Prion Disease
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nvCJD caseload soaring: 5 new cases in July, total 82
200,000 patients received CJD-linked dura mater
Knacker's yard link to Queniborough nvCJD cluster
Pig swill, UK style
US -- is it any better?
Spain reports first declared cases of sheep disease scrapie
Brains in beef: stunning of cattle with captive bolt guns
CWD Canada: disease may wipe out elk industry
French screening program for BSE: more successes
TSE in dogs: new details emerge

Knacker's yard link to Queniborough nvCJD cluster

Sun, 13 Aug 2000 Jonathan Leake and Dipesh Gadher  Sunday Times Additional reporting: Graham Hind 
BRITAIN'S worst outbreak of the human form of mad-cow disease may be linked to a nearby knacker's yard that sold meat from diseased animals. The yard operated just eight miles from Queniborough, the Leicestershire village where health officials are investigating the first known cluster of CJD cases.

Three people who spent time in the village died from CJD in 1998, and a fourth person is suspected of having the degenerative brain disease. Another victim lived just three miles away.

The possible link to the knacker's yard - which recycled animals unfit for human consumption into pet food and other products - dates back 20 years, to about the time when scientists now believe the BSE epidemic may have begun.

Two meat traders from Bedfordshire were convicted in 1982 of buying unapproved beef from W E Mason & Sons of Wigston, near Leicester, and selling it to an unsuspecting butcher in Hertfordshire.

Last week officials seized council documents and court reports relating to the company to determine whether any unfit meat may have entered the human food chain locally.

"We have had a very useful series of conversations about this with Oadby and Wigston council," said Philip Monk, a consultant in communicable disease control at Leicestershire health authority, who is heading the Queniborough investigation. "I am ruling nothing in and nothing out. Anything we have that is potentially helpful in explaining local meat trading practices has to be examined."

The case heard by Leicester magistrates in 1982 was the culmination of Operation Meat Hook, a joint investigation between detectives and environmental health officers from three counties.

The teams covertly observed Peter Fletcher, a partner in a wholesale butcher's business near Dunstable, on four occasions in 1980 when he visited Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an un-marked van, which had been contaminated by a cow's head "fouled by stomach contents", according to evidence given in court. One of the carcasses was later found to have been infected with pleurisy.

Fletcher marked the meat with a fake inspector's stamp, and then left it with a retail butcher near Hemel Hempstead, Hertfordshire.

"A knacker's yard may, and frequently will, deal with diseased cattle," the prosecutor had told an earlier hearing. "Meat may be partly decomposed and contaminated. Disease is rife in such premises and could include anthrax and tuberculosis."

Fletcher was jailed for three months and fined £500. His partner, Francis Fensome, received a suspended prison sentence. Mason was cleared after telling the court that he had been told the meat was to be used to feed animals at Whipsnade zoo [site of two cheetah BSE fatalities -- webmaster]

The knacker's yard, which had been run by the Mason family since 1947, was closed the same year and now stands derelict. Mason has since died.

Last week his brother, Jack Mason, said: "I am confident there is no connection with us and the outbreak in Queniborough. Most of the meat went to zoos. Any meat that was sold locally went to dog owners as pet food."

There is no proof that Mason dealt in cattle infected with BSE, which was not recognised at the time. But such yards commonly dealt in "downer" cows - those displaying symptoms of illness - so any animals that did have BSE were likely to have ended up in such places.

The Queniborough inquiry team is also examining slaughtering techniques at Leicestershire abattoirs and childhood eating habits of those who grew up in the village, although school meals have been ruled out as a possible cause of the CJD outbreak.

Arthur Beyless lost his daughter, Pamela, 24, a bank worker, to the disease after a two-year struggle for survival. Although the Beylesses live in nearby Glenfield, Pamela regularly visited her grandparents in Queniborough and the family often bought meat from Ian Bramley, the village butcher, in the late 1970s and early 1980s.

Beyless said: "On one occasion I was buying some meat when Ian told me he'd got it for 'a good deal'. It does make you wonder when you consider this theory about the knacker's yard. This disease is something that might never have happened if people weren't always grasping for that last penny."

The other two named victims with links to Queniborough are Stacey Robinson, 19, who lived there for 12 years before moving to another part of the county, and Glen Day, 34, who worked on a farm in the area. He regularly ate at the Horse and Groom pub, which was supplied with meat by Bramley.

Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew nothing about Mason's yard. "We bought our meat directly from local farmers," she said. "The animals were slaughtered in Leicester and delivered to us. I don't know anything about this place in Wigston."

Bovine spongiform encephalopathy: Epidemiological studies

The Veterinary Record, December 17, 1988
J.W. Wilesmith, G.A.H.Wells, M.P. Cranwell, J.B.M. Ryan
"There was no clear or single explanation why, in 1982, cattle apparently became first exposed to a transmissible agent sufficiently to result in clinical disease. A number of factors have been identified which when combined are undoubtedly significant in the occurrence of this epidemiological phenomenon. These include; a dramatic increase in the sheep population in Great Britain which commenced in 1980 and has continued (MAFF 1988); a probable increase in the presence of scrapie infected flocks (J.W. Wilesmith unpublished data); the greater inclusion of sheep heads in material for rendering; the greater inclusion of casualty and condemned sheep in material for rendering as a result of the reduction in the number of knackers' yards; the introduction of continuous rendering processes during the 1970's and 1980's which may have resulted in the rendering of animal material at a lower temperature and, or, a shorter time than previously and the decline in the practice of using hydrocarbon solvents and terminal heat treatment for fat extraction since the mid 1970s (MMC 1985)...."

Pig farmers fined for dead-animal feed

PA News Fri, Aug 11, 2000 By Alistair Keely, PA News
Opinion (webmaster): Why the ruckus over the knackers om 1982? This pig swill doesn't sound any better it happened this year..

The pig industry tonight welcomed a 90,000 pound fine imposed on a family of farmers for feeding their livestock dead animals.

Andrew Cotton, 40, appeared at Lincoln Magistrates' Court on behalf of his family and pleaded guilty to five charges under the Animal Health Act for the illegal methods they used to prepare pig swill.

Inspectors from the Ministry of Agriculture, Fisheries and Food carried out a spot check at the family farm at Swinderby near Lincoln last December and discovered the illegal feeding methods being deployed at ME Cotton and Son. Waste being used in the swill included spaghetti bolognese and raw and cooked meats. Inspectors also discovered whole pig carcasses floating in the vats where the pig swill was being prepared.

The court was told the Cottons' methods presented a "serious risk to the food chain." Farmers can use catering waste in their swill but only under licence. The court heard the Cottons' licence had expired.

Andrew Cotton, mother Hilary, father David and brother Ian all faced charges. Each member of the family was ordered to pay 22,500 and legal costs.

Mike Sheldon, chief executive of the National Pig Association, welcomed the fine and described the family's behaviour as "morally wrong and repugnant." He said: "Their behaviour has been disgraceful. Their actions run the risk of generating a bad name for the pig industry. "They have broken the law, it is inexcusable, morally wrong, dangerous and repugnant. We welcome the fine. Hopefully it sends a clear message to other farmers not to break the law."

The court was told the Cottons opted not to renew their catering waste licence because of financial considerations. They were also concerned that customers would be put off by the methods used because of public concerns surrounding the BSE crisis.

A family member at the farm in Lincolnshire declined to comment tonight. A spokesman for MAFF said: "We hope the importance the court attaches to the offences involved sends a clear message to others to act responsibly."

Spain reports first cases of sheep disease scrapie

Tue, Aug 8, 2000 AP WorldStream
MADRID, Spain -- Authorities in Navarra province said Tuesday they had destroyed 2,400 sheep after detecting Spain's first cases of scrapie, an ailment similar to mad cow disease. [ Scrapie was reportedly first described in the 1700s in Spain. -- webmaster]

The animals came from farms in two towns in that northern region. They were sacrificed in June after tests on two samples totaling 12 sheep revealed three cases of scrapie, the Navarra agriculture department said. Officials plan to destroy another 400 sheep in the next few days, spokesman Javier Errea said.

The Spanish Agriculture Ministry confirmed that these were the first known cases of scrapie in Spain. Scrapie is a fatal neurological ailment similar to mad cow disease. Both are believed to be caused by a defective form of brain protein, called a prion. Symptoms in sheep include trembling, loss of coordination and scraping against objects. Veterinarians say scrapie was first diagnosed 250 years ago in Britain and other western European countries and is relatively common among sheep.

There are no known cases of its being passed on to humans directly. But an outbreak in Britain in the 1980s of mad cow disease -- which scientists now say does jump the species barrier -- has been blamed on the practice of feeding cattle the ground up remains of scrapie-infected sheep. The European Union has since banned that practice.

First cases of sheep disease scrapie found in Spain

Reuters World Report Tue, Aug 8, 2000
The regional government of Navarre said on Tuesday it had detected Spain's first outbreaks of scrapie in sheep, a variant of mad cow disease that is not believed to be transmissible to humans. A spokesman for the government of the northern region said the problem was under control, with a herd of 2,200 sheep having already been sacrificed and another 600 due to be culled.

"We have had cases...of scrapie in two farms and they're the first cases (in Spain) to be publicly declared," Navarre agricultural spokesman Javier Errea told Reuters.

The newspaper El Pais reported that there had been other cases in Spain, but they had not been confirmed publicly. No one at the Agriculture Ministry was available to comment.

Scrapie is a variant of the same family as bovine spongiform encephalopathy (BSE), the cattle brain-wasting disorder also known as "mad cow disease," that has been blamed for more than 79 human deaths in Britain alone. The European Union said last week that BSE probably exists in cattle in Spain, Germany and Italy, although those countries deny the presence of the disease.

BSE has yet to show up in sheep, but Professor Emmanuel Vanopdenbosch, one of the European Union's top scientists in the subject, told the Belgian newspaper De Morgen last week: "The BSE question with sheep is a time bomb that continues to tick."

Errea said the risk of the sheep disease being passed on to cows and perpetuated had been eradicated by a 1994 law banning the use of animal remains in cattle feed. "In reality there is no reason for concern," he said.

However concerns that BSE may appear in sheep have recently been highlighted by a case in Vermont in the United States where four sheep tested positive for transmissible spongiform encephalopathy (TSE), of which both scrapie and mad cow disease are derivatives. Experts say it could take years before it is known which form of TSE the Vermont sheep had.

July 2000: 5 new nvCJD cases, total 82

UK Dept of Health monthly statistics, covering through July 00
Comment (webmaster): Comparing to previous month totals, it seems that there have been 12 referals in July, of which 5 were nvCJD, 3 were sporadic and 0 familial or iatrogenic.

nvCJD alive changed from 7 to 9, nvCJD dead awaiting confirmation from 3 to 4, nvCJD confirmed from 9 to 11, and total number of definite and probable cases of nvCJD from 74 to 79, an increase of 5 in 34 days. This is the worst month ever; the previous month saw an increase of 4. At these rates, the total for 2000 will exceed the total for the preceding 5 years combined. It explains why the tone of government announcements has become more cautionary.

The age distribution of onset has stayed within bounds established early on, early teens to early fifties. After adjusting for demographics (more young people), it still has quite a tail on the older side, not to mention the excess of fifty-somethings. What it really means is quite unclear. Asymmetry of common distributions are largely found in the third moment, peakedness in the fourth; all the common ones have been most conveniently tabulated.

age bin  #
10-15  3
15-20  15
20-25  19
25-30  18
30-35  10
35-40  5
40-45  3
45-50  1
50-55  4
total 78
Lancashire Telegraph 10.8.00 Anita Bradshaw, 30, dies of nvCJD on 27 KJuly 00. The husband thinks it was due to her working in a butchers shop 14 years ago. {Dealler web page].

Times 22.7.00: Valerie Elliott, from Cheshire is a new nvCJD case. Kirsty Garven, 20, from the farming village of Waverton near Chester died two weeks ago 14 months after onset.

Referals Sporadic Iatrogenic Familial  GSS  nvCJDP  nvCJDpm confirm Total
113         15        0         0       0     9     4       11      39

To 4 August 2000. Total number of definite and probable cases of nvCJD = 79.
*Including 6 probable deaths from nvCJD where neuropathological confirmation will never be possible. 
The next table will be published on Monday 4  September 2000. 
101         12        0          0      0     7     3        9      31  

 To 30 June 2000. 
 Total number of definite and probable cases of nvCJD = 74. 

 To 2 June 2000. 
 Total number of definite and probable cases of nvCJD = 70.

Zoo animal totals by 30 June 00
lion 2
cougar 3
tiger 1
ocelot 3 
cheetah 7

kudu 6
gemsbok 1
nyala 1
oryx 2
eland 6
bison 1
ankole cow 2

House cats: total: 87 (plus 1 in N Ireland, 1 in Norway, 1 in Lichtenstein)
1990 12
1991 12
1992 10
1993 11
1994 26
1995  8
1996  6  1*
1997  6  2*
1998  4  2*
1999  2  1*
*born after Sep 1990, date ban on specified bovine offals extended to any animal feed

Brains in beef: stunning of cattle with captive bolt guns

Fri, 11 Aug 2000 J Neurosci Methods 2000 Jun 30;99(1-2):53-58
Opinion (webmaster): This study comes 15 year too late. To kill cows at a slaughter house, a captive bolt gun is used. This knocks pieces of brain into the circulatory system where they end up all over the body within seconds. Here brain, a highly infectious tissue, ended up in the blood detectably in 1 of 12 cattle. This shows how nonsensical it was to ban certain low value parts of BSE cows while selling the rest, saying for example that meat was ok.

Methods for detection of haematogenous dissemination of brain tissue after stunning of cattle with captive bolt guns.

J Neurosci Methods 2000 Jun 30;99(1-2):53-58
Love S, Helps CR, Williams S, Shand A, McKinstry JL, Brown SN, Harbour DA, AnilMH
"Because of concern that the stunning of cattle with captive bolt guns (CBGs) could, if used on an animal with bovine spongiform encephalopathy (BSE), cause embolism of infective brain tissue and carcass contamination, the Ministry of Agriculture, Food and Fisheries commissioned research to assess the risk of haematogenous dissemination of CNS material after stunning.

We have devised two methods to investigate this risk. The first involves the concentration of embolic tissue in buffy coat Cytoblocks that can be embedded for sectioning, microscopy and immunocytochemistry. The second method is an ELISA for the presynaptic protein, syntaxin 1B. The methods were validated by analysis of several bovine tissues, including blood samples deliberately contaminated with brain.

We then studied jugular venous blood obtained before and after the stunning of 60 cattle with CBGs. Samples obtained, after stunning, from five of the cattle contained CNS tissue within the Cytoblocks and yielded positive syntaxin assays. Syntaxin was also detected in samples from one other animal that had been stunned with a pneumatically operated CBG. The described methods should allow an assessment of the risk of neuroembolism associated with different types of CBG and may also be useful in other contexts."

CWD Canada: disease may wipe out elk industry

Saturday 12 August 2000 Colette Derworiz, Calgary Herald
The discovery of Saskatchewan's sixth case of a deadly disease in elk may trigger an end to the elk industry in Alberta and the rest of Canada, says one of the country's leading experts on the disease.

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

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

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

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

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

Minister of Agriculture Ty Lund said it is unlikely the elk industry will be outlawed, since it is an important part of diversified agriculture in the province. The antlers and its velvet -- used to make pain remedies for arthritis and aphrodisiacs -- are a $52.5-million industry in Alberta. The herd value on Alberta farms is worth another $101 million.

"But at the same time, we are not prepared to jeopardize livestock," he said. "The health of the animals is one of the primary interests we have in the livestock industry." He said, however, more study on the disease is needed before commenting on its long term effect to the elk industry.

Producers say the destruction of the entire herd was the proper channel for the federal government to take, said Ian Thorleifson, executive director of the Canadian Venison Council, based in Edmonton. He said the disease is a threat to other elk and deer raised on game farms, but was caught before it infected other animals. "It was one source and all the animals at risk are quarantined, all the product at risk is quarantined," said Thorleifson.

Elk in Alberta are harvested primarily for their velvet and antlers, he said. "I don't think we will see any effect on the Alberta industry," Thorleifson said. "All industries, whether it is the oil industry or any other industry, have problems they have to deal with. We are no different."

200,000 patients received CJD-linked dura mater

Kyodo World Service  Fri, Aug 11, 2000
Some 200,000 patients in Japan received transplants from 1973 to 1997 of German-made dura mater that has been reported to cause Creutzfeldt-Jakob Disease (CJD), a fatal brain disorder, the Health and Welfare Ministry said Friday.

The ministry told the House of Representatives the same day that there was no way for it to know at the time that the dura mater -- the fibrous membrane surrounding the brain and spinal cord -- could cause the disease. Lawyers representing a group of CJD patients have claimed that the ministry delayed issuing a ban on the use of the imported dura mater despite knowing for years of a link between it and CJD.

Seventy-two of the patients who received transplants of the dura mater, brand name Lyodura, have been found to have contracted CJD, ministry officials said. Symptoms of the disease are rapidly progressive dementia, loss of cerebral functions, paralysis of limbs, with parts of the brain becoming sponge-like.

The ministry first approved imports of Lyodura from the German medical equipment manufacturer B. Braun Melsungen in 1973, the officials said. The dura mater was processed by the company for use in transplants after being extracted from human corpses. [The company also employed a an orthopedic surgeon to remove dura mater from sheep. This may have been used in humans. He later died of CJD. -- webmaster]

According to the officials, a Tokyo company, Nihon B.S.S., imported 400,000 to 500,000 pieces of Lyodura from 1973 to March 1997, when the ministry ordered the importer to recall the dura mater. The officials said about 20,000 pieces were used by doctors each year during the period. About 200,000 patients received transplants of dura mater that had not been completely sterilized, they said.

Health ministry officials say the use of such dura mater does not necessarily cause patients to contract CJD because only one person in a million contracts the disease and the cause of the disease is unknown in 80% of these cases. [This confusion is with sporadic CJD; iatrogenic disease is under discussion. -- webmaster]

CJD patients and their families are calling on the government to make additional investigations to find out why the patients contracted the disease. A group of 44 people, including CJD patients, have filed compensation lawsuits in Tokyo and Shiga Prefecture against the ministry, Nihon B.S.S. and B. Braun Melsungen for failing to inform them of the risk of contracting the disease.

The officials said the ministry does not have immediate plans to launch an additional investigation, saying the rate of patients contracting the disease is extremely low and the number of patients who had dura mater transplanted is too large.

An internal ministry investigation revealed that the ministry in 1988 ignored a report compiled by a ministry research team warning that transplant patients who received imported dura mater could contract CJD. In February 1988, the head of the research unit submitted the report at a meeting attended by 20 experts and three ministry officials.

The report warned of a link between using imported dura mater and CJD, citing a 1987 U.S. report detailing the first-ever case of a transplant patient contracting CJD. The ministry finally issued a recall of dura mater products nine years later in March 1997, in effect banning the use of dura mater.

France expects tests to uncover many new BSE cases

     QUIMPER, France  - A plan to screen thousands of
French cattle for mad cow disease, or BSE, could uncover an additional 120
cases in the western tip of France alone by the end of the year, scientists
     Authorities have discovered three cases of the fatal brain-wasting
disease since they began testing 48,000 French cattle for mad cow disease
in June in a bid to measure the extent of the epidemic among the country's
21 million cattle. [This presumably means asymptomatic cows headed for the human food supply -- webmaster]

The three confirmed cases to emerge from the programme brought to 34 the number of cases reported in France this year, surpassing the 30 cases detected in 1999 as a whole, the French farm ministry said on Friday.

The bulk of the tests -- 40,000 -- will be carried out in the west of France, the cradle of cattle farming where the majority of cases of BSE (bovine spongiform encephalopathy) have been detected to date.

"We are going to find others, that is the point of the operation," said Andre Manfredi of the General Directorate of Food. "We hope to stand at between four and 120 cases of BSE on the 40,000 (tests planned for the west of France)."

After an initial six-week period, during which participants ranging from veterinarians to slaughterhouses were made familiar with the new procedures, the government started officially tallying results of the tests >from August 7.

Scientists are collecting between 50 and 130 samples per day in the Brittanny region, a rate reflected in neighbouring lower Normandy and Maine-et-Loire. The three regions together account for some 80 percent of the 114 cases of BSE reported in France since the epidemic was detected in 1991.
Testing in other parts of the country should start in late August or early September and the programme is expected to wrap up by the end of the year. Of the total, 12,000 tests correspond to the European Union's compulsory testing programme for cattle.
A similar programme in Switzerland in 1999, using the Prionics test that France has also selected, doubled the number of confirmed cases there to 50. But French scientists are reluctant to draw conclusions from that example because of vast differences in scale.

France remains locked in a legal battle with the European Commission over its refusal to end a ban on imports of British beef because of fears it is not entirely free from BSE.

Britain has reported more than 176,800 cases of BSE, making the French epidemic look small by comparison. But while the number of BSE cases in Britain is falling [actually, Britain still has many more cases than France even with no testing program -- webmaster], the French outbreak is widening despite measures introduced almost a decade ago to combat the spread of BSE through contaminated animal feed.

Many scientists believe BSE-infected meat products cause a new form of Creutzfeldt-Jakob disease, a similar brain-wasting disease that has killed more than 50 people in Britain.

TSE in hounds

Tue, 8 Aug 2000  BSE Inquiry document YB90/11.28/1.1  obtained by Terry S. Singeltary Sr.
37.Putative TSE in Hounds - work started 1990

Robert Higgins, a Veterinary Investigation Officer at Thirsk, had been working on a hound survey in 1990. Gerald Wells and I myself received histological sections from this survey along with the accompanying letter (YB90/11.28/1.1) dated November 1990.

This letter details spongiform changes found in brains from hunt hounds failing to keep up with the rest of the pack, along with the results of SAF [scrapie-associated fibrils] extractions from fresh brain material from these same animals. SAFs were not found in brains unless spongiform changes were also present.

The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord.

However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease.

I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.

I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on hound ataxia’ mirrored those in material from Robert Higgins’ hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him.

This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE.

Gerald Wells confirmed in blind’ examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.

Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal.

Circumstantial evidence suggests that bovine offal may also be causal in FSE in cats and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.

The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL.

I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.

The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.

Opinion (webmaster): It was politically unacceptable to find TSE in dogs. However, hunting dogs in particular received horrific exposure to terminal downer BSE cows, including skull and spinal column. The most interesting aspect is that hound ataxia, taken above as a proxy for dog TSE, goes back to the 1930's, the time of the louping ill vaccine accident causing tens of thousands of sheep to develop scrapie. Some of the dog cases could be due in fact to consumption of sheep scrapie.

USDA allows diseased animals into human food supply

Mon, 14 Aug 2000.  Information provided by Terry S. Singeltary Sr.
Farm Sanctuary web site
March 25, 1999
Kathryn Fugere
Goodin, MacBride, Squeri, Schlotz, & Ritchie
505 Sansome St., Suite 900
San Francisco, CA 94111

Dear Ms. Fugere,

FSIS reviewed the March 4, 1998, petition you submitted on behalf of 
your clients Farm Sanctuary and Michael Baur, asking the Food Safety 
and Inspection Service (FSIS) to amend the Federal meat inspection 
regulations to provide that all "downed" livestock be deemed adulterated 
or condemned and, as a result, removed from the food supply. FSIS has 
denied your request.

FSIS is not required under the Federal Food, Drug and Cosmetic Act 
(FFDCA) (21 U.S.C. 301 et seq.) or its regulations concerning the 
products of a diseased animal, to removed all downed cattle, without 
exception, from the nation's food supply since FSIS is not bound by the 
FFDCA's definition of adulteration (21 U.S.C. 342(a)(5)). By law, 
FSIS must apply the definition of adulteration found in
the Federal Meat Inspection Act (FMIA) (21 U.S.C. 601 et seq.) 
to food and food products from cattle, sheep, swine, goats, and equines. 
The definition of adulteration found in the FFDCA is different from that 
in the FMIA (compare 21 U.S.C. 342(a)(5) and 21 U.S.C. 601(m)(5) 
respectively). Unlike the FFDCA, the FMIA does not automatically 
consider the products of a diseased animal adulterated. Furthermore, 
under the FMIA, as long as an animal, even a diseased animal, 
depending upon the disease, has been passed for slaughter, 
it is possible that the carcass, or a portion of it, may be inspected 
and passed for human food.

It is obvious that the FFDCA and FMIA regulate different foods and have
different areas of concern. Case law has made it clear that the two 
statutes have independent construction and are not applicable to each 
other. In United States v. 2,116 Boxes of Boned Beef, 516 F. Supp. 321, 
344 (1981), the court up held the Department of Agriculture's long 
standing position that, "There is no requirement in the Federal Meat 
Inspection Act that any procedures prescribed in the Federal
Food, Drug and Cosmetic Act be followed in issuing regulations or 
taking other actions under the Federal Meat Inspection Act." 

The court further pointed out that regulations enacted under the FMIA 
are inapplicable in defining adulteration under the FFDCA. Similarly, 
definitions under the FFDCA are inapplicable to regulations
promulgated and interpreted under the FMIA. Thus, FSIS must apply 
the definition of adulteration found in the FMIA, not the FFDCA, 
to livestock brought into a federally inspected slaughter 

You suggest that 9 CFR 301 2(y) means that a non-ambulatory, i.e. 
downed, animal is a diseased animal. Section 301 2(y) clearly includes 
both diseased and disabled livestock. Some disabled or non-ambulatory 
animals are not diseased. Rather, they are affected by a physical 
condition (e.g., a broken leg) and may not be diseased. Such "downers" 
may not require any partial or complete condemnation. 

Any non-ambulatory or otherwise disabled livestock that are
suspected of being affected with a disease or condition which may 
require its condemnation, in whole or in part, are handled as 
"U.S. Suspects" (9 CFR 301.2(xxx)). The carcasses of "U.S. Suspects" 
are subjected to further examination by FSIS veterinary medical 
officers after slaughter to determine the appropriate disposition. 
If products made from the carcasses of non-ambulatory
animals do not pose a threat to human health, then there is no need to
automatically condemn the carcasses simply because they came from 
"downers". They can be examined, and, if part or all of the meat from 
them is safe for human consumption, then it can be used for human food.

The FMIA, FSIS regulations, and past practices clearly provide for the 
slaughter and processing of diseased animals for human food. Such 
animals may be slaughtered and examined to determine if the meat from 
their carcasses pose no threat to human health. If they post no threat, 
the meat may be passed for human food when the disease condition does 
not affect the whole carcass and the diseased part can be removed to 
make a wholesome product (21 U.S.C. 603, and 9 CFR 301.2(xxx), 309.2). 

Any livestock showing symptoms of certain diseases must be identified 
as "U.S. Condemned" and be disposed of according to sections
309.8, 309.13, or 311 (9 CFR 309.4(a)). Section 311 deals with disposal 
of diseased or otherwise adulterated carcasses or parts and states that 
the decision as to the disposal of any diseased items not specifically 
covered on part 311 shall be left to the veterinary medical officer, 
who is to exercise judgement regarding the disposition of all carcasses 
or parts to ensure that only wholesome, unadulterated product is passed 
for human food.

When an FSIS veterinary medical officer is presented with a cow that is 
unable to rise, a differential diagnosis of the etiology of the 
syndrome must be made. It is not difficult to distinguish a recumbent 
cow due to one of the acknowledged "downer cow" syndrome etiologies 
from one that is affected with a central nervous system
(CNS) condition. If proper clinical observations are combined with 
an adequate history and appropriate laboratory test evaluations, a 
differential diagnosis is possible in the vase majority of cases. 
[BSE cannot be diagnosed by visual inspection of a "recumbent" cow. -- webmaster]

Apparently, you assume that FSIS veterinary medical officers are 
unable to make a differential diagnosis of "downer cows." Such 
reasoning contravenes long established principles of veterinary
medicine: differential diagnosis and attribution of disease etiology. 
Moreover, regardless of the diagnosis, all animals with CNS disorders 
are condemned on antemortem inspection.

Your petition also stated that all downed cattle must be labeled 
adulterated or condemned and removed from the Nation's food supply 
because doing so is necessary to prevent the transmission of deadly 
diseases to humans, particularly bovine spongiform encephalopathy (BSE). 
However, the consensus of the scientific literature is that BSE does not 
exist in the U.S. BSE has not been detected in this country, despite 
active surveillance efforts for several years. Since 1990, nearly 6,500 
specimens, from animals in 43 states, have been laboratory
tested by an ongoing BSE surveillance system in the U.S. No evidence of 
BSE (in the form of characteristic lesions) or related transmissible 
spongiform encephalopathies (TSE) has been seen. In addition, the 
prevent BSE-contaminated animals or animal products from entering 
the U.S., severe restrictions exist on the importation of live 
ruminants and ruminant products from countries where BSE is
known to exist.

No cases of BSE have been diagnosed in the United States. In FY 1997,
137,663,099 livestock were slaughtered in USDA inspected establishments. 
Of those, 358,270 (0.002603 percent) were labeled "U.S. Suspect". Only 
827 red meat animals (0.000006 percent) were condemned on antemortem 
inspection for CNS condition. In FY 1998, 1,340 read meat animals 
(0.000001 percent) were similarly condemned. Regarding the other 
"serious diseases" listed in your petition that may pose a serious 
health threat to humans, none, including bovine leukemia virus,
bovine immunodeficiency virus, brucellosis, rabies, and Johne's disease, 
are considered to be meat borne zoonotic diseases. Thus, it would be 
extremely remote that any of those diseases would be passed on by 
consumption of red meat animals.

The U.S. has one of the most aggressive BSE surveillance programs in 
the world, one that is designed to keep the U.S. free of BSE. A United 
States Department of Agriculture (USDA) BSE Working Group, in 
coordination with other government, has been regularly reviewing the 
available science and implementing appropriate regulatory measures to 
prevent BSE. These measures include the 1989 ban on cattle and cattle 
products from countries where BSE has been reported and active
inspection, testing, and education programs. 

USDA has also entered into a cooperative agreement with Harvard 
University's School of Public Health to analyze and evaluate the 
Department's current measures to prevent BSE. The two-year study will 
review current scientific information, characterize the hazards of BSE 
and other TSE agents to human and animal health, assess the way BSE 
could potentially enter the U.S., and identify any additional measures 
that could be taken to protect human and animal health.

Finally, you state that "downed animals represent an extremely small 
percentage of all livestock slaughtered and banning their use would 
cause no undue economic hardship." This is incorrect. Condemning all 
meat and meat products from a carcass with any degree of disease would 
have a serious economic impact. Using your interpretation of the term 
"downer", that is, any diseased animal is a downer (based on the Federal 
Food, Drug and Cosmetic Act and its implementing regulations), there 
would be a substantial increase in the number of carcasses condemned. 
For example, a large percent of the livers of livestock (greater than 10
percent) are condemned because of disease conditions. Under your 
definition, a diseased liver would make an entire carcass adulterated. 
Such an interpretation would most certainly impact meat availability 
and prices.

Thank you for allowing FSIS the opportunity to respond to your petition 
and the questions it raised. You may contact Victoria Levine, Petition 
Manager, Regulations Development and Analysis Division, at 
(202) 720-5627, if you have any questions.

Daniel L Engeljohn, PhD.
Director, Regulations Development and Analysis Division
Office of Policy, Program Development and Evaluation
USDA, FSIS, Washington, DC 20250 
Comment (J. W. Wilson D.V.M.):   
I received my D.V.M. degree in 1942 and engaged in a predominantly dairy
practice for 25 years. I then worked for Wisconsin meat inspection for 7 
years. I feel that I am familiar with the down cattle phase from both 
sides. I am aware that down cows which do not readily respond to 
treatment are for the most part dying cows. They may die in a few hours 
but for the most part they are in the way and 
are disposed of to get rid of them.

These down cows are dragged out of the barn or pasture on to a truck and 
hauled to a slaughter plant where they are dragged from the truck to the 
kill floor. These cows live in a state of suspended animation, do not 
eat  and do not get better. If in lateral recumbency will stay that way 
and if helped up on their belly the head will flop down to the side. 
When slaughtered the lymph nodes do not usually show involvement.

During my work with Wisconsin meat inspection I spent quite some time 
working on the kill floor of two plants which more or less specialized 
in the slaughter and processing of down cattle. I concluded as a result 
of my working with this class of cattle that if these diseased and dying 
cattle were to be kept out of the food chain it would have to be on the 
basis of ante mortem inspection. The post mortem inspection is based on 
the response of the immune system to infection. It seems as
though these cows have their own version of AIDS.
To: Commissioner
U.S. Food and Drug Administration
Dockets Management Branch
Room 1-23
12420 Parklawn Drive
Rockville, MD 20857

From: Lester Friedlander, DVM

Subject: Docket Number 98P-0151/CP1 Disallowing the use of 
non-ambulatory animals for human food.

I urge the Food and Drug Administration to act on Docket Number 
98P-0151/CP1 and stop the slaughter of downed animals for the human 
food supply. I was a USDA, FSIS Veterinarian from January, 1985 to 
April, 1995.

I was the Supervisory Veterinary Medical Officer (SVMO) for the 
largest culled cow slaughter in the United States. I have literally 
seen thousands of non-ambulatory cattle (Downers), being slaughtered, 
which in my professional judgement should have been condemned.

Due to the very liberal policy on non-ambulatory animals and the 
ineffective inspection process, they are generally passed to be 
slaughtered. USDA-FSIS does not use any scientific or microbiological 
testing to determine their true condition.

If these downers pass for human food they are ground up for the federal 
school lunch program or often sold to fast food restaurants. In either 
event our children's health is jeopardized.

The USDA claims that animals with Central Nervous System (CNS) are kept 
out of the food supply. As somebody who has trained close to one hundred 
(100) veterinarians in the USDA and received the veterinary trainer of 
the year award, I can tell you that CNS animals are not carefully 
examined nor prevented from entering the food supply.

In the absence of a clear policy prohibiting the use of downer animals 
for human food, I am concerned that CNS and other potentially 
transmissible diseases will be passed on to human consumers.
April 22, 1997 

Dockets Management Branch (HFA-305) 
Food and Drug Administration 
12420 Parklawn Dr. 
Rm. 1-23 
Rockville, MD 20857

RE: Proposed Rule to Ban Substances in Animal Food [Docket No. 96N-0135]

Dear Madam or Sir:

I am writing on behalf of Farm Sanctuary, a national non-profit 
organization which works to stop irresponsible agricultural practices.

While we commend the Food and Drug Administration (FDA) for addressing 
the risk of transmissible spongiform encephalopathies (TSEs) spreading 
in the U.S., we write today because we believe the agency is taking 
inadequate action to ameliorate this risk.


As we have previously stated, we believe it is important to rethink the 
efficacy of using rendered products in poultry feed, especially since 
poultry litter is often used in cattle feed. We believe the feed ban 
should apply to protein recycled through poultry and then fed back to 

We appreciate the agency's recent proposal to prohibit a broader range 
of animal proteins from being fed to ruminants. However, we do not 
believe there is adequate knowledge to justify the agency's excluding 
"blood and blood products" or "porcine protein" from the ban. 
A Texas A & M study found that prions are spread from the brain into 
the bloodstream during mechanical slaughtering procedures. We also 
oppose excluding "porcine protein" from this ban, especially in
light of Dr. Masuo Doi's newly rediscovered study of hogs at an 
Albany, NY slaughterhouse between 1978 and 1980.

Dr. Doi noticed behavioral symptoms indicating central nervous system 
(CNS) disorders in slaughterbound hogs, and so he extended his usual 
two day ante-mortem observation period to three or four days. Based 
upon his extended examinations, Doi retained over 100 pigs on 
ante-mortem inspection. Laboratory analysis of pigs retained by Doi 
raises a very real possibility that U.S. hogs may harbor a TSE of their 
own. A USDA lab report of one of Doi's hogs (November, 1979) noted:

"Microscopic examination of the barrow tissues revealed an 
encephalopathy and diffuse gliosis characterized by vacuolated neurons, 
loss of neurons and gliosis in a confined region (nucleus) of the brain 
stem (anterior ventral midbrain). Only an empty sometimes divided 
vacuole was present instead of the normal morphology of a nerve cell. 
Occasionally a shriveled neuron was seen. According to ...Pathology of 
Domestic Animals,...'The degeneration of neurons, the reactivity of 
glia...are the classical hallmarks of viral infection of the central 
nervous system'...Scrapie of sheep, and encephalopathy of mink, 
according to the literature, all produce focal vacuolation of the 
neurons similar to the kind as described for this pig." 

There is no reference to BSE (bovine spongiform encephalopathy or 
"Mad Cow Disease") in this report, because BSE was not discovered 
until 1986.

Insufficient information is available to combat TSE in the U.S.:

We believe that current surveillance efforts, including slaughterhouse 
inspection procedures, are inadequate to detect CNS disease in farm 
animals. This belief is supported by Dr. Doi's experience.

When Doi extended his ante-mortem observation period to look more 
carefully for CNS symptoms in pigs, the condemnation rate for CNS rose 
dramatically. In fact, the slaughterhouse where Doi conducted his 
observation accounted for more than 70% of all pigs condemned for CNS 
disease throughout the U.S. during the same time period.

In addition to pigs, other farm animals entering the human food supply 
may be afflicted with CNS diseases. I have personally visited several 
slaughterhouses which slaughter downed animals (i.e. animals too sick 
to stand), and I have been struck by the minimal inspection at these 

Despite the fact that downed animals are considered "suspect" and thus 
subject to additional observation, ante-mortem inspection of downed 
animals commonly takes less than 10 minutes. It would be very difficult 
to identify CNS symptoms in this amount of time, especially since a 
downed animal's state of immobility makes identifying CNS symptoms even 
more difficult.

Every year, tens of thousands of downed cattle, along with countless 
incapacitated pigs and other farm animals, are slaughtered for human 
food at U.S. slaughterhouses. These animals are not adequately examined 
for CNS disease. The reason for the animals' debilitated condition is 
typically unknown, and we are gravely concerned that some of these 
animals may be afflicted with a TSE.

If TSE infected animals are being killed and used for human food in the 
U.S., the consequences could be devastating. We cannot afford to take 
this risk. Thank you very much for you concern and thoughtful attention to this 

Gene Bauston, 
Executive Director 
Farm Sanctuary, Inc.

In Confidence - Perceptions of unconventional slow virus diseases of 
animals in the USA - Report of a visit to the USA - April-May 1989 - G A H Wells [head of England's main veterinary  lab -- webmaster]

2. Meeting with USDA, BSE Task Force

This group comprises Alex Thierman (USDA-APHIS, International Programs)
(Chairman), Roger Breeze (USDA-ARS Director, Plum Island), Bill Hadlow
(Neuropathologist - retired, formerly of NIH Rocky Mountain Laboratory, 
Hamilton, Montana), John Gorhan and Mark Robinson (USDA-ARS, Pullman, 
Washington) and Dick Marsh (Dept. Vet. Science, Univ Wisconsin - 

The objectives of the group are to assess the implications of 
the occurrence of BSE for US cattle particularly the risk 
of BSE occurrence in the US in relation to endemic scrapie agent.
The purpose of my invitation to this meeting was to discuss aspects of
research which are of common interest and to identify a tentative USDA
research programme including any potential collaborative projects. The
discussions were informal and there was no agenda. 

The general opinion
of those present was that BSE, as an overt disease phenomenon, could
exist in the USA but if it did it was very rare. The need for improved
and specific surveillance methods to detect it was recognised. Clinical
similarities between BSE and rabies suggested one means of sampling the
cattle population which might increase the probability of detection of
BSE. It was clear that the bovine rabies negative rate would vary
greatly between States but initially this should be determined and as it
would inevitably be high relative to positive cases, some differential
diagnosis carried out.

The work of Wilbur Clarke at Mission, Texas was discussed briefly. the
results of this study in which 10 calves were inoculated with scrapie
has not been published and perhaps would not be published. USDA are
sensitive regarding publicity of the results of the study which remain
far from conclusive. Apparently only 3 of the inoculated animals
developed neurological signs. The neuropathology of the affected cattle
has not been examined in any depth but Hadlow has the material. Marsh
indicated the requirement to obtain the fresh brain material from this
study in order to perform PrP extractions.

Because of the successful transmission of the Brecke (Stetsonville)
isolate of TME to cattle and the subsequent passage history in mink it
was generally considered important that comparisons be made with BSE
isolates in mink. Is BSE like scrapie in mink? Is BSE like the Brecke
isolate of TME?

Very little was said about CWD but some present considered that its
occurrence may indicate a sylvatic origin of agent. It was also agreed
that the role of possible subclinical infection in the
epidemiology of transmissible spongiform encephalopathies could well
be important but was unknown. 

Marsh remarked on the possibility that
BSE was due to an extremely thermostable strain of agent. His
experience in the past with one particular Wisconsin isolate of TME
(Hayward strain) suggested that i/c biopsy needles could not be
effectively "scrapie sterilised", even employing an experimental
autoclave system capable of 60 psi and 300"C+ for 5 hours. This
experience led him to the policy that in scrapie or TME transmission
studies re-use of instruments or glassware that had contained agent was
an unacceptable protocol.

I was given confidential access to sections from the Clarke scrapie- 
cattle transmission experiment. Details of the experimental design were 
as supplied previously by Dr Wrathall (copy of relevant information
appended). Only 3 animals (2 inoculated with 2nd pass Suffolk Scrapie
and 1 inoculated with Angora goat passaged scrapie) show clinical signs.

Clinical signs were characterised by weakness, 
"a stilted hindlimb gait", disorientation, ataxia and, terminally,
lateral recumbency. The two cattle from which I examined material were
inoculated at 8 months of age and developed signs 36 months pi (goat
scrapie inoculum) and 49 months pi (one of the Suffolk scrapie
inoculated) respectively. 

This latter animal was killed at 58 months
of age and so the clinical duration was only 1 month. The neuropathology
was somewhat different from BSE or the Stetsonville TME in cattle.
Vacuolar changes were minimal, to the extent that detection required
careful searching. Conversely astrocyte hypertrophy was a widespread and
prominent feature. The material requires detailed neuropathological
assessment but whether or not this will be done remains in question.

Appendix I


1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has
successfully transmitted ovine and caprine scrapie to cattle. The
experimental results have not been published but there are plans to do
this. This work was initiated in 1978. A summary of it is:-

Expt A 6 Her x Jer calves born in 1978 were inoculated as follows with a
2nd Suffolk scrapie passage:-

i/c 1ml i/m, 5ml; s/c 5ml; oral 30ml.

1/6 went down after 48 months with a scrapie/BSE-like disease.

Expt B 6 Her or Jer or HxJ calves were inoculated with angora Goat virus
2/6 went down similarly after 36 months.

Expt C Mice inoculated from brains of calves/cattle in expts A & B 
were resistant, only 1/20 going down with scrapie and this was the 
reason given for not publishing.

Diagnosis in A, B, C was by histopath. No reports on SAF were given.
Dr Warren Foote indicated success so far in eliminating scrapie in
offspring from experimentally (and naturally) infected sheep by ET. He
had found difficulty in obtaining emhryos from naturally infected sheep (cf SPA).

3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr A Thiermann showed the picture
in the "Independent" with cattle being incinerated and thought this was
a fanatical incident to be avoided in the US at all costs. BSE was not
reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increase
since 1978. In 1953 when the National Control Scheme was started 
there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitary fetus.
6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of
the national sheep scrapie programme survey indicated;

   17/33 wished to drop it
   6/33 wished to develop it
   8/33 had few sheep and were neutral

Information obtained from Dr Wrathall's notes of a meeting of the U.S.
Animal Health Association at Little Rock, Arkansas Nov. 1988.

6.1 BSE to pigs
SE1802/SE1816 - Transmissibility of BSE to pigs by injection with brain homogenate;
SE1803/SE1817 - Transmissibility of BSE to pigs by oral exposure with brain homogenate.

6.1.3 The possibility that pigs could be recycling the BSE agent through
rendered material, which was then passed back to cattle via the
spreading of pig manure, was discussed. However, it was agreed that no
action was required (see para. 2.1.3).

6.2 BSE to chickens
SE1805/SE1806 - Transmissibility of BSE to domestic fowl

6.2.1 Material from a number of tissues including spleen in the
experimental chicken should be sub-passaged through further birds and
mice: by the intracerebral routes in birds and the intracerebral route
in mice. CSG would ask CVL to prepare a proposal.

6.3 Scrapie to pigs  SE1813/SE1822 - Transmissibility of scrapie to pigs by oral exposure to brain homogenate.

6.3.1 SE1822 will replace SE1813. No amendment of this project was required.

6.4 BSE to sheep
SE1402/SE1418 - Transmission of BSE and natural scrapie in sheep and goats by intracerebral and oral routes

6.5 BSE to cattle via exposure to placenta


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