeted the news with concern. "If BSE is found in sheep it will be very serious indeed," he said. [Indeed, how could BSE be detected in these odd Vermont flocks but not in Belgium or the UK, with its 5 million sheep and massive BSE feed exposure? This requires a massive cover-up. -- webmaster]

Tomorrow Seac will meet to discuss Britain's first cluster of four variant CJD cases around the village of Queniborough in Leicestershire. The USDA's fear that it has found BSE in sheep will also be discussed.

Detwiler said it would take a year for further tests to confirm whether or not the sheep have BSE. If so it will raise the possibility that Americans may be at risk from nvCJD since milk from the sheep has been sold for human consumption as has a limited amount of meat.

Opinion (webmaster): For every 1 sheep in the US that has BSE, there must be 50 such sheep in Belgium and 1000 in the UK, for a decade. If these tests, run since July 10, are so quick and certain, why haven't mad sheep been seen in these countries? Detwiler is basically calling them liars.

How could the 4 tests, no matter what they were, rule out scrapie?

BSE (or CWD or CJD), once transmitted to sheep, are then from that point on, by definition, merely further strains of scrapie. So it is contradictory to say scrapie is ruled out in a case of sheep TSE.

One could list the specific strains by name that have been ruled out. However, no agreement exists on the number of distinct strains of conventional scrapie (22 is sometimes mentioned), no agreed-upon nomenclature exists, no ATCC-type tissue collection exists (rather cultivars with undocumented, passage histories through unsequenced genotypes are passed around by labs), and no agreed-upon definition of strain type.

No one seems to have genotyped these odd inbreed Belgium sheep (though the owners may have done this). All it would take is for some odd polymorphism to have been fixed by the inbreeding that established the high milk yield. This means that a novel prion protein polymorphism in this breed could very well manifest itself as a new strain of scrapie -- since a strain really amounts to a unique host-produced protein fragment with a specific induction history. Of course, this hypothetical new strain of scrapie could be as bad or worse than BSE as far as transmission goes.

What species of livestock were previously kept on both the Vermont and Belgium pastures. The Vermont sheep may simply have picked up a little-studied EU strain of conventional scrapie.

Now the USDA never gave two hoots about game farms importing and exporting mad elk or hunters eating animals known to be infected with CWD. Americans have long had the USDA's blessing to go on eating sheep with scrapie even though these are just as efficient as BSE in converting human prions with proxy tests. So why a multi-million dollar monitoring program with black helicoptors sweeping in to seize 3 small flocks of sheep?

Let's put it this way: the US doesn't export billions of dollars of meat, cosmetics, and pharmaceuticals made from elk or sheep.

USDA is a little schizophrenic about the sheep industry. On the one hand, a $100 million emergency subsidy, plus huge new bailouts of the wool and mohair lobby. On the other hand, the USDA is destroying the sheep industry with disasterous worldwide publicity saying mad sheep reached the US in 1996 and you may have already eaten one (or had the cheese or milk).

Where is the 35-member SWAT team that was lined up to minimize the damage from the first announced case of BSE in US cattle? It all boils down to sheep and cattle are both to receive ag subsidies but when there is a conflict, cattle rule. Indeed, the USDA has equated the welfare of the cattle industry with that of the nation:

''The high stakes involved mandate very conservative measures if there is a possibility of the sheep being infected with the BSE agent,'' Alfonso Torres, deputy USDA administrator, told the Faillaces [the Vermont flock owners] in a letter this spring.

''This is a case in which the welfare of our nation must be placed above any other consideration.''

Reading over again the mad sheep newspaper reports and press releases, some things are worth reflecting upon:

1. USDA says in its press release that it learned in 1998 that "European sheep were exposed to feed contaminated with BSE." Passing over the first obvious falsehood, note that they leave the impression that the parental sheep flock in Belgium to the Vermont flock was fed contaminated feed. They didn't say this directly because, as the webmaster heard it, the records show that the Belgium flock was in fact strictly pastured and never fed any kind of feed concentrate, much less English MBM.

2. USDA says the 4 sheep were confirmed positive for TSE. Were these sheep displaying clinical symptoms or simply sheep slaughtered for meat or sheep that died of other reasons? Few studies are done on strain-typing asymptomatic sheep early on in disease progression. Maybe these sheep would have had more typical scrapie symptoms had they lived longer.

USDA has been gunning for these sheep for a long time. They became an obsession, obstacles to getting a better beef import rating from the EU.

Looking again at the 4 tests, the first says histopathology found spongiform lesions and possibly gliosis. Inconclusive. The second, immunohistochemistry for Prp-res, was negative. Not that unusual, even in TSE, but can't seize the sheep. So, three, they took it to Schmeer and ran her unvalidated test with its secret methodology, which was positive. Sheep seizure wouldn't stand up in court. Then, four, they took it next door to Miller, who may have found Prp-res by a blot test.

The scrapie strain they are familiar with doesn't usually give contradictory tests results ("negative for scrapie") but perhaps it was a TSE, indeed perhaps BSE. There are millions of sheep out there in flocks meeting these criteria that aren't being seized.

Conveniently, "further testing, which will take several years, is required to determine which type of TSE has infected these sheep." This means attempting to passage isolates in experimental animals. Isolates commonly fail to passage. USDA is left with a debatable TSE diagnosis, and the BSE call looks like total nonsense.

It doesn't matter: they issue themselves an order to forcibly seize and incinerate the sheep and have a fair shot of winning a court battle. If they're wrong, so what, the owners received fair market value [see below]. Beef-based cosmetics and pharmaceutical exports can resume from a country still riddled with TSEs but one that has taken draconian and highly visible steps to avoid even far-fetched BSE. Mission accomplished.

3. "The owners will receive fair market value." Had the owners obtained permission to distribute the breedstock, their value would be astronomical since the import window has closed and the sheep are unique. Without this permission, they are just assessed as sheep that produce a small amount of milk relative to a cow and aren't worth much at slaughter -- what are they worth now as USDA-certified BSE mutton?

What happens to the farm -- do they bring in new sheep, turn it over to dairy cattle, or make a deer park out of it? How much idle time will be necessary? Will there be chemical decontamination as in Colorado and Wyoming CWD paddocks? What has become of other domestic and wild animals that have been on the farm?

5. "Offspring of these sheep were slaughtered for human consumption." Was this done at a slaughter-house and what else did it process 1996-2000? Will the slaughterhouse now be specially cleaned or not? How were the offal disposed of -- landfill, rendered, or composted on the land?

The Queniborough cluster

A large collection of Queniborough updates is here
Comment (webmaster): Queniborough (rhymes with 'when it burrow') is a village of 2297 people 6 miles northwest of Leicester (pronounced Less-toh), population 330,000, the 9th largest city in Britain in the centre of England, 100 miles directly north of London. The village extends along the line of a stream running from Leicester towards Melton Mowbray. A detailed street map is available online.

Ironically, the Surveillance Unit just got done telling us in their Annual Report that there were no clusters and no caseload acceleration; obviously they knew of all 5 cases below before publication. Technically it was the 1999 annual report but the associated press release was irresponsible.

To sumarize the various stories below, there are now 5 nvCJD cases in a small region of Leicestershire, 3 of these associated with the village of Queniborough itself:

-- Glen Day, age 35, died late Oct 1998, resident
-- Stacey Robinson, mother of one, age 19, died early Oct 1998, former resident, moved to Thurmaston
-- Pamela Beyless, aged 24, onset August 1996, died August 1998, frequent visitor from nearby Glenfield
-- Martyn Wormleighton, age 19, died May 2000 in Leicester hospital, Wigston four miles from Queniborough [News of the World 6.8.00]
-- unnamed man, age 24, diagnosed by May 2000 in Leicestershire, doing poorly, village 2 miles away from Queniborough.

James Meikle, reporter for the Guardian, reports 25 new cases in the last 12 months including 7 since June 1. Seven more people are also probable nvCJD based on either brain scans or tonsil tests. 12 deaths have occurred in 2000 (3 of these are awaiting autopsy.)

It must have been the case diagnosed in May that pushed them over the edge statistically. In a sense the Dept of Health had painted itself into a corner by statistical methods used to pooh=pooh the previous cluster in Kent, England. The very same logic now shows that Queniborough must be a valid cluster. There is no word on the children of Stacey Robinson. The baby with apparent nvCJD is apparently a separate West Midlands case.

If something is learned from this cluster, that would represent a great step forward. Beyond the special met/met genotype at codon 129, possessed by 40% of the people in England, there is no understanding of who gets nvCJD and why. Occupation and medical procedures have already been ruled out for the Queniborough cluster. The likliest explanation at this point is common exposure of village residents to an exceptionally infectious BSE cow slaughtered locally.

Both Queniborough and Kent clusters were in villages in agricultural areas. Locally-made sausages are a village butcher speciality, often made from cattle slaughtered for "farmers own use", but by custom sold on to avoid taxes, bypassing the slaughterhouses and their controls. Such sausages often include minced offal. The higher incidence of nvCJD in the North is sometimes attributed to cultural preferences for offal products such as black pudding, haggis, cheap sausages, hamburgers, and so on The North generally has a higher incidence of heart disease and cancer, with accompanying shorter life spans, associated with poor diet and smoking.

England has properly mobilized its medical investigative services to find out what has happened in Leicestershire. While this may never be known, something good might yet come out of it, possibly an estimate of special risk factors and from them an estimate of the scope of the epidemic, or at least an explanation for why some people have succumbed.

One thing is for sure. Life will never be the same for the little village of Queniborough -- it has gone from zero internet presence to a household word in the space of a few days.

Village's deaths are focal point in `human BSE' probe

PA News Thu, Jul 13, 2000  By Damien Brook, PA News
A village has become the centre of a major investigation into the deadly human form of mad cow disease, the Government revealed tonight. Junior health minister Lord Hunt of Kings Heath confirmed there had been four definite cases and one probable case in Leicestershire of new variant CJD, believed to be the human form of BSE (bovine spongiform encephalopathy).

And three of the cases appeared to have links to the village of Queniborough just north of Leicester, said local health authority officials. Now the research in the village, which is a key point in the top level probe into the CJD "cluster", will help determine if the people in the area are more susceptible to the disease.

Lord Hunt said in a Lords written answer that the four confirmed cases in Leicestershire compared with the 75 known or probable cases throughout the UK. He said statistical experts had advised that it was unlikely the higher number of cases in the county would have happened by chance.

In May, when a 19-year-old man died at the Leicester Royal Infirmary, health officials said it was "highly probable" that a 24-year-old man in the county also had the bug.

So far, it has been revealed that victims Stacey Robinson, of Thurmaston, formerly lived in Queniborugh; Pamela Beyless, of Glenfield, was a frequent visitor to the village; and a third victim, who has not been named, lived their too. All three died in 1998.

Lord Hunt said a locally based investigation into the circumstances of the apparent cluster includes a large team of officials from bodies such as the National CJD Surveillance Unit and the Communicable Diseases Surveillance Centre of the Public Health Laboratory Service. They will work closely with the Department of Health, the local health authority, the Ministry of Agriculture and the London School of Hygiene and Tropical Medicine.

Health officials in the county have so far been operating a wait and see policy on how the disease will develop. They have discovered two factors which play a part in contracting the bug -- genetic susceptibility and exposure to the infection.

Dr Philip Monk, a consultant in communicable diseases at Leicestershire Health Authority, said recently: "We don't know if genetic susceptibility is evenly spread throughout the county or unevenly spread. "If we assume it is evenly spread it is highly probably we will have 5% of a big number of deaths nationwide. "But if it is not evenly spread that figure could be worse or better." Support groups for CJD victims and their families welcomed the investigation into the Leicestershire "clusters".

CJD Support Network chairman Clive Evers said he thought the cluster was "clearly significant". "It certainly merits further investigation and if something is found it might minimise the risk for other people in the future. "It may be that the investigation yields some other possible links of causal effect. "We need more knowledge about CJD to see if there is some environmental effect, particular to the area, which might switch the disease on in some people."

Frances Hall, secretary of the Human BSE Foundation, also welcomed the probe, saying: "These cases obviously need investigation. "Sadly, it comes too late for the people who have already died." Tonight a former neighbour and close friend of victim Stacey Robinson, Baz Lockwood, 45, a computer technician, spoke of his "worries" about the apparent outbreak of the disease in the village.

Mr Lockwood said: "It was so sad with Stacey. I watched her grow up for 15 years, from when she was just a little kid to when she moved away to Thurmaston.

"I didn't see her deteriorate after she got the disease because she had moved by then. "It is worrying to know we have more cases in the village than elsewhere, but as to why, I have my own ideas -- I think it's down to the insecticides." He added: "People are talking about the news, of course they are, but no one has been to see us to set our minds at rest -- where are the answers?"

Staff at the Britannia Inn, centre of the small community, said people had been coming to the pub and talking about the outbreak and about how many cases appeared to be linked to the village of just 2,297 souls. One, who asked not to be named, said: "We still serve beef on a Sunday and our sales haven't been affected at all."

Villager John Shelley said: "This is a worrying thing, and it is surprising that this link is to our own village. It's not a good thing for Queniborough.

"They have got to get on top of it, and from a research point of view it's probably good that they can concentrate on such a small area. It's not like they found three people with a link to London. "Then again, experts seems to know more and more about less and less."

CJD `cluster' is not the first

Thu, Jul 13, 2000 By David Barrett, PA News
The Leicestershire CJD "cluster" was first reported in November 1998, more than a year after a similar cluster was identified in Kent. Seven people have died of the old-type Creutzfeldt-Jakob Disease and the so-called "variant CJD" (vCJD) in Kent, near where a vet discovered Britain's first BSE herd in 1985.

Victims included 24-year-old Clare Tomkins, of East Peckham, who had been a vegetarian for 13 years, suggesting one of the disease's longest periods of incubation. In Leicestershire the disease claimed three young lives within 12 weeks in 1998. Glen Day, 35, from Queniborough, north of Leicester, died at the end of October, Pamela Beyless, 24, died in nearby Glenfield three weeks earlier and 19-year-old mother Stacey Robinson, from neighbouring Thurmaston, lost her battle against the disease the previous August.

Statistics released 10 days ago by the Department of Health show there have now been 74 victims of vCJD, including four new cases in June. There are additionally two confirmed deaths in France and one in Ireland. Reports from the Edinburgh-based CJD Surveillance Unit have suggested the rate of incidence of the disease, which causes decay in the brain and which is always fatal, varies across Britain.

Northerners could be at several times more risk from vCJD than those living elsewhere, it was suggested. In the north of England, there were 3.14 cases per million people over the five years to 1999, while Scotland had the second highest rate, of 2.98 cases per million. East Anglia's figure was 0.93, the South's 1.37 and the West Midlands' 0.36 per million.

In another study, published in April, tissue samples from more than 3,000 people seemed to suggest the risk of a CJD epidemic was receding. The anonymous tests of 3,170 tonsils and appendixes from hospitals across the country showed no evidence of variant CJD, a result which the Chief Medical Officer Professor Liam Donaldson described as "welcome news". But he added: "These early results should not be taken as an indication of an `all clear'."

Some experts have suggested the sample was too small to offer a satisfactory picture of the disease's prevalence, and said a major epidemic could still not be ruled out. Scientists behind the research are now studying a further 15,000 samples in a bid to build a more wide-ranging picture.

A few weeks after this report, a leading member of the RSPCA became the latest person to die of the disease. Baroness Wharton, vice-president of the animal rights charity, was 66, and died of the "sporadic" form of CJD.

Scientists are far from fully understanding the disease, but research published in the scientific journal Nature in 1997 was said to prove that BSE can affect humans. The study showed there is no theoretical molecular barrier to infection of people by BSE, but cases will remain extremely low because of barriers between the species.

BSE, scrapie -- the spongiform disease in sheep -- and the variant form of CJD are thought to be caused by an abnormal form of a brain protein called a prion. The researchers showed prion proteins from animal brains infected by BSE or scrapie can convert human prion protein to the abnormal insoluble form linked to disease. But the likelihood of this reaction between BSE infected tissue and human prions is very low, as the differences between prions in cows and humans affects the efficiency of transmission between the two.

Sharp rise in deaths raises alarm over human BSE

James Meikle Guardian ... Thursday 13 July 2000
A sharp rise in the death toll from the human form of BSE is being investigated by the government's scientific advisers with 67 people in Britain now thought to have been poisoned by infected beef. The figure is 25 more than a year ago and seven up on those for the beginning of June although comparisons are difficult because of changes in the way the inevitably fatal condition is diagnosed and confirmed.

Seven more people are also believed to be suffering the condition following either brain scans or tonsil tests, statistics that will at the very least ensure there is no political complacency that either BSE or its human equivalent, vCJD, is under control . The advisers will consider the latest figures at a meeting on Monday but it will be another two weeks before they announce whether there is cause for further public concern .

Also next week the food standards agency continues its review on whether it would be safe to reduce some anti-BSE controls introduced over the past 12 years, but widely flouted . Confidence was recently dented by the announcement that a cow born after extra controls were introduced on Europe's orders in March 1996 had contracted BSE, which had in the past few years only been killing older dairy cows that had not been culled as they were not destined for food.

This autumn, Lord Phillips, head of the BSE inquiry, will also deliver his verdict on the Conservative government's handling of the crisis up to 1996.

The Department of Health said yesterday the latest nvCJD figure was "higher than we have seen of late , but we should be cautious about drawing too many conclusions from one set of monthly statistics. It is the underlying trend that is more important and the spongiform encephalopathy advisory committee will, as usual, be studying the latest data at their meeting this month. It is too early to predict the ultimate size of the nvCJD epidemic."

Since April, the CJD surveillance unit has been adding figures of people still alive, but thought to have vCJD, to the monthly figures and there have been other changes to its monitoring. These have included increasing the recorded death toll for 1998 and 1999. The monthly figures often do not reflect when people died but when cause of death was established.

Even estimating the pattern of the disease from the date friends, relatives and GPs first identified symptoms to when people might die is difficult because so far this has varied from seven to 38 months, with an average of 14 months. In addition scientists do not know how long victims might have had the condition before it became apparent through depression and mood swings at first to problems with balance and other coordination later.

Three people are thought to have died in 1995 , 10 in 1996 , the year the probable human link to beef was first accepted by ministers, 10 in 1997 , 18 in 1998 and 14 last year. A dozen more people are thought to have died in the first six months of this year, although in three cases, postmortem confirmation is still awaited.

Inquiry to look at 'cluster' of CJD cases

Independent ... Thursday 13 July 2000 By Severin Carrell
An official investigation has been launched into a "cluster " of cases of the fatal brain disease CJD in Leicestershire , which experts believe are probably linked. Lord Hunt of Kings Heath, a Health minister, disclosed last night that four confirmed cases of Creutzfeldt-Jakob disease and one probable case had been detected in the area last year . Three of the victims died from the disease.

In a written answer, Lord Hunt said 75 confirmed and probable cases of CJD - the human form of "mad cow disease" or bovine spongiform encephalopathy (BSE) - had been recorded in the UK. "Statistical experts advise it is unlikely that the higher number of cases in Leicestershire will have occurred by chance ," he said.

His comments cast doubt on claims by health officials last year that the three deaths in a small area covering three villages north of Leicester were a "coincidence " and no cause for alarm .

Lord Hunt stressed the five cases were linked to infected meat eaten "many years ago ".But many specialists believe the full impact of the disease has yet to be felt, and have predicted that thousands of people could eventually die from it. Lord Hunt said controls introduced "progressively" since 1989 had made it highly unlikely that more people would contract the disease.

But he added that the Food Standards Agency, which has agreed to assist with the inquiry, would "wish to be satisfied that no new factor is involved in these cases which requires further action to ensure the safety of food". The Government would "continue to take whatever steps the experts recommend", he said.

The investigation involves officials from the National CJD Surveillance Unit and the Communicable Diseases Surveillance Centre of the Public Health Laboratory Service. They will work with the Department of Health, the local health authority in Leicestershire, the Ministry of Agriculture, Fisheries and Food and the London School of Hygiene and Tropical Medicine.

Worried villagers in alert over CJD cluster

Telegraph ... Thursday 13 July 2000 By Andrew Hibberd
A Village has become the centre of a major investigation into new variant Creutzfeldt-Jakob disease. Lord Hunt, the junior health minister, said last night that there had been four definite cases and one probable case of nvCJD, the human form of mad cow disease, in Leicestershire. Three deaths in 12 weeks appeared to be linked to Queniborough , which has a population of less than 2,500, health authority officials said.

Research in the village, which extends along the line of a brook from the main Leicester to Melton Mowbray road, will help to determine whether people in the area are more susceptible to the disease than elsewhere in the country. John Shelley, one of the villagers, said: "This is a worrying thing. They have got to get on top of it."

Lord Hunt said in a Lords written answer that the cluster of four confirmed cases linked to Leicestershire compared disfavourably with the 75 known or probable cases throughout the country. Statistical experts had said it was unlikely that this high proportion could have happened by chance .

The Food Standards Agency "stands ready to assist" with the inquiry, Lord Hunt said, and would "in particular wish to be satisfied that no new factor is involved in these cases which requires further action to ensure the safety of food".

The investigation into the circumstances of "this apparent cluster " includes a large team of officials from bodies such as the national CJD surveillance unit and the communicable diseases surveillance centre of the Public Health Laboratory Service. The officials will work closely with the Department of Health, the local health authority, the Ministry of Agriculture and the London School of Hygiene and Tropical Medicine.

Lord Hunt stressed that the cases involved would have been exposed to the infective agent "many years ago". Control measures to protect public health had been in place since 1989 and "progressively strengthened". The Government would "continue to take whatever steps the experts recommend".

In May, when a 19-year-old man died from nvCJD at the Leicester Royal Infirmary, health officials said it was "highly probable " that a 24-year-old man in the county also had the bug . The three victims with Queniborough connections died in 1998.

Stacey Robinson , a 19-year-old mother who had recently moved to neighbouring Thurmaston, died that August; Pamela Beyless , 24, died in nearby Glenfield early in October; and Glen Day , 35, from Queniborough, died three weeks later. A former neighbour of Miss Robinson, Baz Lockwood, 45, a computer technician, said: "No one has been to see us to set our minds at rest. Where are the answers?"

At the Britannia Inn, a member of staff who did not want to be named said: "Some people are a bit frightened; others are just shrugging it off. I expected our Sunday lunch sales to be down, but we sold out of beef."

In February 1996 a "cluster" was identified in Kent when two deaths were reported in the south-west of the county, although not with the same links as those at Queniborough.

10,000 tonsils in Leicestershire to be checked

London Times 14 July 00 By Valerie Elliott, Countryside Editor
Online postcards of Queniborough (pronouced kwen ih burra)
The Department of Health has ordered urgent tests on more than 10,000 tonsils and appendices removed in Leicestershire to assess the scale of possible infection of the human form of "mad cow" disease within the county.

The checks are to be anonymous but are to include every operation since 1985 and any new patients. This week's decision was made after experts said the pattern of five cases of variant Creutzfeldt-Jakob disease (vCJD), the fatal brain illness, around the village of Queniborough, north of Leicester, could not have happened by chance.

Whitehall officials have asked Dr Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, to prepare a testing plan. Last night Dr Monk confirmed to The Times that the Government had asked him to submit a testing proposal but he emphasised "it will be completely blind".

He believes the cases in the village are linked to the eating of beef infected by bovine spongiform encephalopathy (BSE). He has already spoken to the parents and relatives in four of the five cases and has built up a clear picture of their eating habits and where they bought their food.

He said he did not believe the cases are linked to an abattoir but did not wish to develop any possible theories until he had all the relevant information. His initial investigation is to be discussed shortly by experts from the CJD Surveillance Unit in Edinburgh and the Communicable Diseases Surveillance Centre of the Public Health Laboratory Service.

Once a possible cause has been established a new control area involving more people in the community is likely to be set up for further testing. Yesterday all local schoolchildren were handed questionnaires to find out where households bought their meat, and over what period, as part of the government checks.

Tests will be made on tonsil and appendix operations performed at the Leicester Royal Infirmary, its sister site, the Glenfield General Hospital, and also at Leicester General Hospital. A target of 18,000 tonsils and appendices are to be analysed. So far a test of 3,000 has failed to find a positive case. [reporter in confusing new with previous tonsil test series -- webmaster]

Stephen Dorrell, the former Tory Health Secretary who announced in March 1996 the link between eating BSE-contaminated beef and CJD, who is the MP for the area, said last night: "It is important that we don't jump to conclusions. I think the Department of Health are conducting this inquiry in exactly the right way."

Leicestershire has an average number of BSE cases in cattle. Since November 1986 there have been 3,649 cases on 547 farms.

CJD probe centres on abattoirs

Sat, Jul 15, 2000 By PA News Reporters
An investigation into a cluster of CJD cases centred on a country village is now focusing on local abattoirs and their slaughtering practices, an expert said today. Scientists investigating three deaths from variant CJD of people who had links with Queniborough, Leicestershire, are working to pinpoint the source of infected meat the victims may have eaten 20 years ago.

Dr Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, said: "We have been looking to see what patterns of slaughtering took place in this area of Leicestershire and where meat from these slaughterers entered the food chain. "We have to look at all the slaughterers in this area, which historically is rich in small abattoirs.

"There are seven independent slaughterers plus three big slaughterers in the city of Leicester, which is only five miles away, so there is a lot of teasing out to do there," he told the BBC Radio 4 Today programme.

Professor Peter Smith, acting head of the Government's Spongiform Encephalopathy Advisory Committee, said that it might never be known whether the Queniborough cases were linked. "The cluster that has been observed in Leicestershire is unlikely to be due to chance, but at this stage we can't be sure it isn't due to chance," he told the Today programme. "There is little evidence that other cases of clustering in this way were linked. "Clearly, abattoirs are one of the areas which have to be investigated."

It was too early to say whether Britain was heading for a large-scale epidemic of CJD, Professor Smith said. "There has been somewhat of a rise over the last year, but whether that is temporary or the onset of something larger is unclear," he went on. "Because we have no test for who is infected with this agent, it is very difficult to predict what the evolution of the epidemic is going to be."

Investigators from the CJD Surveillance Unit, Leicestershire Health Authority, the Communicable Diseases Surveillance Centre and the Department of Health are also examining the victims' genetic susceptibility to variant CJD[meaning that the two new cases are being genotyped for met/met at codon 129 of the prion protein -- webmaster].

CJDSU director Professor Robert Will said other possible links, such as where the people lived and worked, and their medical histories, had already been ruled out by investigators. There have been 75 cases throughout the UK since the first cases of the disease came to light in 1996. "What we are seeing in Queniborough could just be a statistical blip, but it would be wrong for us to assume that -- we must investigate it fully," Professor Will added.

Investigations have so far shown that mother-of-one Stacey Robinson, 19, of Thurmaston, formerly lived in Queniborough; Pamela Beyless, of Glenfield, was a frequent visitor before she died aged 24; and Glen Day, who lost his battle against the disease aged 35, lived there too. They all died in 1998.

A fourth Leicestershire man died of variant CJD in May and a fifth probable case from the county is currently "poorly" in hospital.

The village's butcher David Clarke, who took over his shop 19 years ago, said he was "obviously worried" about the effect the deaths would have on his income.

Leicestershire Health Authority gave Queniborough Primary School's 250 pupils a questionnaire to take home to their parents on Tuesday, asking detailed questions about where they live and what they eat, headteacher Chris Davis said.

Stop the madness: CWD threatens Wisconsin's elk, deer and, ultimately, people.

15 July 00 The Isthmus magazine By BRIAN McCOMBIE
Reprinted with permission
Imagine a disease worse than AIDS rippling through Wisconsin's deer herd. One that's always fatal, cannot be tested for in live animals, and has the chance of spreading to anyone who eats the infected venison. Sound like the premise for Michael Crichton's next apocalyptic thriller?

Unfortunately, such a disease already exists in epidemic levels in the wilds of Colorado and Wyoming. It's infected some game farms, too, and Wisconsin game farmers have imported more than 350 elk with the potential for this disease, including elk from farms known to be infected.

"If most people knew what kind of risk this disease poses to free-ranging deer in the state, they'd be very concerned," says Dr. Sarah Hurley, Lands Division administrator for the Department of Natural Resources. The DNR is now testing free-ranging deer around these game farms for the disease: "We're focusing our energies on those areas where we think there's the greatest possibility of transmission."

The malady the DNR's looking for is chronic wasting disease (CWD)--better known, to the extent it is known at all, as mad elk disease. It's a form of the mad cow disease that devastated Britain's cattle industry in the 1980s, scared the bejesus out of the populace, and is believed to have killed at least 70 people to date. An elk or deer with CWD can be listless, may walk in circles, will lose weight and interact progressively less with fellow animals.

The corresponding human affliction is called Creutzfeldt-Jakob disease (pronounced Croytz-feld Yawkob) or CJD. People with CJD experience symptoms similar to Alzheimer's, including memory loss and depression, followed by rapidly progressive dementia and death, usually within one year. While CJD is rare (literally one in a million odds of getting it), over the last few years at least three deer hunters have died of it. There is no proof either way whether they contracted the disease from CWD-infected venison, but new research says it is possible.

All three varieties--mad cow, mad elk and CJD--belong to a family of diseases called transmissible spongiform encephalopathy. These diseases alter the conformation of proteins in the brain called prions; after-death brain samples usually show a series of microscopic holes in and around brain cells.

No one is exactly sure how mad elk disease spreads. At first, transmittal through blood seemed likely, as from mother to fawn. But CWD has moved between adult animals at game farms, leading scientists to conclude that it can be spread through saliva or simple contact. Also, the rates of transmission are higher in areas where animals have the most opportunities for contact. Wisconsin's concentrated population of 1.7 million deer interact freely with each other, and scientific modeling suggests CWD could tear through our deer herd devastatingly fast. Despite the danger, Wisconsin and other states are relying on only sporadic testing and a system of voluntary compliance. It's a system that some say has more holes in it than a CWD-infected brain.

At present, Wisconsin game farm owners, even those harboring elk and deer brought in from farms with known cases of CWD, do not have to call a veterinarian if a deer or elk suddenly dies or acts strange. They're also not required to inform the state Department of Natural Resources (DNR) or the Department of Agriculture, Trade and Consumer Protection (DATCP) if animals escape into the wild.

"The lax attitude is pretty shocking," says John Stauber, a Madison activist and co-author of Mad Cow U.S.A. To protect people and deer, Stauber argues for an immediate importation ban for game farms, plus programs of testing and surveillance. He suggests both DATCP and DNR aren't taking such measures because, as the regulators in charge, they don't want to find the CWD he thinks is likely already in state. "It's in their bureaucratic interest to not [actively] look for CWD in the game farms," says Stauber. "Because if they find it, who's to blame?"

In the wild and especially out west, chronic wasting disease is spreading fast. Northeastern Colorado documented its first case in 1981. By the mid-1990s, samplings of mule deer brains showed 3% to 4% testing positive for CWD. Within a few years, the rate was 8%, and now Larimer County, the center of the endemic area, has a 15% rate of infection among mule deer. It's also being found in deer and elk in Wyoming.

"Fifteen percent of a wild population of animals with this disease is staggering," says Dr. Thomas Pringle, who tracks CWD-type diseases for the Sperling Biomedical Foundation in Eugene, Ore. "It's basically unheard of. This appears to be an unusually virulent strain. with highly efficient horizontal transmission mechanisms."

CWD could eventually spread to Wisconsin on its own, animal to animal. But that would take decades. Game farms, though, provide a mechanism to cut through all that time and distance and drop CWD smack in the middle of the state.

An open-records search by Isthmus reveals that the first shipment of farm elk from areas with CWD in the wild occurred in 1992, with 66 Colorado elk going to a game farm in Plymouth. In April 1998, DATCP was informed that a Bloomer game farm had purchased one elk from a Nebraska farm later found to be CWD-infected. This prompted a Sept. 15, 1998, memo from Steven Miller, head of the DNR's Lands Division, to Secretary George Meyer, with copies to DATCP chief Ben Brancel and Gov. Tommy Thompson. In it, Miller recommends that Wisconsin follow the lead of Montana (which found CWD on two game farms) and place "a moratorium on the importation of all game farm animals.... At present it appears the only way to help assure the disease does not spread into Wisconsin."

But the moratorium was never put in place, so it's possible that even more elk potentially carrying CWD are now in state.

Instead of a moratorium, Wisconsin has opted for testing. It is among 12 states and two Canadian provinces that currently test deer for CWD. Last year, the Wisconsin DNR began testing road- and hunter-killed deer in 1999 within a five-mile radius of game farms that have brought in elk from CWD-infected areas. Test areas include all or part of Fond du Lac, Dodge, Jefferson, Sheboygan and Washington counties. All of the approximately 250 brains examined in 1999 came back negative; this year, 500 to 600 deer will be tested.

Meanwhile, DATCP is asking owners of game farms that have animals from herds known to have cases of CWD infection to voluntarily enter a surveillance program. The agency's top veterinarian, Dr. Clarence Siroky, argues that voluntary compliance makes more sense than a moratorium because, ban or no ban, game farm operators "are going to find a way to bring these animals into the state. We don't have police patrols and impregnable borders to keep anything in or out."

With voluntary compliance, Siroky says, at least there are records of animals entering the state. So if CWD or other diseases are discovered, the animals can be traced back to their original herds and other farms they may have been at. "It's better to know where the animals are coming in from," he insists.

Siroky may be right that an importation ban would result in some game farms smuggling in animals. But currently, game farmers can bring in any deer or elk, even those from known CWD-infected areas, so long as they can produce a health certificate showing the animal's been tested. The problem is that no test exists to find CWD in live animals. Animals can carry CWD for years and still look healthy, so some of the 370 elk shipped into Wisconsin between 1996 and 1999 from CWD areas could have the disease. The odds are even higher for animals purchased from farms later found to have CWD.

Wisconsin has approximately 100 deer or elk farms and they're big business. On the Internet, prices for elk calves start at $1,500, and breeding bulls go for up to $20,000. Some farms sell venison and the velvet that peels from new elk antlers (considered an aphrodisiac in Asia). Others offer "hunts" costing between $1,000 and $5,000 for trophy deer, to more than $10,000 for bull elk with massive antlers.

Given these economics, it's reasonable to question why anyone with a suspicion of CWD in his or her herd would call in state regulators or a vet. A farm with a proven CWD case, confirms Dr. Robert Ehlenfeldt, DATCP's director of Animal Disease Control, would be shut down indefinitely.

And if a problem develops on a Wisconsin game farm, there's no guarantee that's where it will stay. Dr. Hurley says even fenced-in animals have easy nose-to-nose contact with wild and other farmed animals. Besides, as the DNR's chief of special operations Thomas Solin has documented, many game farms are not secure. Gates are sometimes left open. Fences rust and break, rot and topple, get crushed by fallen trees. Even if game farm animals don't escape, such breaches allow wild deer to get in, mingle with the farmed deer and elk, then leave.

Unlike other diseases, there's no test for CWD in living animals because it doesn't create an immune system counter-response, detectable through blood analysis. You can't kill CWD and related diseases by cooking the meat. One test Stauber recounts in Mad Cow U.S.A. found that scrapie, a sheep form of CWD, stayed viable after a full hour at 680 degrees Fahrenheit. Most disinfectants don't kill these diseases, either, and they can exist in the soil for years.

And while diseases like mad cow and mad elk do have some trouble jumping from species to species, it can happen. This May, Byron Caughey of the National Institutes of Health announced that he had converted human brain materials with mad-elk-contaminated brain matter at rates roughly equal to the transfer between mad cow and humans.

Says Dr. Pringle, referring to Caughey's work, "CWD may not transmit that easily, but the rate isn't zero." Pringle notes that the test Caughey used has been a very reliable proxy in the past in determining transmission possibilities for other diseases, including mad cow.

Once they jump the species barrier, transmissible spongiform encephalopathy diseases adapt to fit the new host and are then passed on rather easily within that species. Unfortunately, says Pringle, no one is trying to determine if CWD has jumped into people as Creutzfeldt-Jakob disease. Making matters more difficult is the fact that the disease can incubate for decades before symptoms are seen.

In states with CWD-infected deer, thousands of people have undoubtedly been exposed to CWD-infected venison. A February 1998 Denver Post article tells of one hunter who's venison tested positive for CWD. By the time he was notified, his meat had already been ground up and mixed with meat from hundreds of other deer for venison sausage.

With AIDS, Pringle notes, there was a definite overreaction, with people initially afraid to even shake hands with people infected with the virus. Looking at the CWD situation in Colorado, he says there's been complete underreaction. "It's like, oh, what the hell. Nobody's died yet--so keep eating the venison!'" Pringle worries that if the disease is found in humans, it will be so only after years of spreading through the human community.

Looking over documents obtained by Isthmus through its open-records request, Stauber says DATCP is behaving more like a lobbyist for the game farm industry than an agency bent on protecting Wisconsin's people from CWD. He points to DATCP's Cervidae Advisory Committee as a prime example. In a Nov. 11, 1998, memo from Siroky to DATCP secretary Ben Brancel, Siroky notes that the committee is needed to "obtain information from the public concerning disease regulation" of farmed deer and elk, and "to help formulate action plans for importation requirements, prevention and control" of CWD. But of the 12 people Siroky nominates, one's a DNR warden, one's a DATCP employee, and the other 10 are game farm owners. And two of these owners were among those DATCP knew had purchased elk from farms at high risk of having CWD.

"There's no significant input from anyone else," says Stauber. "Farmers, deer hunters and consumers are all left out. Meanwhile, the government's failing to take all necessary precautions to alert the public to this potential health threat."

How CWD spreads from Colorado

15 Jul 00 webmaster opinion
Fore background on CWD, see  1, 2, 3, 4
McCombie, in the article above, obtained access under Wisconsin's Freedom of Information Act to various CWD documents that since have been circulating on the internet, which the webmaster believes are authentic being plausible, consistent with previously disclosed information, and bearing signatures on official state stationery. These letters suggest that widespread traffic in elk coupled with minimal or non-existent state regulation and monitoring has the potential to spread CWD far and wide. Indeed, Colorado, Wyoming, Nebraska, Montana, Oklahoma, and Saskatchawan have announced cases; South Dakota,Missouri, Vermont, and numerous others have documented trace-back or trace-forward herds.

The first document, dated 17 Apr 98, on state letterhead from Nebraska State Veterinarian Dr. Larry Williams to Dr. Clarence Siroky, Wisconsin State Veterinarian, is a routine herd trace-forward letter. It states that on 9 April 98, an elk from a "domesticated cervine herd " at Heartland Elk Ranch in Cody, Nebraska, was diagnosed positive for CWD. The letter states that on 7 Feb 98 an elk (tagged as "Blue 40") was sold to T/D's Elk Farm in Bloomer, Wisconsin.

The webmaster is not aware of any follow-up on "Blue 40" or any other animal on either recipient Wisconsin farm; these animals are not necessarily infected or a risk though the potential is there. None of the game farms mentioned here did anything improper, imprudent, or illegal -- CWD emerged as a serious concern subsequent to these animal transfers; there was no way of knowing the status of a given game farm at the time. There is no indication that anyone sold or purchased an animal known to be at risk of CWD. The game farms here cannot be singled out as being at more risk than other game farms nationwide which have trace-back connections, direct or indirect, to Colorado.

As noted in McCombie's article, the first shipment of farm elk from areas with CWD in the wild occurred in 1992, with 66 Colorado elk going to a game farm in Plymouth, Wisconsin. The above is apparently the first shipment to Wisconsin from an elk farm with a confirmed case of CWD.

The second public document, dated 27 May 98, on state letterhead from Dr. Robert Ehlenfeldt, Wisconsin Bureau of Animal Disease Control, to the owner of an elk game farm in West Bend, Wisconsin, provides trace-back provided by the State of Colorado, which according to the letter, identifies the Elk Echo Ranch in Stoneham, Colorado as the source herd for the CWD-diagnosed elk in Nebraska and further imported Wisconsin elk. it is important to stress that no CWD is suspected or diagnosed so far in Wisconsin cervids at any facility.

Dr. Ehlenfeldt says that "the state of Wisconsion currently has no rules covering CWD and is taking no action at this time. No restrictions are being placed on your herd."

Stoneham, Colorado is located near the Pawnee National Grasslands in Weld County, which borders to the east Larimer County and the epicenter of every case of CWD, the Foothills Research Station in Ft. Collins. It is entirely possible that surplus elk were sold from the Foothills Research Station to the game farm.

In the webmaster's opinion, the primary responsibility for the creation and spread of CWD falls upon the incompetent and unethical cervid nutrition researchers at the Foothills Research Station who let 67 cervids die of an infectious disease (said by staff in 1967, repeat 1967, to resemble scrapie) over a 13 year period without conducting an autopsy, while selling off surplus infected animals to zoos, game farms, and 5 other research facilities, not to mention releasing infected animals to the wild.

This facility was so shabbily constructed and maintained that according to their annual reports, feral dogs in the Ft. Collins area were repeatedly allowed to dig under the fences to attack and kill captive deer. In short, CWD, like killer bees, the algae Caulerpa taxifolia, and many others, was released into the environment by careless research scientists.

McCombie reports that in Dec 1999, researchers Mike Miller, Beth Williams, et al, reported on CWD at a Geneva, Switzerland TSE conference hosted by the World Health Organization conference. Gregory Raymond, in Caughey's lab, spoke on prion conversions in vitro. CWD prions converted human prions to a reliable proxy of the infectious form. This was counterbalanced by saying conversion is "weak" and transmission rates "low."

Raymod states: "PrP CWD induced moderate conversion of ovine PrP-sen and similarly weak conversions of human and bovine PrP-sen molecules. The efficiency of PrP CWD -induced conversion of human PrP-sen was similar to that induced by PrP BSE and less than that induced by ov-PrP Sc."

Similar to that induced by PrP BSE? Hello, is anybody home??? We have a BSE-to-human epidemic epidemic going on. Yet Colorado DoW still sold game tags to Larimer County in the fall of 1999 despite their people speaking at this WHO conference. Surely this is not public policy based on the best available science.

In their recommendations, they concluded that "there is currently no evidence that CWD in Cervidae is transmitted to humans..." (safe enough: no one has a method for diagnosing such an event) but go on to recommend that people don't eat CWD infected animals or feed them to other animals. See section 4.3, with recommendations at the end, of this pdf.

Note a single Colorado herd sourced both Nebraska herds [see below] and also supplied animals to 2 Wisconsin game farms; Colorado has not publicly released a full set of trace-forwards from this herd nor is the source of its animals known. A second herd in Colorado had a positive case in an animal from an unidentified, possibly third Colorado game farm.

The third document, dated 23 Sept 98, on Wisconsin Commerical Deer & Elk Farmer's Association letterhead from its president Mike Monson to the Wisconsin State Veterinarian, states that "the mention of a moratorium [on importing elk from CWD affected states until a diagnostic test is available, suggested by Wisconsin Dept of Natural Resourses] is not only premature, but shows, in my opinion that some people in the DNR are out to get us. We made it out of the department regulation [from DNR to the friendlier state ag dept] and as you know we have been a very cooperative and clean industry. A moratorium imposed by the DNR smacks of the way the DNR has always looked at our industry and why the industry has been so slow to grow, and provide the venison the US consumer is demanding...."

Recall that "A Model Program for Surveillance, Control, and Eradication of Chronic Wasting Disease (CWD) in Domestic Elk" presented at the October 1998 meeting of the United States Animal Health Association (USAHA) and developed through the initiative of the North American Elk Breeders Association, sets minimum standards for herd surveillance and monitoring by calling for voluntary CWD testing of all elk 16 months or older that die in a herd. An annual herd inventory with verification by a state or federal animal health officer would be made, and elk herds would obtain a graded status depending upon the number of years monitored without evidence of CWD. Animals could be transferred among herds of equal or lesser status.

In the event of a diagnosis of CWD in a captive elk herd, the Model outlines mandatory steps for herd disposition. The length of the quarantine period depends upon whether the state veterinarian believes there is evidence of spread of CWD within the herd as opposed to a single case. The minimum quarantine for high risk animals (those that were in contact with a CWD-positive elk) is 4 years. Herd surveillance, which includes mandatory death reporting and CWD testing on all dead animals, must be done for 5 years after the last CWD case is diagnosed. [Dr.Victor Nettles, SCWDS Briefs, Jan 99, a newsletter from the Southeastern Center for Wildlife Disease Study at the University of Georgia]

There are provisions for sacrificing and testing the high-risk animals in the herd. High risk animals are elk that were pen-mates of an affected elk for any time up to 1 year prior to the death of the affected animal. Trace-back and trace-forward elk herds must be monitored for 3 years from the date of exposure to the affected animal.

The Model has been distributed to all state veterinarians. Although the surveillance aspect in the Model is intended to be voluntary among elk farmers, it is likely that many state veterinarians will make it mandatory. [Few did.]

The questionnaire CWD Survey by SCWDS found 6 of 31 responding states identified and pursued diagnostic evaluation on high-risk symptomatic animals; individuals in 13 states in addition to Colorado and Wyoming collected brains from 1,830 normal wild cervids for surveillance.

Another CWD case in Nebraska occurred in a captive elk in Nebraska in a captive herd of approximately 10 animals in Cheyenne County, Nebraska near Scottsbluff. It died on April 26, 1999. The Nebraska Game and Parks Commission will periodically check this herd and wild elk and deer in the vicinity for clinical signs of CWD.

According to the SCWDS newsletter, all captive elk on this newly diagnosed farm came from a single source herd in Colorado, which was the same source herd [Elk Echo Ranch in Stoneham, Colorado] where the first CWD elk in Nebraska of April 1998 was diagnosed (SCWDS BRIEFS Vol. 14, No. 1). There is an investigation underway to determine if other Nebraska elk herds may have received elk from this Colorado herd as well. There were no movements of elk from the second affected Nebraska herd since it was established in March 1997. Nebraska Game and Parks Commission personnel collected a wild elk that had been interacting with the captive elk through the fence; the brainstem from this wild elk was negative for CWD.

In September, 1999 according to this same wildlife veterinarian newsletter, CWD was diagnosed in a single elk from a small alternative livestock farm in Colorado. The herd was placed under quarantine and arrangements are being made for the state to purchase and depopulate the animals [not explained why public should pay for this]. Approximately 18 months prior to the CWD diagnosis, the affected elk was purchased from a larger commercial herd in Colorado, and this larger herd remains under quarantine until the completion of an epidemiological investigation. Colorado initiated mandatory CWD surveillance of privately-owned cervids in 1998. {SCWDS Briefs, John Fischer, October 1999]

Montana to ban new game farms

July 13, 2000 PRESS  RELEASE SPORTSMEN FOR  I-143: Game farm reform initiative  qualifies for ballot
Contact: Stan Frasier,  406-439-2705
Sportsmen for I-143, backed by Montanans Against the Domestication and Commercialization of Wildlife (MADCOW), the Montana Bowhunters Association and the Montana Wildlife Federation, have gathered more than the necessary signatures  required to place a game farm reform initiative on the November ballot.  Volunteers from Libby to Glendive in only 40 days collected over 29000 signatures.

If passed by Montana voters in November, I-143 will place a moratorium on the licensing of new game ranches in the state and ban the paid shooting of captive big game animals on game farms in the state. Existing game farms that raise elk and deer for meat and velvet will remain in business.

The Secretary of State's office has thus far tallied 20,545 qualified signatures from 39 legislative districts -- well beyond the 19,862 signatures from 34 legislative districts that were required by June 23. Signatures are still coming in from county offices.

"We have received overwhelming support from hunters and conservation groups throughout Montana," says MADCOW President Gary Holmquist. "In a one four day period alone volunteers went out and gathered more than 20,000 signatures. This tremendous effort emphasizes what we've known all along -- the citizens of Montana are fed up with big game ranching, problems such as Chronic Wasting Disease that this industry has brought into our state, the threats to our wildlife heritage through commercialization and privatization of big game animals, and the appalling violation of our fair-chase hunting traditions when wealthy people pay to kill domesticate elk and deer behind high fences. Now, in November, voters have a chance to do something about it and protect the wildlife and hunting heritage we cherish.".

School meals linked to CJD deaths

16 July 2000  Independent on Sunday 
By Geoffrey Lean, Environment Correspondent 
Infected baby food and school meals have helped to cause the human equivalent of BSE, Britain's top watchdog on the disease fears.

Dr Robert Will, director of the government's CJD surveillance unit, believes that those foods may be responsible for the alarmingly high proportion of young people among those dying from the incurable illness, which now appears to be increasing sharply.

He was speaking to The Independent on Sunday as ministers launched an investigation into a cluster of four cases of the disease ­ including two teenagers ­ in three villages north of Leicester. The government's scientific advisory panel will discuss the cluster tomorrow. Dr Will believes one of the greatest mysteries of the disease is why "new variant CJD", the human BSE, disproportionally hits young people.

The youngest two victims first showed symptoms at the age of 14, and many others were also teenagers. Stacey Robinson, 19, and an unnamed 19-year-old man are among those who have died in the cluster centred on the Leicestershire village of Queniborough. Conventional CJD rarely strikes people under 50, and most victims are in their 60s.

Dr Will said one explanation of new variant CJD in the young could lie in the amount of mechanically extracted meat in the food of babies and children in the 1980s. Dr Will said that it "could have contained remnants of the spinal cord", one of the parts of a cow which becomes most highly infected when the animal develops BSE.

"Foodstuffs which contained that material could be particularly dangerous," he said. "One possible explanation for the age distribution is that young people tend to eat these products more than the adult population."

Mechanically recovered meat was widely used in baby foods and was likely to have been common in school meals. "It was used in the cheapest products," he added. Sausages, pies, burgers, patés and some ready-cooked meals ­ as well as infant foods ­ were particularly likely to contain it.

Dr Will stressed that he had no proof that this explained the toll among young people. Other explanations could be that children absorbed the prion that carries the disease more than adults, or that it affected their cells more.

Dr Erik Millstone, senior lecturer at Sussex University's science policy research unit and an expert on food policy, said "one very plausible explanation" of the higher rates of the disease among young people lay in the food given to babies and young children. Another cause might be that young people were more vulnerable to infection. He thought that both factors were probably involved.

The investigation into the Leicestershire cluster ­ by Dr Will's unit, the Public Health Laboratory Service, London School of Tropical Hygiene, Leicestershire Health Authority, the Department of Health and the Ministry of Agriculture, Fisheries and Food ­ is focusing on local abattoirs and their slaughtering practices.

Last week Dr Will's unit disclosed that 67 Britons are now thought to have caught the disease from infected beef, an increase of 25 on a year ago.

American nvCJD involved in Queniborough cluster?

17 Jul 00 correspondence, used with permission; names withheld

This Leicester issue caught my eye.

I'm sure you don't remember, but my mother in law died of CJD (clinical diagnosis but no autopsy at my father in law's request) in November 1998. She was British by birth but had lived here since 1947. In the early 70's my father in law was transferred by Caterpillar to England to help open a plant in Leicester.

She jumped at the chance to "go home". My husband was in law school and didn't go, but my brother in laws were in high school and did go with their parents. They lived in Leicester for 3 years before moving back to the states (Cleveland, OH area).

Anyway, this may be our connection. She would be the 5th or 6th case. My father in law is trying to contact the authorities there. Do you have any e-mail addresses for either the writers, newspapers, or health authorities? We would like to pursue this.

I do wish we'd had an autopsy, but my father in law didn't want to "subject her to any more"--he was on over load and just couldn't bear the thought. I do know how hard it will be, but we do have the clinical diagnosis, but we'd never know sporadic from nvCJD. She was cremated so there's no chance. Maybe a possible additional case will wake them further--it just can't be ignored. It's too coincidental to be a statistical anomaly.

Thanks for any help,
[name withheld]

Comment (webmaster): This case warrants further investigation as possibly another element of the Leicester cluster and also the first case of nvCJD in the US. (Note blood banks have recently pooh-poohed any such risks.) The odd part is that the November 1998 date dovetails with other cases. If nvCJD could be reliably inferred from still-existing clinical records and codon 129 analysis showed met/met in the absence of other familial mutation, this case should be added to the cluster. It would be of importance to determine if the victim lived in Leicester or a suburban village such as Queniborough. The other point of interest would be pinpointing the time of exposure to BSE agent.

Note the mid-70's seems a bit too soon relative to the timing of the BSE epidemic. This case could simply be sporadic CJD. The age of the victim is , 75, at death is well beyond the outer limits of known nvCJD cases. Leicester, based on its population of 300k, should report a case of CJD every 3-5 years. Leicestershire CJD cases put down to sporadic over the last 20 years should all be reviewed as well.

Further details (disseminated with permission):

"age at onset and death:  75
First notice strange symptoms in June 98
Became very noticeable in early August 98
Died November 1998
Clinical Diagnosis received Sept 16, 1998 from private neurologist who first
saw her in July 1998.  Symptoms:

June/July 98: Confusion, absentmindedness, hallucinations, total lack of
inhibition (prim British woman so very pronounced change), forgot how to
do simple tasks like cooking, laundry, etc., constipation (5-7 days w/o
"going"), stomach pains, no medical reason after many G.I. tests at
Cleveland Clinic. 

Dr. said it was "neurological, not gastrointestinal problem
(message from bowel to brain and back not being received)
August:  Couldn't toilet, dress, bathe without help, hallucinations
continue, disorientation, unaware of surroundings (tried to walk off 5th floor
balcony to get to beach), gross confusion, child-like (from a very assertive woman), 
needed help with eating, reminders to chew, etc.  Panicked one night, got violent with husband and nurses
aide and was hospitalized, went from there to nursing home (Alzheimer's wing),
posture changed - neck began to be at 90 degree angle from back (like
some Parkinson's), sleeping very little

Sept 98:  Sometimes recognized husband, sons, but totally forgot
grandchildren, speaking very little, sleeping a lot, not eating (put on
Ensure) but again needed  to be reminded to swallow, etc., all "manners"
gone, unable to use utensils, toilet, bathe without extreme help.
Sleeping VERY little--  micro-naps other than an occasional hour
at night. Frenzied walking/racing around nursing home using a walker.
Crashing into walls, carts, people, etc. Would stand and sleep for a minute and
move on.  
Seemed  like her frontal vision was gone, seemed she had peripheral
vision only, based on heading straight for people but able to steer
clear of something along the sides of the walls such as parked laundry carts.
Lasted about 3-4 weeks.

Oct 98  Sleeping most of the time, basically unresponsive, eating only
when prompted and nurses would find unswallowed food next day.  Choked
occasionally.  Didn't recognize husband, sons, or me.  Avid crossword
puzzler and one day mid-October able to give husband correct verbal answers to 3
or 4 crossword puzzle hints.  Strangest thing, hadn't talked in a week or so
and he was just trying stimulate her and she responded!  Nothing else from
her. Almost last time she ever talked.  It was as if that little part of her
brain hadn't been destroyed.  No one could explain.

Nov.98 Basically comatose most of the time.  Rarely able to arose her.
Unable to arose her for food and water from about November 12.  Died on Nov.18.1998

Born in UK - Northhampton
During WW II - served with Royal Air Force in Algiers for one year (met husband there)
Moved to US (Detroit) in August 1947, moved to Cleveland Ohio in 1951
Back and forth to England many times beginning in 1948, 1950, etc.  

Transferred to Leicester in 1972.  Lived there 3 years, then returned to
Cleveland, OH
Will double check on exact location of home, but was in Leicester immediate area.
Back and forth to England 5 or 6 times between then and time of death.
Wasthere in late 80's.  Usually went to Northhampton where family still resides.

Mad sheep: a rush to judgement?

17 July 2000 webmaster opinion
The USDA is claiming that 4 sheep on a Vermont form tested positive for TSE, with scrapie being ruled out, and BSE the likliest possibility (from British feed used in Belgium in the flock's antecedents, not actually documented).

The USDA issued itself a Superduper Urgent Emergency Forcible Seizure Order on Friday afternoon (to bypass the judicial system) to seize and incinerate the sheep, take off 6 inches of topsoil off three farms (400, 96, and 150 acres) for hazardous waste treatment, incinerate all other animals on the farm including lamas and family dogs, and shoot and examine deer adjacent to the property.

Let's see: 644 acres of land, 42000 square feet per acre, 6" deep -- that pencils out to 50,000 dump trucks of 10 cubic yards. Where are these being taken? Dust and erosion measures? How did they arrive at the figure of 6 inches?

The sheep have been on these pastures since 1996, so what exactly is the rush? None of the sheep displayed any symptoms whatsoever. They were culled for mastitis and as excess rams.

USDA further tracked down purchasers of sheep meat sold from one farm and sent them a warning letter saying they may have been exposed to BSE and would they like to be notified if a vaccine is later developed? This premeditated nonsense is meant to undercut community support for the sheep farmers.

Allegedly, MJ Schmeer informed the USDA that her test was not yet validated, gave mixed results on the sheep, and could not be used to enforce the seizure order.

Reportedly, for the western blots, Detwiler sent samples to Richard Rubenstein of the Department of Virology, NYS Institute for Basic Research in Developmental Disabilities, Staten Island New York. His most recent paper was "Characteristics of scrapie isolates derived from hay mites." J Neurovirol. 2000 Apr;6(2):137-44.

In the webmaster's opinion, you can't believe in the western blots without also believing in hay mites.

Apparently the family can document that the samples were neither single nor double-blinded and contained no internal control lanes. Detwiler has yet not produced any chain-of-custody paperwork which is essential in hazardous material handling and required for credibility.

It is not clear why adequate material was not sent to Janice Miller at the same time it was sent to Schmeer.

Detwiler reportedly told the family there was no more sample material available, it had all been used up. We heard this before in the New York pig incident. Reportedly, some of the 499 imported cows to the US from the UK examined histopathologically had a number of problems similar to the sheep, but they were blown off as negatives, no western blot, no IHC, no action taken.

USDA has been gunning for these sheep for a long time -- they decided in 1998 that the flocks had to be taken out. The "diagnosis" was just a matter of time.

It would make anyone a little jumpy to have the government served a forcible seizure order, timing it for the weekend to preclude a temporary restraining order issued by a judge that would allow for expert outside review of the data or a repeat of western blot by an independent laboratory.

The federal government is feeling good about itself again. They just got acquitals for the murder of 80 people at Waco (including 20 children) and for Ruby Ridge sharpshooter Ron Horiouchi (FBI agent who shot the wife of a suspect in the head), meaning government resources are available if needed for the sheep operation.

In my opinion, the USDA does not have a diagnosis of TSE at this time that would stand up to expert review. The connection to BSE is far-fetched speculation. The US has many undisputed more significant and insiduous exposures to BSE, including importation of many tons of MBM. Who ate this and where are they now?

So what is this all about? It is about sending a message, demonstrating our incredible resolve to the Europeans to do whatever it takes to lick BSE, even if that means sacrificing a few of our own small businesses.

Meanwhile, Vermont imports elk from Colorado and nobody cares. Other Vermont flocks have proven scrapie, yet off goes the mutton to the supermarket. There is solid scientific support for transmission of both to humans from in vitro proxy tests, yet nothing is done. Those cosmetics, beef, pharmaceutical export sales are beef-based, not sheep or elk.

This is all about (surprise!) money, not managing public health risks.

Secretary Glickman's signed order is now available. It is actually a "Declaration of Extraordinary Emergency Because of an Atypical Transmissible Spongiform Encephalopathy of Foreign Origin". It starts off,

"A transmissible spongiform encephalopathy (prion disease) of foreign origin has been detected in the United States. It is different from TSE's previously diagnosed in the US. The TSE was detected in the progeny of imported sheep... TSE's are caused by similar, as yet uncharacterized, agents [sic] that usually produce spongiform changes in the brain. Post-mortem analysis has indicated positive results for an atypical TSE of foreing origin in four sheep in Vermont.

Because of the potentially serious consequences of allowing the disease to spread to other livestock in the US, it is necessary to seize and dispose of those flocks of sheep in Vermont that are affected with or exposed to the disease, and their germ plasm. The existence of the atypical TSE of foreign origin represents a threat to US livestock. It constitutes a real danger to the national economy and a potential serious burden on interstate and foreign commerce... Based on review and consultation the Department has determined that Vermont does not have the funds [sic] to compensate flock owners. Without such funds, it will be unlikely to achieve expeditious disposal of the flocks and germ plasm. Therefore..." the USDA has to take over the operation, citing 21 USC 134-134b of 1962.

What was the factual basis for (1) TSE, (2) atypical, (3) foreign origin (4) including germ plasm?

1. They have no data on transmissibility, stating that it would take several years to test this plus the samples have been thrown out already. The webmaster has not been able to review the histopathology slides -- these would best be scanned and posted to the Internet by the USDA.

2. If there was such a thing as typical TSE, it would be easier to assess an atypical one. The webmaster questions the ability of anyone to state that it is "different from TSE's previously diagnosed in the US". No one person has looked at even a fraction of the slides. This is a disease with tremendous natural variability -- look at CJD variability within identical twins. There are a great many origins of spongiform condition having nothing whatsoever to do with TSEs; indeed,"atypical" here apparently means all the slides were IHC-negative for prion protein, not supportive of the diagnosis.

3 The logic used here, applied to the first case of CWD, says that disease too is of foreign origin, yet we know it is not. Were Marsh's cases of TME from Wisconsin downer dairy also of foreign origin? These were 'different' too. If USDA needs proof of foreign origin, they aren't even close.

4 There have been massive numbers of embryo transplants and sperm imports to the US from England during 1980-2000. Where is the tracking and seizure of these animals? Where are the published papers establishing these materials as a threat?

Getting on to the data on the 4 positive sheep. I also received a faxed report dated 7 July 00 from Rubenstein to Detwiler and a 25 May 00 report from Schmeer to Detwiler.

Rubenstein starts off saying "As per your request, we processed these Vermont sheep brain samples" making it clear that Detwiler and Al Jenny, who provided the samples, had told the lab about the samples origin and, one supposes, their threat to US livestock, the real danger to the national economy, and interstate and foreign commerce.

The 7 samples are not coded in any way but given straightforward identification numbers familiar from the Ames facility. No control samples were included. Rubenstein ran western blots using rabbit polyclonal antibody left over from the 1993 Kascsak paper, finding staining for Prp-Sc as follows (no gel scan was included, gel lanes were not described):

3714 +
3711 +
3715 ++
3701 -
3706 +
6633 +-
C070 +-
Prior to this, MJ Schmeer had received 5 of these same 7 samples on 8 Jun 99. In a letter to Detwiler dated 25 May 00, not describing the methodology but stating "if the number is below 70% the animal is considered positive" Schmeer provided this data:
3714  53.9
3711  47.1
3715  56.7
3701  31.7
3706  52.9
6 other samples not shown; one run twice giving 70.2 and 100.
Note that the animal having the most dramatic Prp-res score on the Schmeer test, scored negative on the Rubenstein western blot. The sheep with the least dramatic Prp-Sc score got the highest ++ rating on the blot.

This contradiction was not ironed out, but instead Detwiler went forward with the 4 that gave the answer that Glickman wanted. Any test will give false positives so this ensures that if enough samples are run, there will always be some that agree on the two tests. And the ones used here are tests have notable uncertainties. Schmeer's letter in particular provides a disturbing lack of reproducibility in test scores; hay mites speak for themselves.

Schmeer also knew beforehand that the samples came from the controversial Vermont sheep, stating "Dear Dr. Detwiler, These are the results from some of the samples of sheep that were submitted from the flocks in Vermont."

There are a great many reasons why it is standard scientific practise to double-blind samples even in non- controversial situations. It makes especially good sense for a USDA-dependent lab processing samples that are a threat to US livestock, a real danger to the national economy and interstate and foreign commerce.

In the webmaster's opinion, there has been a rush to judgement. The USDA does not have its scientific ducks in row. This site supports USDA in taking draconian action but not willy-nilly nor as a publicity stunt to impress EU regulators. These sheep have a very extensive history of intense scrutiny. The owners would be within their rights to ask for a repeat of the western blot from a respected neutral laboratory such as O'Rourke or Miller that included appropriate internal controls. If the USDA has tossed out the brain samples already, it is their own fault and they need to start over.

The quarantine itself has always made sense, the last thing the US needs is possibly affected breeding stock distributed over 40 states, like what happened and is happening with CWD. TSE remains to be established in these non-clinical animals whose parents also remain asymptomatic; BSE is remote, but these tests raise concerns and call for further work and further quarantine. Britain has 40 million of sheep that ate megatonnes of MBM -- where is the BSE in sheep there?

If USDA wants to send out warning letters about possible exposure to TSEs, why don't they send out a similar letter to Americans who have eaten CWD venison or scrapie sheep or downer dairy or had a blood transfusion? Answer: these Americans are not a threat to US livestock nor a real danger to the national economy and interstate and foreign commerce.

Vermont sheep: send 'em back where they came from

17 Jul 00 news update and webmaster opinion
America has long wanted to solve its problems by sending 'em back where they came from. At various times, re-exporting was seen as the solution to perceived problems with Irish, German, African, Polish, Hispanic, Cuban, Chinese, Japanese, etc. etc. immigrants. The Indians even started driving around with bumper stickers saying "US out of North America".

Now the USDA wants to get rid of its perceived Vermont sheep problem by sending 'em back where they came from, namely Belgium.

The webmaster learned that Craig Reed, a big honcho at APHIS/USDA, contacted the Belgian Ministry on Friday 14 Jul 00, asking a ministry official if Belgium would accept the sheep back, and threatened Belgium that if they didn't take them that they would be destroyed because a new law and recent funding makes it easier for USDA to sieze them, so it would all be Belgium's fault. Reed told the official point-blank that the reason for this action was the EU's refusal to accept US beef.

By sending 'em back to their country of origin (how will they clean the charter jet?), a media circus could be avoided this coming Friday 21 Jul 00 over the announced forcible seizure under the Declaration of Extraordinary Emergency Because of an Atypical Transmissible Spongiform Encephalopathy of Foreign Origin.

Picture hundreds of little lambs crying plaintively as they are roughly loaded onto USDA trucks bound for slaughter at a hazardous waste incinerator. Heavyset men in moon suits chase down sheep on motorcycles while SWAT teams stand by. Naturally, for their own safety, the media cannot be allowed to enter the biosecurity hazard zone.

Will USDA really shoot Linda Faillace's dogs, as threatened, on national TV? Sure, if it would get the EU to lift the beef import ban.

It's all about demonstrating resolve. Like the Berlin airlift. Or after Sputnik, marching little boys off to summer Math Camp in a sweltering, alligator-infested Florida swamp (actually it was fun!).

Now the government has a point here in that the beef barrier is equal measures of sincere concerns over hormones and TSE mixed with insincere exploitation of these same issues to exclude competition.

With 20/20 hindsight, the USDA should not have sent out a press release saying the sheep had BSE and warning letters to people who had purchased the milk, cheese, and meat. This establishes for official purposes that BSE had reached the United States by 1996. This is what they concluded after duly considering the results of the 4 tests.

Nothing was done for 4 years and here we are today with 376 live BSE sheep on our hands, 3 thoroughly contaminated farms needing 6 inches of topsoil removed, deer on and off the property, hundreds of people needing long term monitoring for nv CJD. Will this take care of the problem or has BSE established a beachhead?

Why did they send out such a stupid press release? It was all posturing. Send a message to those dimwit eurocrats. They weren't thinking about the implications for the US or how it could drag on and on if the farmers found some way to delay or resist.

So how will it all end? The USDA is bound by federal law to only pay fair market value, but they will offer some huge settlement bonus on the sly. The sheep go quietly into the night, confidentiality agreements keep anyone from talking, private property no trespassing.

More western blots are run in-house and it turns out that it was just typical scrapie after all. No topsoil need be removed, no years of follow-up monitoring are warranted. This dis-establishes for official purposes that BSE had reached the United States by 1996. This is what they will conclude after duly considering the results of the 5 tests.

Let's wait and see. Farmers can be obstinate. Farms have to make money but people do not take up farming to make money.

CWD research update

Dec 99 WHO Consultation on Public Health and Animal TSEs, html, pdf

4.3 Assessing interspecies Transmissible Spongiform Encephalopathy transmission using the cell-free conversion assay

Gregory Raymond
Wild or captive deer and elk in certain areas of the US (Colorado, Wyoming, South Dakota, and Montana) are infected with CWD. Little is known about the transmissibility of CWD from deer and elk to other species. At least one animal transmissible spongiform encephalopathy (TSE) strain, BSE, has infected humans and many other mammalian species through the food supply. Hence, it is important to consider the possibility that CWD could also be transmitted to humans or other mammals exposed to CWD-infected cervid species. The transmissibility of CWD to animals can be tested directly; however such experiments will take years to complete because of the long incubation periods encountered in interspecies TSE transmissions. In addition, since CWD transmissibility cannot be tested directly in humans, we have sought alternative methods to help provide clues to the potential interspecies transmissibility of CWD.

The conversion of the normal protease-sensitive host prion protein (PrP-sen) to a disease specific protease-resistant isoform (PrP-res) is a key biochemical event that occurs in the pathogenesis of all TSE diseases. We have developed a cell-free system in which PrP-sen is converted to PrP-res 6 . The conversion efficiencies of various combinations of PrP-res and PrP-sen that have been analyzed correlate remarkably well with known in vivo transmissibility, suggesting that this assay could be useful as an indicator of the transmission potential between species 7 . Previously we used this conversion system to gauge at the molecular level the potential transmissibility of scrapie and bovine spongiform encephalopathy (BSE) to humans. We observed weak conversion of the normal human PrP-sen to the abnormal PrP-res isoform when incubated with PrP-res isolated from the brains of either scrapie-infected sheep (ov-PrP Sc ) or BSE-infected cattle (PrP BSE ). However, the efficiencies of these heterologous conversion reactions were much lower than homologous human, bovine, and sheep conversions. The similar but poor efficiency of PrP BSE or ov-PrP Sc to convert normal human PrP-sen suggests that, at least at the molecular level, the inherent ability of these diseases to affect humans following exposure is likely to be finite, but similarly low.

Using the cell-free assay, we have found that the CWD-associated PrP-res (PrP CWD ) of cervid species induced the conversion of cervid PrP-sen molecules robustly, correlating with known transmissibility of CWD amongst cervid species. In contrast, PrP CWD induced moderate conversion of ovine PrP-sen and similarly weak conversions of human and bovine PrP-sen molecules. The efficiency of PrP CWD -induced conversion of human PrP-sen was similar to that induced by PrP BSE and less than that induced by ov-PrP Sc.

The implication of these results regarding the potential transmissibility of CWD to humans is unclear because, while BSE appears to be at least weakly transmissible to humans, there has been no epidemiologically measurable transmission of sheep scrapie to humans. Nonetheless, these results suggest the existence of a molecular incompatibility that is likely to limit the transmissibility of cervid CWD strains to these non-cervid species.

6 Nature, V.370:471-474; Chem. & Biol. V-2:807-817.
7 PNAS, V.92:3923-3927; PNAS, V.94:4931-4936; Nature, V.388:285-288.

4.2 Diagnosis and pathogenesis of Chronic Wasting Disease

Beth Williams
CWD is a prion disease naturally affecting mule deer, white-tailed deer, and elk (wapiti). Research by a number of agencies and institutions is in progress on many aspects of the disease. Several pathogenesis studies are nearing completion: mule deer and elk have been orally inoculated and the progression of disease is being followed sequentially.

Dr. Williams described three major studies underway in her laboratory, involving oral exposure of both mule deer and Rocky Mountain Elk to brain material >from elk with clinical disease. These studies are being used to study the incubation period of CWD and to examine tissues of deer at sequential periods after infection, to determine which tissues are infected and when. Preliminary results to date indicate that these species are readily infected orally, PrP res may be detected by immunohistochemistry (IHC) in the parasympathetic vagal nucleus of the medulla oblongata of the brain by approximately 6 months post-inoculation, and that accumulation of PrP res occurs in lymphoid tissue months prior to the ability to detect it in the central nervous system. Appearance of clinical signs is associated with development of spongiform change. The pathogenesis of CWD in mule deer and elk appears to be similar.

Dr. Williams provided detailed information on the methods used for histopathology and immunocytochemistry, and a comparison of the efficacy of a number of antibodies for Western blotting. She states that immunohistochemistry is an extremely valuable diagnostic and research tool for CWD and is more sensitive than standard histopathology and western immunoblotting. Approximately 40% of deer diagnosed with CWD in surveillance studies show only accumulation of PrP res in the brain by IHC and do not have spongiform lesions. Optimization of the techniques for conducting IHC for CWD is necessary for each laboratory. A mouse monoclonal primary antibody (89/160.1.5 prepared by Dr. K. O¹Rourke) gives excellent results.

Strain typing by mouse passage suggests that CWD is unique and does not resemble BSE, CJD, or scrapie (Dr. M. Bruce, personal communication). A series of studies regarding cattle susceptibility are also underway. Cattle >from non-CWD endemic areas have been challenged intracerebrally and orally with innoculum from a mule deer brain pool. In addition, cattle have been left to co-habitate with clinically ill deer. Finally, the brains of cull cattle are being examined. At this point, CWD has not been diagnosed in any of the cattle experimentally exposed to CWD, nor in the contact and cull populations. Other studies of CWD in progress include additional strain typing, development of techniques for antemortem diagnosis, and investigations of PrP genetics in deer.

6.3 Are there prion diseases of fish?

Dr. Pocchiari
The recent description of a normal form of the PrP isoform in the brain of a salmon triggered a proposal to study the susceptibility of fish to the agents of BSE and scrapie. If infection can be established, the expression level of these agents in various fish tissues will be evaluated.

A series of experiments is underway in a number of European laboratories to study the normal isoform of the prion proteins in normal fish, and to study the abnormal isoform in fish experimentally exposed to the TSE agents. The methods of exposure and assessment of TSE infection are being developed. No results were presented. The project is expected to run for two more years; some results will be available shortly.

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