Document Directory

21 Mar 01 - CJD - The Queniborough Report
21 Mar 01 - CJD - The Queniborough PowerPoint Presentation
21 Mar 01 - CJD - CJD report: Reaction - Lacey rubbishes report
21 Mar 01 - CJD - Too soon to tell scale of human BSE deaths -scientist
21 Mar 01 - CJD - Old just as vulnerable to CJD as young
21 Mar 01 - CJD - Study: Older People Can Get Mad Cow
21 Mar 01 - CJD - Supermarket to test for BSE
21 Mar 01 - CJD - CJD cluster deaths linked to butchery
21 Mar 01 - CJD - Cause found for BSE hotspot
21 Mar 01 - CJD - Traditional methods 'caused vCJD deaths'
21 Mar 01 - CJD - vCJD: the tragedy of lessons unlearned
21 Mar 01 - CJD - CJD deaths due to 'traditional' butchers
21 Mar 01 - CJD - Butchers' knives link to village disease deaths
21 Mar 01 - CJD - 'Contaminated meat to blame for CJD deaths'
21 Mar 01 - CJD - Slaughter and butchery practices to blame for CJD deaths
21 Mar 01 - CJD - Cattle Brains From Bse Cows 'Caused vCJD Deaths'
21 Mar 01 - CJD - Report on "Mad Cow" death cluster eyes butchers
21 Mar 01 - CJD - Butcher welcomes CJD report
21 Mar 01 - CJD - Mad Sheep: Federal Government Arrives to Take Sheep Suspected of Disease
21 Mar 01 - CJD - U.S. seizes sheep with possible strain of Mad Cow disease
21 Mar 01 - CJD - Mad Cow In The U.S.?
21 Mar 01 - CJD - Mad Sheep Scare In Vermont
21 Mar 01 - CJD - U.S. seizes sheep with possible strain of Mad Cow disease
21 Mar 01 - CJD - Vermont Sheep May Have Mad Cow
21 Mar 01 - CJD - We have to learn CJD lessons, says butcher
21 Mar 01 - CJD - U.S. Remains Free of BSE And FMD
21 Mar 01 - CJD - BSE and the sheep disease scrapie have similar symptoms
21 Mar 01 - CJD - CJD report 'won't help me'
21 Mar 01 - CJD - Italian farmers seek government help
21 Mar 01 - CJD - U.S. supplement firms pledge anti-Mad Cow steps
21 Mar 01 - CJD - DG Bank struck by Mad Cow disease
21 Mar 01 - CJD - BSE report published



21 Mar 01 - CJD - The Queniborough Report

MAFF

MAFF---Wednesday 21 March 2001


SUMMARY OF THE FINAL REPORT OF THE INVESTIGATION INTO THE NORTH LEICESTERSHIRE CLUSTER OF VARIANT CREUTZFELDT-JAKOB DISEASE

The investigation was carried out by Dr Gerry Bryant and Dr Philip Monk who have prepared and present this report to Leicestershire Health Authority.

INTRODUCTION

The cluster of variant Creutzfeld-Jakob disease in the North Leicestershire area was first recognised in July 2000. Between August 1996 and January 1999 five people developed symptoms that were later recognised as being those of variant Creutzfeld-Jakob disease and they have all died. They all lived in the Wreake and Soar Valley area in North Leicestershire from 1980 until 1991.

The investigation concentrated on the period from 1980 to 1991, as this was the only time period when a common exposure could have occurred.

INITIAL FIELDWORK

At least one relative of the people with variant Creutzfeld-Jakob disease was interviewed to examine possible exposures to the BSE (Bovine Spongiform Encephalopathy) Agent.

A questionnaire study was carried out with parents of children at Queniborough Primary School to examine their purchase and consumption of food when they were children themselves during the 1980s.

This was followed by a questionnaire study of people living in the village of Queniborough. The questionnaire asked those people who were parents of children of similar age to the cases about their purchase and consumption of meat during the 1980s.

Butchers, farmers, auctioneers and others involved in the meat trade were interviewed to build a picture of the dairy and beef industries during the 1980s.

RESULTS FROM THIS INITIAL FIELDWORK

Possible Exposures

A number of possible risk factors were unlikely to provide an explanation for a link between the people with variant Creutzfeld-Jakob disease. They were: -

Surgery and blood transfusions

Dental surgery

Occupational exposure

Immunisations

Injections, body piercing, cuts and animal bites

Baby foods, school meals and drinking water

Manganese (data from the British Geological Survey showed that the area of Leicestershire does not have a high level of Manganese in the soil)

INVESTIGATION INTO FARMING PRACTICE

Cattle raising practice

Local beef cattle were raised alongside dairy cattle. This meant that beef cattle were fed meat and bone meal supplements from the age of 6 days rather than 6 months, which is the case for pure beef herds. They therefore had a greater lifetime exposure to the BSE agent in meat and bone meal than cattle who did not receive meat and bone meal until the age of 6 months.

The area of Leicestershire that supplied beef cattle to the local food trade had a moderately high incidence for BSE meaning that a number of cattle across a number of farms had the disease.

At the beginning of the 1980s, local beef cattle were a by-product of the dairy industry and were usually Friesian Hereford crossbred cattle. Such cattle were usually slaughtered between 30 and 36 months of age because they are slower to fatten.

The average age for onset of BSE in cattle was between 4 and 5 years. However, the BSE Inquiry notes that although the numbers were small, there were a few cases in which clinical onset occurred between 20 and 30 months. The youngest animal in England being just 20 months old. This means that the older Friesian cross bred cattle used in the meat trade in Leicestershire were more likely to have sub-clinical BSE infection and to be infectious. Back calculations from the BSE epidemic suggest that British cattle must have had BSE from the mid 1970s onwards. This area of Leicestershire reported BSE as soon as it became notifiable in 1988 which means that some cattle were likely to have had BSE during the period that we were investigating.

Cattle slaughtering practice

In both large and small abattoirs, cattle were slaughtered using a captive bolt. In the local abattoirs and butchers who slaughtered, a pithing rod was also used. (see appendix - terminology) In small abattoirs the carcass was wiped down with a cloth to remove unwanted tissue. In large abattoirs the carcass would be hosed down. In the early 1980s there was no legal requirement to hose down a carcass. Skilled butchers reported that hosing a carcass down would make the meat go 'sour'. The practice of wiping a carcass with a cloth meant that there was a possibility of cross contamination of meat with brain and nervous tissue in those butchers who removed the brain from a beast's head.

Carcass purchase

Most butchers in the area bought meat from wholesale suppliers. A small number would select cattle at Melton Market or directly from a local farm for slaughter either by themselves or in a small nearby abattoir.

Wholesale meat suppliers purchased carcasses from a number of abattoirs that in turn selected cattle from a number of cattle auctions.

RESULTS OF INVESTIGATION INTO BUTCHERING AND MEAT PROCESSING

Butchering practice

Whole carcass processing

A small number of butchers either slaughtered beasts in their own small abattoirs or had beasts slaughtered by a nearby small abattoir. The butcher then processed the whole carcass. For those butchers who had a market for brain, they removed it from the beast during the process of recovery of head meat. The rest of the carcass was then boned and jointed. The removal of brain meant that there was the possibility that other meat could be contaminated with brain material. Brain contains the BSE agent and is therefore potentially infectious. During the 1980s, this process was legal and represented traditional butchering practice. It was decreasing because of a declining consumer market for brain. In the past and in particular during the war years, brain had been seen as an excellent source of protein.

Wholesale purchase

By the beginning of the 1980s many butchers had moved to purchasing either sides of beef, quarters of beef or vacuum packed pre-prepared cuts of beef rather than whole carcasses. A small number of these butchers would also purchase heads in order to remove the tongues to prepare for pressed tongue and sometimes the cheek meat usually for pet food. It was very rare for such butchers to remove the brain, as by this time there was often no market for brain.

THE HYPOTHESIS

Our initial work suggested that there was an association between the cases of variant Creutzfeld-Jakob Disease and the consumption of beef purchased from butchers where there was a risk of 'cross-contamination' of beef carcass meat with bovine brain.

The essential elements of our hypothesis are that:-

the beasts used were locally reared cattle

the beef cattle were a by-product of the beef industry and therefore fed meat and bone meal from day 6 onwards giving them a greater lifetime exposure to feedstuff that was potentially contaminated with the BSE agent

they were predominantly Friesian crossbred cattle which were slow to fatten and therefore slaughtered at close to three years

they were slaughtered in small abattoirs which employed pithing and without the washing down of the carcass

the heads were split to remove the brain

during brain removal, if the meninges (the membrane that covers the brain) are broken, because brain is of a gelatinous consistency, when handled, it then has a tendency to be adherent carcasses were wiped with cloths increasing the risk of cross-contamination

TESTING THE HYPOTHESIS

Our study to test the hypothesis was carried out with the approval of the Leicestershire Research Ethics Committee.

A relative of each case variant Creutzfeld-Jakob Disease in the cluster was re-interviewed using a structured questionnaire. The questionnaire asked about dietary history including meat consumption and from where meat was purchased during the period 1980 to 1991. A relative of each of thirty age-matched controls, six for each case, was interviewed using the same questionnaire. An attempt was made to interview all butchers, supermarkets and freezer food centres identified by the controls to ascertain whether they or their suppliers used cattle heads and removed bovine brain, thus creating the opportunity for cross-contamination.

RESULTS OF THE CASE CONTROL STUDY

Butchers, supermarkets and freezer food centres used by cases and controls

Four of the people who developed variant Creutzfeld-Jakob Disease bought and consumed beef from one of two butchers during the early 1980s. One of these butchers slaughtered beasts in his own abattoir. This butcher normally processed three beasts a week. He ceased trading in 1989. The other butcher had beasts slaughtered in a small nearby abattoir. He processed four to five beasts a week. This butcher's business ceased trading in December 1982. It has not been possible to trace the butcher who was used regularly by one family during the first half of the 1980s. It is unlikely that he removed brains or even purchased the heads of beasts. He did not slaughter beasts himself or use a small local abattoir.

People acting as controls used a total of twenty butchers, four freezer food centres and seven supermarkets. With the exception of one butcher who supplied one of the controls, all other outlets were traced and staff interviewed to ask whether they removed the brain. Three butchers used by controls removed brains. One of these butchers was also used by one of the people who developed variant Creutzfeld-Jakob Disease. With the exception of this one butcher and one other who also split the heads, the butchers used by the controls processed between one and two sides of beef or less a week. A side of beef is half of the carcass. We were able to trace both butchers and buyers who worked for the supermarkets used by people in this study. None of them reported the use of head meat during the 1980s. Sides of meat or vacuum packs were purchased from wholesalers. The wholesalers who supplied the supermarkets and freezer centres did not split heads to remove the brains. The skulls were sent either to specialist head boning plants or to renderers after removal of the head meat. The skulls were never split.

Interview results

The study showed that the relatives reported that the people with variant Creutzfeld-Jakob Disease were 15 times more likely to have purchased and consumed beef from a butcher who removed the brain from a beast compared with controls who purchased meat from outlets where cross contamination with brain material was not a risk. This result is statistically significant and is therefore very unlikely to be a chance finding. (p = 0.0058 and the 95% confidence interval is 1.6 to 138.9)

LIMITATIONS OF THE STUDY

A number of factors may have influenced the result.

Our work suggests that family size, age and education of children of controls were representative of people living in the area meaning that our controls who were randomly selected are likely to be representative of people living in the area.

It is possible that we were in some way able to influence the outcome of the study by the way in which we wrote down the answers to the questions that we asked both of the cases and controls. Wherever possible we interviewed people together to ensure that the interview technique was the same. All interviews were recorded on tape. Whilst we knew the hypothesis that was being tested, at the time of the interviews we had no knowledge of the butchering practices of most of the meat suppliers identified by the controls. These butchers were interviewed after the interviews with controls had been completed.

People may not be able to remember what they gave their families to eat twenty years ago. However, our hypothesis was dependent on where people shopped and we were not attempting to identify every single item of food that was eaten. Rather we explored the usual patterns of meat consumption and the usual sources where beef was purchased.

CONCLUSIONS FROM THE STUDY

We have found an association which provides a biologically plausible explanation suggesting that four out of the five people with variant Creutzfeld-Jakob Disease may have been exposed to the BSE agent through the purchase and consumption of beef from a butcher's shop where the meat could be contaminated with brain tissue. On a national basis, it is unlikely to explain how all of the people who have developed this disease were exposed to the BSE agent.

Assuming that we are correct in our explanation, we have shown that for one of the butchers, the exposure took place before December 1982. For the other the risk of the exposure continued until that butcher ceased trading in 1989. Analysis of the exposure of our cases to this butchering practice points to an incubation period for the development of variant Creutzfeld-Jakob Disease of between ten and sixteen years. This is the first time that it has been possible to provide an estimate of the incubation period.

We have shown that it is possible to examine by traditional epidemiological methods exposures that took place twenty years ago.

RECOMMENDATIONS

The explanation that we have found needs to be tested for other people who have died of variant Creutzfeld-Jakob Disease.

Surveillance for variant Creutzfeld-Jakob Disease needs to be maintained in Leicestershire.

THANKS

We would like to pay tribute to the courage of the families of those people who died of variant Creutzfeld-Jakob Disease who through their willingness to relive their pain have helped us to learn more about the way in which this disease spread from animals to people.

We thank all of those who willingly gave of their time to be interviewed as controls together with the butchers, farmers, auctioneers and others in the food trade who helped us with our study.

We would also like to thank all of those who provided us with help and advice in developing our study. In particular we would like to thank

Professor Paul Burton, Professor of Genetic Epidemiology, University of Leicester for advice and help with the statistical analysis of the study.

Professor Bob Will and Dr Hester Ward from the National Creutzfeld-Jakob Surveillance Unit.

Dr Roland Salmon and Dr Martin Wale from the Communicable Diseases Surveillance Centre of the Public Health Laboratory Service together with Dr Susan Hahne EPIET fellow, CDSC Wales.

Colleagues from

University of Leicester Department of Public Health

Department of Health

Ministry of Agriculture Fisheries and Food

Food Standards Agency

London School of Hygiene and Tropical Medicine

Local Authorities

We would also like to thank the following without whose help and support we would not have been able to do this work.

The clinical nurse specialists in the Communicable Disease Unit at Leicestershire Health Authority who through their dedication and support enabled us to have the time to carry out this study.

Our families who have put up with our frequent absences from home and long hours of work whilst we completed this study.

Gerry Bryant and Philip Monk

Leicestershire Health Authority 17 March 2001

BACKGROUND INFORMATION

INVESTIGATION OF VARIANT CREUTZFELD-JAKOB DISEASE

All people with variant Creutzfeld-Jakob disease are reported to the National Creutzfeld-Jakob Disease Surveillance Unit, which is based in Edinburgh. Doctors from the unit visit all people with the disease. A detailed history is taken and the diagnosis reviewed.

MEAT AND BONE MEAL

meat and bone meal is a protein supplement fed to cattle. It was made from rendering the parts of sheep, pigs, chickens and cattle that were not consumed by people.

As little as I gram of infected material when fed to cattle is known to cause BSE in 70% of those animals fed the infected material.

In December 1988 it was recognised that the likely way in which BSE was spread was through feeding cattle protein supplements containing meat and bone meal. In November 1989, through a voluntary ban, Animal food manufacturers stopped the inclusion of any Specified Bovine Offal in meat and bone meal fed to ruminants. This voluntary ban was made law in September 1990 in the Bovine Spongiform Encephalopathy (No 2) Amendment Order 1990.

TERMINOLOGY

BSE Bovine Spongiform Encephalopathy

Captive bolt stunner

An instrument for stunning animals before slaughter, powered by a cartridge or compressed air which drives a bolt out of a barrel for some four inches and then retracts it into the barrel.

Pithing

Insertion of a rod through the stun hole in the head of cattle to prevent the animal kicking (a reflex action which sometimes occurs after stunning)


21 Mar 01 - CJD - The Queniborough PowerPoint Presentation

MAFF

MAFF---Wednesday 21 March 2001


Queniborough PowerPoint presentation as presented to Leicestershire health Authority:

Click here


21 Mar 01 - CJD - CJD report: Reaction - Lacey rubbishes report

Staff Reporter

BBC--Wednesday 21 March 2001


Professor Lacey says nobody knows how disease spreads from cows to humans

The microbiologist who first raised the possibility of a link between BSE in cows and CJD in humans says scientists have no idea what caused the cluster of cases in Leicestershire.

The official report into the deaths of five people from variant CJD in the village of Queniborough, released on Wednesday, has put part of the blame on the way butchers in the area prepared meat in the1980s.

However, Professor Richard Lacey, who is based at Leeds University, told the BBC Today programme that it was not possible to draw firm conclusions about the spread of the deadly disease.

He said the report, from Leicester Health Authority, was simply searching for a scapegoat.

'No idea'

"They have no idea, it is just guess work, speculation. The aim is to reassure, rather than to get at the truth.

"This has been the whole basis of CJD over 15 years - not to get at the truth, but to reassure in the short term."

Professor Lacey said nobody knew when humans were first exposed to infection, if there was a single dose or many sources.

Neither did scientists have any detailed knowledge about how the disease spreads from cows to people.

Laboratory tests on mice had shown that it was difficult to transmit the infection by taking it in through the mouth.

"It is not clear exactly how it spreads, it could be more than one way."

'Important study'

Professor Hugh Pennington, an expert in BSE from Aberdeen University, agreed that the study had to be speculative because it was looking at events which took place many years ago.

But he said: "It is an important study. It is only by studying clusters that we can look for factors in common that all the unfortunate victims shared."

Professor Pennington said some of the butchery practices highlighted in Queniborough were widespread at the time across the country.

He said: "It does raise the question of why Queniborough and why young people?

"It still leaves many questions unanswered."

Government response

Dr Pat Troop, Deputy Chief Medical Officer for England, said the report had not proven any cause, but had provided significant evidence of a possible route of transmission.

She said the data would be analysed by the government's expert advisory committee on CJD.

She told the BBC: "We will ask them a number of questions. Does this help in our understanding of the cause of the disease? What will it tell us about the course of the epidemic? How many patients might we expect in the future? And also whether or not any similar work might be carried out elsewhere in the country?"

Dr Troop said the Food Standards Agency had advised that the practice of removing brains from slaughtered cows - highlighted in the Queniborough report - was not particularly common.

But she said: "We do work on the basis that we could have a very large epidemic."


21 Mar 01 - CJD - Too soon to tell scale of human BSE deaths -scientist

Reuters

YAHOO--Wednesday 21 March 2001


PARIS (Reuters) - It is too early to say how many people will die from the human form of Mad Cow disease, the scientist who won a Nobel prize for discovering the agent that causes the fatal brain-wasting illness said on Wednesday.

Stanley Prusiner, who won the award in 1997 for concluding that a distorted protein called a prion was responsible for causing Mad Cow disease, noted the long incubation period made forecasts difficult.

"It is too soon to have even the slightest idea of how the human epidemic will evolve," Prusiner told French daily Le Monde in an interview.

"The first cases were described in 1996 but we don't know if there were several cases that went unnoticed in previous years," he said. "Five years have passed since 1996, which is a small duration of time compared to that of the potential incubation period. All forecasts are hazardous."

Prions have mystified scientists since Prusiner discovered them. Normally present in the brain and other tissue, they can take on an abnormally folded form that causes the brain to become spongy and, eventually, to wither.

They can replicate, clump up and cause disease without the use of any kind of genetic material at all, unlike viruses, bacteria and parasites.

They are blamed for a range of diseases known as transmissible spongiform encephalopathies (TSEs), including Mad Cow disease, scrapie in sheep, Creutzfeldt Jakob disease (CJD) in humans, and a new kind of CJD -- new variant CJD (vCJD)-- that is caused by eating mad-cow-infected beef.

Prusiner said it was unclear how many prions were needed to infect humans and that there were probably different risk levels associated with transmitting prions.

"Oral contamination is surely less efficient than direct infection of the brain, as could happen during neurosurgery," he said.

According to official data, 90 people in Britain and two in France have died or are believed to have died from vCJD. Another five people in Britain and one person in France are believed to be dying from the illness.


21 Mar 01 - CJD - Old just as vulnerable to CJD as young

Staff Reporter

International Herald Tribune--Wednesday 21 March 2001


A study in monkeys suggests that older individuals are just as susceptible as young individuals to contracting Mad Cow disease, a brain-wasting illness.

The study, appearing Tuesday in the Proceedings of the National Academy of Sciences, involved injecting monkeys with the new variant of Creutzfeldt-Jakob disease, also known as vCJD; waiting for the animals to develop disease symptoms, and then performing autopsies on their brains.

Older monkeys, the researchers found, developed brain plaques and disease just as readily as the younger monkeys, although the disease was more severe in the younger animals.

"This is important with regard to the fact that vCJD has been diagnosed mainly in teenagers and young adults," the authors said in the report.

The research was conducted by scientists in France and Britain.

Dr. Paul Brown, an expert on the disease at the National Institutes of Health, said the discovery that the variant version can affect older people "is bad news."

"The study showed that young and old monkeys were equally susceptible," Dr. Brown said. "That strikes a warning note about infection in humans and the possibility that older people will become infected. It makes you worry more that over the course of time, older people will come down with the disease," he said.

The disease has been linked directly to eating meat from cattle infected with Bovine Spongiform Encephalopathy. At least 87 people in Europe have died of the variant version since the mid-1990s. It was first diagnosed in Britain. Cases also have been reported in France, Italy, Germany and Spain.

It is estimated that about 450,000 kilograms (1 million pounds) of contaminated beef may have entered the human food chain. Authors in the study estimate that this could result in up to 136,000 cases of the disease.

Because of a long disease incubation period, it may be years before the exact toll is determined. BSE is thought to have spread to cattle through the use of feed enriched with body parts from sheep infected with scrapie, a related disease. WASHINGTON A study in monkeys suggests that older individuals are just as susceptible as young individuals to contracting Mad Cow disease, a brain-wasting illness.

The study, appearing Tuesday in the Proceedings of the National Academy of Sciences, involved injecting monkeys with the new variant of Creutzfeldt-Jakob disease, also known as vCJD; waiting for the animals to develop disease symptoms, and then performing autopsies on their brains.

Older monkeys, the researchers found, developed brain plaques and disease just as readily as the younger monkeys, although the disease was more severe in the younger animals.

"This is important with regard to the fact that vCJD has been diagnosed mainly in teenagers and young adults," the authors said in the report.

The research was conducted by scientists in France and Britain.

Dr. Paul Brown, an expert on the disease at the National Institutes of Health, said the discovery that the variant version can affect older people "is bad news."

"The study showed that young and old monkeys were equally susceptible," Dr. Brown said. "That strikes a warning note about infection in humans and the possibility that older people will become infected. It makes you worry more that over the course of time, older people will come down with the disease," he said.

The disease has been linked directly to eating meat from cattle infected with Bovine Spongiform Encephalopathy. At least 87 people in Europe have died of the variant version since the mid-1990s. It was first diagnosed in Britain. Cases also have been reported in France, Italy, Germany and Spain.

It is estimated that about 450,000 kilograms (1 million pounds) of contaminated beef may have entered the human food chain. Authors in the study estimate that this could result in up to 136,000 cases of the disease.

Because of a long disease incubation period, it may be years before the exact toll is determined. BSE is thought to have spread to cattle through the use of feed enriched with body parts from sheep infected with scrapie, a related disease.


21 Mar 01 - CJD - Study: Older People Can Get Mad Cow

By Paul Recer, AP Science Writer

Austin360.com--Wednesday 21 March 2001


WASHINGTON (AP)--A study in monkeys suggests that older individuals are just as susceptible as the young to contracting Mad Cow disease.

The study, appearing Tuesday in the Proceedings of the National Academy of Sciences, involved injecting monkeys with new variant Creutzfeldt-Jakob disease, waiting for the animals to develop disease symptoms, and then performing autopsies on their brains.

Older monkeys, the researchers found, developed brain plaques and disease just as readily as the younger monkeys, although the disease was more severe in the younger animals.

``This is important with regard to the fact that vCJD (variant Creutzfeldt-Jakob disease) has been diagnosed mainly in teen-agers and young adults,'' the authors report.

The research was conducted by scientists in France and the United Kingdom.

Dr. Paul Brown, an expert on CJD at the National Institutes of Health, said the discovery that vCJD can affect older people ``is bad news.''

``The study showed that young and old monkeys were equally susceptible,'' said Brown. ``That strikes a warning note about infection in humans and the possibility that older people will become infected.

``It makes you worry more that over the course of time, older people will come down with the disease,'' he said.

Variant Creutzfeldt-Jakob disease, or vCJD, has been linked directly to eating meat from cattle infected with Bovine Spongiform Encephalopathy, which is commonly called ``Mad Cow disease.'' At least 80 people in Europe have died of vCJD since the mid-1990s.

Mad Cow disease destroys the brain, causing infected animals to act in bizarre ways. It was first diagnosed in Britain where about 177,000 cattle were found to be infected. Cases also have been reported in France, Italy, Germany and Spain. Thousands of cattle, in herds where Mad Cow was discovered, have slaughtered to prevent spread of the disease.

It is estimated that about one million pounds of contaminated cattle may have entered the human food chain, and authors in the study estimate that this could result in up to 136,000 cases of vCJD. Because of a long disease incubation period, it may be years before the exact toll from vCJD is determined.

Although another form of Creutzfeldt-Jakob, called ``sporadic,'' occurs in the U.S., it is not linked to eating meat. There have been no identified cases of vCJD, the form of the disease linked to beef, in the U.S.

BSE is thought to have spread to cattle through the use of feed enriched with body parts from sheep infected with scrapie, a related disease.

The FDA in 1997 banned the use of any proteins from cows, sheep, goats, deer or elk, all of which get BSE-like diseases, from being used in feed for cattle, sheep or goats. The agency in January, however, said that 30 percent of U.S. plants that render animal products into livestock feed had no system to prevent the inappropriate addition of animal body parts into feed.

All of these brain-wasting diseases, in animals and humans, are caused by a prion, a misshapen protein that alters the shape of other proteins, and causes the formation of cavities in the brain.


21 Mar 01 - CJD - Supermarket to test for BSE

Staff Reporter

BBC--Wednesday 21 March 2001


Sainsbury's has announced that it will introduce BSE testing on beef supplied to its supermarkets. It intends to pioneer a process developed by Swiss scientists to test beef at abattoirs after cattle are slaughtered.

BSE, commonly known as "Mad Cow disease", has been linked to the potentially fatal human brain condition new variant CJD.

The EU validated tests, which Sainsbury's will use, will search for abnormal prion protein (PrPres) in meat samples.

Meat will be tested for abnormal prions

Scientists believe the presence of PrPres in meat samples is a marker for the disease.

Sainsbury's announced the measure on the same day as the results of a public health inquiry into a cluster of vCJD cases in Queniborough, Leicestershire, were made public.

The Leicestershire Health Authority inquiry said the Queniborough outbreak could have been spread from outdated butchering practices.

High-risk brain tissue may have come into contact with meat cuts intended for human consumption via butchers' hands or knives.

Alec Kyriakides, Sainsbury's chief microbiologist, said: "We set ourselves very high food safety standards, so we will always trial the latest techniques.

"We are also the only UK supermarket to sell beef that is DNA tested so that we can trace back to the animal.

"Our research shows that BSE is still one of the highest issues of concern for our customers in terms of health and food safety.

"We hope that the British beef industry will benefit from this move to further increase consumer confidence in British beef, which we believe is the safest in the world."


21 Mar 01 - CJD - CJD cluster deaths linked to butchery

Staff Reporter

BBC--Wednesday 21 March 2001


Traditional butchery practices are the most likely cause of Britain's first variant CJD cluster, say experts.

The Department of Health has pledged to examine the findings of the report into the deaths of five young people in the village of Queniborough from vCJD, unveiled at a public meeting on Wednesday.

vCJD (Creutzfeldt Jakob Disease) is the human form of BSE, or Mad Cow disease. It is thought it can be contracted by eating meat contaminated with BSE, but the link has not been categorically proved.

According to latest figures, 95 people have died from definite or probable vCJD.

The inquiry team, from Leicestershire Health Authority, believe the infection that caused the Queniborough outbreak could have been spread from high-risk brain tissue to cuts intended for human consumption via butchers' hands or knives.

They say the critical period occurred between 1980 and 1991, and believe the incubation period for the disease could be up to 16 years.

They also believe only small amounts of contaminated material are enough to put humans at risk.

The inquiry found that although all the victims did not use the same butcher, they all ate beef or beef products.

The experts believe out-dated techniques used by some small abattoirs and butchers probably spread the disease from cows to humans.

Infected cattle

Dr Philip Monk, consultant in communicable disease control at Leicester Health Authority, told the meeting that it was "plausible and possible" that cows infected with BSE were slaughtered in abattoirs in the area during the 1980s.

He said the people who died purchased meat from butchers where it was common practice for the heads of slaughtered cows to be split open so the brain could be removed.

The brain is protected by a membrane, but if this is split, the brain material has a tendency to ooze out and to stick to things, increasing the risk that BSE-contaminated material will infect joints intended for human consumption.

The risk of transmission was also increased by practices used in smaller abattoirs, where rods were inserted into the animals brains to ensure they did not kick out during the slaughtering process. In addition, slaughtered animals were wiped with cloths, rather than hosed down.

Report co-author Dr Gerry Bryant stressed butchers had done nothing wrong.

She said: "This represented traditional butchering craft, and was quite legitimate practice.

"In all of the 22 local retail butchers we had to interview, we only found four who practised in this way. None of the supermarket chains or freezer food centres we identified practised in this way.

"This practice was stopped very quickly after BSE became recognised."

Dr Bryant called for a national investigation of the team's hypothesis.

She said: "It is unlikely that this is the only means of exposure of humans to the BSE agent.

"But by testing this hypothesis it will help determine how important this is as a possible means of transmission of the disease and may help in predicting the future size of the epidemic."

No other common connection

The experts ruled out any other common connection between the five victims of vCJD.

There was no evidence that the five victims had undergone similar types of surgery, had similar vaccinations, or even shared the same dentist.

Neither was there any link between their jobs. The theory that the water supply was contaminated was also ruled out.

Professor Roy Anderson, a member of the Government's Spongiform Encephalopathy Advisory Committee (SEAC), who advised the Leicestershire Health Authority, said the report had come to "a very plausible explanation".

However, Professor Richard Lacey, of Leeds University, a microbiologist who first suggested the link between BSE and vCJD, said nobody knew for certain how vCJD was transmitted.

He said the Leicestershire report was "pure speculation".

Clive Evers, from the CJD Support Network, said: "This inquiry is important because it has explained in some detail what went on at that time and families want explanations and want to know why this happened to their particular relative at that time."

The cluster was first reported in 1998 after three people died within 12 weeks.

Glen Day, 35, from Queniborough and Pamela Beyless, 24, from nearby Glenfield died in October.

Stacey Robinson, 19, formerly of Queniborough, had died two months earlier in August.

A 19-year-old man then died in May and at the same time health officials said it was "highly probable" that a 24-year-old man in the county had also contracted the disease.

A fifth person, a male farm worker, died in September.


21 Mar 01 - CJD - Cause found for BSE hotspot

Andrew Veitch

Channel 4 News--Wednesday 21 March 2001


Snow:

We'll get more of the details tomorrow but how does this impact on our understanding of CJD?

Veitch:

Well it starts to tell us just how infectious the disease is: these children were not eating cows' brains, they were eating beef that had been contaminated with brains, because of the peculiar way cows were reared and butchered in this part of Leicestershire. Find other areas where the same sort of thing happened and you might be able to start to look at specific risks. I think it does suggest also that we can worry perhaps less about the vaccines given to children.

Snow>:

It makes it seem even more extraordinary that 12 years after Britain banned things like brain and spinal cord, we're still finding bits of spinal cord and brain in meat from places like Germany...

Veitch:

It is, isn't it. Europe is coming down hard on German suppliers - but it is quite extraordinary that this is still going on. We know what a terrible tragedy it has probably caused. Of course, we know there is not very much BSE in Germany, but as we import more food, more beef, because of the foot-and-mouth disease, epidemic here, then the problem just gets worse and worse -- something is going to have to be done about that.

In the small Leicestershire village of Queniborough, where five people died from the human form of BSE, doctors have come up with the first hard evidence that food was indeed to blame.

Tomorrow the team investigating the cluster of cases will give their report to health chiefs and the parents of those who died.

Channel Four News can disclose that a series of coincidences in the food chain there was to blame - specifically the way meat was prepared in the village.

Our Science correspondent Andrew Veitch reports:

It became obvious in the summer that something odd had happened in Queniborough: five cases of variant CJD in one village - and there are still only 95 in the whole of Britain.

It's the first and the largest cluster.

Arthur Bayless and his wife June lost their 24-year-old daughter - finding the cause, he says, will not soften the pain.

Arthur Bayless:

"No -- not at all. I've got to come to terms with it. If I want to carry on, if June wants to carry on and the family want to carry on -- we've all got to come to terms with it. Knowing that it's meat is not going to help us come to terms with it. I want to dwell on positive things. I want to dwell on the kind of person Pamela is, not what killed her."

But Leicester's public health team was determined to find out what happened: they've worked flat out for nine months and they've traced what they believe to be the common factor: meat contaminated with the brains of infected cattle.

Tomorrow they will tell parents and Leicestershire health authority that it's the first hard evidence that infected meat has caused the human form of BSE.

Channel Four News learnt of this last year when we talked to doctors investigating three more cases in Arnthorpe near Doncaster: they were looking at a similar source but we agreed to withold the information lest it prejudice the Queniborough inquiry.

Dr John Radford, DOncaster Public Health Consultant:

"The most likely cause is eating contaminated meat at some time between the period about 1982 to 1988/89. We are working very much along the lines of an investigation that has been done in Queenborough in looking at the food history of the cases and investigating the food history -- looking at the pattern of distribution in meat outlets locally and how that worked and how potential contaminated sources could get into the food chain."

The danger should have ended when brain, spinal cord and other infected offal was banned in 1989.

But lax controls in abattoirs, highlighted in Lord Phillips' report on BSE, meant millions more were exposed to the mutant prion proteins that carry the disease.


21 Mar 01 - CJD - Traditional methods 'caused vCJD deaths'

Staff and agencies

Guardian--Wednesday 21 March 2001


Traditional techniques at local abattoirs linked to small-scale butchers led to the deaths of five people in the Leicestershire village of Queniborough from variant Creutzfeldt-Jakob disease (vCJD), the official public health report into the cluster said.

Dr Philip Monk, consultant in public health for Leicestershire health authority, said small slaughterhouses in the area had used a recognised technique involving inserting something into the brain to ensure cows did not kick out during the slaughter process.

However, because it was "plausible" that herds in the area were infected with BSE, this technique led to infected material leaking to other parts of the animal.

Dr Monk said: "These were traditional craft butchering practices carried out by people who were experts in their tradition. None of them were illegal. They were both legal and crafted processes that were going on in the 1980s."

He added: "The people who had vCJD were exposed to the BSE agents through the consumption of beef which had been processed from butchers where there was a risk of cross-contamination of bovine brain material during the boning and cutting process in those butchers premises where the skull was split to remove the brain."

The report into Britain's first cluster of deaths from vCJD revealed that a series of coincidences in the way the meat was prepared was responsible for the disease.

The study looked at farming methods and food supplies in the area between 1980 to 1991, the period of time in which all five victims lived in the area.

The investigation was launched in July last year and a progress report in November indicated that lines of inquiry had been narrowed and were concentrating on the meat supply chain as "the only remaining factor".

At the report's publication in Queniborough, Tim Healey, chairman of Leicestershire health authority, which prepared the report, paid tribute to the victims' families for their help in the "painful experience" of investigating the deaths.


21 Mar 01 - CJD - vCJD: the tragedy of lessons unlearned

Hugh Warwick

Guardian--Wednesday 21 March 2001


If we fail to reform our food industry, more deaths will follow the five in Leicestershire.

The tragedy of the cluster of variant Creutzfeldt-Jakob disease (vCJD) cases in Leicestershire is that we appear to have failed to learn some hard-taught lessons.

In the UK, 90 people have been killed by a disease that, apparently, could never happen. Experts assured us repeatedly that humans could never pick up the disease that has blighted the beef industry. But the food industry continues to become more about industry than food.

The local abattoirs at the centre of this cluster are just the unwitting pawns in a game that the corporations are playing with the lives of consumers. Traditional butchering is not the problem; small abattoirs are not the problem. The problem is the commoditisation of food.

Yet we are faced with an industry that continues to pursue this end. It does not matter that, on the one hand, the industry that creates genetically engineered food claims it is exactly the same as traditional food, to avoid any testing; while, on the other, claiming that they are entirely different to gain a patent.

Consumer pressure has had an impact in the UK. But this is not a real change of heart by the industry. It is just a tactic. The food producers are simply waiting until all the fuss has died down. And they are waiting until the Britain's countryside is so contaminated by GM crops that they can safely say it is impossible to guarantee anything is truly GM-free.

As yet, there is no proof that GM food is bad for us. But there is also no proof to the contrary. So, should we just wait? Or should we look at the chaos an intensive industry has already caused through BSE and start to question their rights to use us as guinea pigs in their latest experiments?

Hugh Warwick is the editor of Splice, the magazine of The Genetics Forum


21 Mar 01 - CJD - CJD deaths due to 'traditional' butchers

Staff Reporter

ITN--Wednesday 21 March 2001


"These were traditional craft butchering practices carried out by people who were experts in their tradition. None of them were illegal. The were both legal and crafted processes that were going on in the 1980s." - Dr Phillip Monk

Butchers using traditional techniques passed on the human form of Mad Cow disease to five people from Leicestershire who later died of it, a report has found.

Leicestershire Health Authority commissioned the study into the cluster of deaths from new variant Creutzfeldt-Jakob disease (vCJD) around the village of Queniborough.

Report author Dr Philip Monk found the victims had all eaten beef that had been contaminated by animal brains infected with Bovine Spongiform Encephalopathy (BSE).

The inquiry into the deaths was launched last summer in the hope that it would unlock the key to how BSE, the brain-wasting disease in cows, was passed to humans.

Over 80 people have died in Britain from vCJD, the devastating condition which leads to the gradual loss of co-ordination and speech and ultimately death.

The report into the unusual cluster was read out to a packed meeting at Queniborough village hall.

Dr Monk first outlined how BSE, made notifiable in 1986, developed and how vCJD was officially recognised in 1996.

The investigation found that many cows in the area intended for beef production had been fed with meat or bonemeal at an early age - a factor blamed for causing BSE.

Dr Monk said: "It is possible that a small number of cattle in this area could have been incubating BSE at the time of slaughter in the early 1980s."

While most cows were slaughtered in large abattoirs with modern techniques, a number were killed in small abattoirs or in butchers with a slaughterhouse attached

He said that it was likely that it was the way that these cows were slaughtered that passed on the disease.

Dr Monk said their skulls were split so the brains could be sold and a "pithing rod" - designed to stop the cows kicking in the enclosed space of a small slaughterhouse - was inserted into the spine.

This, he said, was "an extremely tricky and messy process" in which there was a tendency for material from the brain to ooze out.

"These were traditional craft butchering practices carried out by people who were experts in their tradition. None of them were illegal. The were both legal and crafted processes that were going on in the 1980s," he said.

And added: "The people who had vCJD were exposed to the BSE agents through the consumption of beef which had been processed from butchers where there was a risk of cross-contamination of bovine brain material during the boning and cutting process in those butchers premises where the skull was split to remove the brain."

He added that this may not have been the only way that people were exposed to the disease.

Tim Healey, chairman of Leicestershire Health Authority, paid tribute to the victims' families for taking part in the "painful experience" of investigating the deaths.


21 Mar 01 - CJD - Butchers' knives link to village disease deaths

Ananova

PA News--Wednesday 21 March 2001


Evidence that contaminated meat is to blame for the human form of Mad Cow disease is expected to be revealed today.

An investigation in Leicestershire is believed to have found contamination via butchers' knives between animal brains infected with BSE and cuts of meat.

Five people died from vCJD in the village of Queniborough between August 1998 and October last year.

The report into Britain's first cluster of deaths from vCJD is thought to reveal that a series of coincidences in the way meat was prepared is to blame.

The findings of the investigation by Leicestershire Health Authority are due to be published this morning.

The Department of Health has refused to comment ahead of a meeting in the village.

Leicestershire Health Authority says it has discovered how the people died but has refused to reveal its findings until later.

The investigation was launched last July and a progress report in November said lines of inquiry were concentrating on the meat supply chain.

Professor Richard Lacey, one of the first scientists to raise the alarm about vCJD, has cast doubt on the value of the report.

He told the BBC: "It is really pure speculation, I'm afraid. We don't know the year it started, we don't know if the dose was on one occasion or over many years, we don't know how it got from cows to people."


21 Mar 01 - CJD - 'Contaminated meat to blame for CJD deaths'

Ananova

PA News--Wednesday 21 March 2001


The first conclusive evidence that contaminated meat is to blame for the human form of Mad Cow disease is due to be revealed, Channel 4 claims.

An investigation into Britain's first cluster of deaths, from the variant form of CJD, will reveal that a series of coincidences in the way meat was prepared is to blame.

Five people died in the Leicestershire village of Queniborough from vCJD.

The findings of an investigation by Leicestershire Health Authority are due to be published tomorrow.

Channel Four News said villagers died because brain and spinal cord from cattle infected with BSE found their way into the food chain.

The Department of Health refused to comment.

Leicestershire Health Authority says it has discovered how the people died, but has refused to reveal its findings.

Arthur Bayliss, whose 24-year-old daughter Pamela died of the disease, told Channel Four News: "I've got to come to terms with it, my wife has got to come to terms with it and so has my family.

"Knowing it is meat is not going to help us come to terms with it."

Channel Four News claimed doctors in Doncaster are looking at a similar scenario which is believed to be responsible for three deaths.


21 Mar 01 - CJD - Slaughter and butchery practices to blame for CJD deaths

Ananova

PA News--Wednesday 21 March 2001


Traditional slaughter and butchery practices used in the 1980s are likely to be the cause of the vCJD cluster in Leicestershire.

A report by Leicestershire Health Authority has revealed the only factor to link the five people who died between 1998 and 2000 was the fact they ate beef.

Dr Philip Monk, the consultant in public health who led the investigation, stressed there was no one butcher or slaughter house involved in the outbreak.

He said in the 1980s at small abbatoirs the membrane around cow's brains was often punctured by an instrument intended to stop them kicking.

This meant when butchers removed the brains, some oozed out of the membrane and could have contaminated other meat or instruments.

He said such practices were legal then, and were skilled crafts carried out by experienced workers.

He concluded it was likely "these five people were exposed to the BSE agent through the consumption of beef carcass meat purchased from butchers where there was a risk of cross contamination."

Dr Philip Monk, consultant in public health for Leicestershire Health Authority, said small slaughterhouses in the area had used a recognised technique involving inserting something into the brain to ensure cows did not kick out during the slaughter process.

But because it was "plausible" that herds in the area were infected with BSE, this technique led to infected material leaking to other parts of the animal.

Dr Monk said: "These were traditional craft butchering practices carried out by people who were experts in their tradition. None of them were illegal. The were both legal and crafted processes that were going on in the 1980s."


21 Mar 01 - CJD - Cattle Brains From Bse Cows 'Caused vCJD Deaths'

Staff reporter

YAHOO--Wednesday 21 March 2001


A cluster of cases of variant CJD in Leicestershire was probably caused by the victims eating meat which had come into contact with cattle brains.

The findings were made by the official investigation into the deaths of five people with contacts to the village of Queniborough.

They found they had all eaten meat from small butchers and abattoirs where traditional butchering techniques led to particles of brains from infected cows being mixed in with meat.

Dr Philip Monk (pictured), consultant in public health for Leicestershire Health Authority, said butchers' knives that became contaminated also passed on the disease.

The five victims died between August 1998 and October last year. But Dr Monk warned he could not guarantee that no-one else had been infected with the disease.

Skulls split

He said the deadly disease spread through cows' bodies when a device was inserted into their skull during slaughter to prevent them from kicking out.

It had the effect of splitting the skull and mushing up the animal's brain, parts of which then leaked into other parts of the animal which was sold on to butchers.

The disease was also passed on by butchers who cut up whole carcasses, including the animal's head. Brains were removed as there was a commercial market for it at the time, he said.

This, he said, was "an extremely tricky and messy process" in which there was a tendency for material from the brain to ooze out. In cases where the animal had BSE, this could lead to other meat and knives becoming contaminated.

Dr Monk said: "These were traditional craft butchering practices carried out by people who were experts in their tradition. None of them were illegal. The were both legal and crafted processes that were going on in the 1980s."

'Assumed not proven'

But Professor Richard Lacey, one of the first to highlight vCJD, dismissed the report's findings as "speculation".

He said: "We don't know the year it started, we don't know if the dose was on one occasion or over many years, we don't know how it got from cows to people."

He added the report had the premise that BSE was transmitted to humans by eating meat, which, he said, was "assumed but not proven". He said milk or transmission through the air could also be to blame.Sainsbury's to test meat

Claims of a similar occurrence in Doncaster have also emerged, with doctors allegedly investigating three deaths.

Clive Evers, from the CJD Support Network, said the group was looking forward to results of the "extensive" investigation.

In the wake of the latest health scare, supermarket chain Sainsbury's said it would become the first UK retailer to introduce BSE testing on its beef.


21 Mar 01 - CJD - Report on "Mad Cow" death cluster eyes butchers

By Ian Hodgson

YAHOO--Wednesday 21 March 2001


QUENIBOROUGH, England (Reuters) - A report into five deaths from the human form of "Mad Cow" around one village has found that local butchers were the most likely cause of the cluster of fatalities.

The inquiry into the deaths in and around Queniborough, central England, was launched last summer in the hope that it would unlock the key to how Bovine Spongiform Encephalopathy (BSE), the brain-wasting disease in cows, was passed to humans.

There are 95 confirmed or probable cases of new variant Creutzfeldt-Jakob disease (vCJD), the devastating condition which leads to the gradual loss of coordination and speech and ultimately death.

More than 80 of those have been fatal, and two people are also reported to have died in France of vCJD.

"We developed the hypothesis that the people who had developed variant CJD were exposed to the BSE agent through the consumption of beef and carcass meat," said Dr Philip Monk, one of the authors of the report.

He specified that meat from the butchers in question was at "risk of cross-contamination with bovine brain material during the boning, jointing and cutting process in those butchers' premises where the heads of beasts were split to remove brain."

For the local community, the report helped clarify some of the mysteries of the disease.

"Everyone came here with a very limited understanding of what CJD was and I think they have a better feeling now for how it happened and what is being done to stop it," said 17-year-old James Webster, who is from a nearby village.

"The disease has had a huge impact on the local community. You hear how drastic the effects of the disease are."

CONTACT WITH BRAIN NOW BANNED

Professor Roy Anderson, epidemiologist at Imperial College, London and an adviser to the inquiry, said butchers around the Leicestershire village had apparently continued to have physical contact with cows' brains despite the fact that the practice had become increasingly rare in the 1970s.

It was banned by the government in 1989, three years after BSE was first identified in Britain.

Anderson warned against reading too much into the report but said it shed some light on the likely incubation period of vCJD.

"It's beginning to put the incubation period in the region of perhaps 10 to 16 years," he said.

Some top scientists dismissed the inquiry as a gimmick aimed at finding a scapegoat without addressing the causes of vCJD.

"We don't know the year it started. We don't know how it got from the cows to people. We have no idea as to how it spread in detail from cows to people," said Professor Richard Lacey, one of the first people to link BSE and CJD some 10 years ago.

"It's not really being very honest... This has been the whole basis of BSE over 15 years -- not to get at the truth but to reassure in the short term," he told BBC radio.

Mad Cow disease was first identified in Britain in 1986 and peaked in 1992. It cost the country billions of pounds in lost exports and farmers were only just recovering from the crisis when the present outbreak of foot-and-mouth disease struck.

Since first identifying vCJD in 1996, scientists remain divided as to how the disease is passed to humans. Most believe it is caused by prion proteins which can cause the brain to become spongy and eventually wither.


21 Mar 01 - CJD - Butcher welcomes CJD report

Staff Reporter

BBC--Wednesday 21 March 2001


Traditional butchery practices implicated in vCJD cluster

An award-winning butcher has welcomed the official report into the cluster of variant CJD deaths in his village as "fair and well-researched".

Leicestershire Health Authority reported on Wednesday that traditional butchery practices could be partly to blame for the high incidence of vCJD (Creutzfeldt Jakob Disease) in Queniborough.

Five people from the village have died of vCJD, the human form of Mad Cow disease.

David Clarke, 63, a butcher in Queniborough since 1981, said the report highlighted practices which had been commonplace but were no longer used due to what the meat industry had learned about BSE.

The report highlighted a critical period between 1980 and 1991, a time when Mr Clarke said no one knew what BSE was.

"It is terrible what happened to these people, not only in this area, but in other parts of Britain," said Mr Clarke.

"But you can't blame anyone. Butchers were using methods that were traditional that everyone used."

Learning the lessons

The report found the vCJD victims had bought meat from butchers who typically split open the heads of slaughtered cows to remove the brains.

BSE-risk material - which is thought to be concentrated in the brain and spinal cord - may then have contaminated other cuts of meat via equipment such as knives.

It cannot be destroyed by usual cleaning methods.

Pamela Beyless, a regular visitor to Queniborough, died of vCJD

"No one had heard of BSE and no one knew there was any danger," said Mr Clarke.

The important thing was for Britain to learn lessons from BSE, he added.

Such a cluster of vCJD deaths could have happened in other parts of the country because "everyone was using the same methods," he said.

Mr Clarke who has been in the meat trade for 47 years said: "When I started there was food rationing.

"You needed all the food you could get. The head was regarded as a bonus.

"No one knew about the dangers of spinal cords and so on.

"But we have learned. The British meat industry is now squeaky clean."

However, Queniborough had been under a cloud since the CJD cluster was confirmed, he said.

"Queniborough used to be such a lovely name, a name you could trade on," he said.

"Now it just leaves a terrible feeling in your stomach. It really does."


21 Mar 01 - CJD - Mad Sheep: Federal Government Arrives to Take Sheep Suspected of Disease

By Wilson Ring, The Associated Press

ABC News--Wednesday 21 March 2001


G R E E N S B O R O, Vt., March 21 - Federal agents went to a farm early today to seize sheep feared infected with a version of the Mad Cow disease.

Houghton Freeman's flock of 233 sheep is one of two that has been at the center of a storm of protests since the U.S. Department of Agriculture ordered that they be seized and destroyed. The department says the sheep, imported from Belgium, could be carrying a disease akin to Mad Cow disease.

Ed Curlett of the USDA, speaking from the Freeman farm, confirmed the seizure was under way. Inspectors arrived between 6 a.m. and 6:30 and trucks arrived around two hours later, he said.

The sheep are to be taken to federal laboratories in Iowa for scientists there to take samples from their brains to study. They will eventually be slaughtered.

"We intend to collect the sheep," Curlett said. "We are very grateful for the owner's cooperation."

The other disputed flock, 140 sheep, is owned by Larry and Linda Faillace of Warren. They were to be seized later, and they will receive notice the night before the seizure, as Freeman did, Curlett said.

The seizure at the Freeman farm came one day after supporters of the owners held the latest in a series of protests, marching to the Vermont offices of the three members of the state's congressional delegation. All three have supported the seizure.

The government says the sheep may have been exposed to Mad Cow disease through contaminated feed before they were imported from Europe in 1996. The owners say the sheep are healthy and the tests are not conclusive. They have urged that the sheep be studied and tested more extensively.

After losing their case in U.S. District Court in February, the Faillaces and Freeman appealed to the federal circuit court and asked that the seizure order be put on hold until the case had worked its way through the courts.

The circuit court refused to stay the seizure order last week but said it would hear the appeal.

The USDA maintains that four of the sheep culled from Freeman's flock showed signs of transmissible spongiform encephalopathy. That is a class of neurological diseases that includes both Bovine Spongiform Encephalopathy, or Mad Cow disease, and scrapie, a sheep disease that is not harmful to humans.

The government says the sheep may have been exposed to Mad Cow disease through contaminated feed before they were imported from Europe in 1996, and have quarantined the sheep since 1998.

The human version of BSE, which like the animal version has a lengthy incubation period, has killed almost 100 people in Great Britain since 1995, when it virtually wiped out the British beef industry.


21 Mar 01 - CJD - U.S. seizes sheep with possible strain of Mad Cow disease

Staff Reporter

CBC--Wednesday 21 March 2001


GREENSBORO, VT. - Fears that a version of Mad Cow disease may have spread to the U.S. have prompted officials to seize a flock of sheep in Vermont, in spite of angry protests by the animals' owners.

The animals were loaded onto trucks in the town of Greensboro early Wednesday morning by teams of vets and government officials. Two dozen police officers stood guard.

The sheep have been at the centre of a controversy since four of the 355 animals tested positive last summer for a class of neurological disease that includes both Mad Cow and the sheep disease scrapie.

Officials maintain the sheep may have eaten contaminated feed before they were imported from Belgium in 1996. They've been under quarantine since 1998. Although no cases of Mad Cow disease have been found in sheep the government says it is taking no chances.

Their owners say the flocks are healthy and the tests are inconclusive. They've called on the government to conduct further tests. An appeals court had been scheduled to hear their objections next month.

Their lawyer calls the government's move, "sad, depressing and a rush to judgement."

Two flocks are affected. The second one is expected to be seized later today. It's the first time such fears have prompted officials to seize livestock.

On Tuesday supporters of the owners held a march to the offices of their political representatives, who have all come out in favour of the seizure.

Several prominent Vermont farm groups also support the move, saying it's better to be safe than sorry.

The human version of Mad Cow disease has killed almost 100 people in Britain.

The sheep are being taken to a national veterinary laboratory in Iowa, where scientists will study samples from their brains. The flock will eventually be destroyed.


21 Mar 01 - CJD - Mad Cow In The U.S.?

Scott Pelley

CBS News--Wednesday 21 March 2001


Will Disease Strike American Cattle?

Some Poke Holes In U.S. Testing And Rules

Should Officials Adopt European Precautions?

(CBS) Mad Cow disease has spread throughout Europe. Could it come to the United States?

The disease first surfaced 15 years ago in Britain. The bizarre illness swept through cattle herds, and then, researchers say, started killing people who ate infected meat. Europe imposed strict laws to stop the spread of the disease. But this winter Mad Cow disease appeared in Spain and Germany. Now it has been reported in 15 countries in Europe and the Middle East. There's been one case reported in Canada.

Mad Cow has never been found in the United States. But this country is not taking all precautions. Experts warn that America is risking too much.

"She was bright full of life, intelligent going places, my best friend," says Annie McVey of her daughter Claire, who died at 15.

Claire was one of the youngest victims in Britain. She suffered the human form of Mad Cow, a brain disorder called variant Creutzfeld-Jakob Disease, or CJD. The first sign that her mother noticed was when Claire suddenly insisted on eating alone.

"And I found later that she was eating on her own because sometimes the food went in her mouth, and sometimes it didn't," McVey says. "Sometimes it fell off the fork, and she couldn't control her movements."

Claire knew well before her mother did that there was something seriously wrong. It was a secret she could not keep for long.

"She would scream, ah, the terrible screaming that went on for hours and hours and she had no idea why she was screaming," McVey says.

Her mother first recognized symptoms in Claire only five months before she died.

The decline was much the same for another young English woman. Just a year ago, Sarah Roberts told her parents Sheila and Frank Roberts that her legs hurt. Doctors said it was stress.

"She took herself to the hospital," Sheila Roberts says. "The doctor said to her you need to go home....And if you don't stop what you're doing, you'll end up in the psychiatric block."

But Sarah Roberts wasn't losing her mind; she was losing her brain. She began to stagger and then to forget how to do things she did every day - like turning on her computer.

Her mother had to show her how to take a shower.

With more tests, doctors finally diagnosed variant CJD. Sarah Roberts rapidly descended from forgetfulness to insanity.

"She used to scream. It was terrible to hear her scream," recalls Sheila Roberts. "She saw things. She saw animals, she saw monsters."

Sarah Roberts became blind and helpless within nine weeks of the doctor's diagnosis of variant CJD.

"When he told me, I was so shocked," Sheila Roberts says. "And he just said there's nowhere in the world you can go, and no one, no amount of money can pay for treatment....There is no cure. There isn't any treatment at all. We just have to wait."

Researchers believe that variant CJD entered the food chain in the English countryside. The problem stems from the way cattle have been fed in Western Europe and in the United States for decades. When cattle are slaughtered, part of the remains go into cattle feed.

At Your Table How have reports about Mad Cow disease changed your eating habits? Share your views on 60 Minutes II's bulletin boards.

The leftover bones, brains and blood help cows grow larger, faster. But in Britain diseased cows were mixed in the feed, and Mad Cow disease spread rapidly. In the final stages, animals suffer tremors and can't walk.

At one point it was thought Mad Cow wouldn't infect humans. Initially Britain's agriculture minister told consumers, "British beef is safe." But most researchers now believe varient CJD is transmitted by eating infected beef. In the United Kingdom that mostly likely included anything ranging from steaks to baby food. Victims never know exactly how they get it, but in Britain, so far 86 people have suffered the staggering gait, failing coordination and madness.

"It's a bit like a nightmare movie really," says Dr. Philip Monk, a British epidemiologist who is leading a new investigation into the spread of Mad Cow.

The disease is currently untreatable. Scientists believe it's a totally new kind of infection, not a bacterium or a virus.

A protein called a prion is found in all of our brains. Prions are harmless molecules unless they get twisted out of their normal shape.

"You would either chuck chemicals at them that kill infectious agents or you heat them to high temperatures and that kills the infectious agents," Monk says. "The problem with prions is that none of this seems to kill them and stop them being infectious."

There's nothing you can do in the slaughterhouse or kitchen to prevent the transmission of the prion, Monk says. "The only thing you can do is to make sure that you don't get exposed to it."

Europe has tried to curb exposure with very tough animal feeding regulations. Fifteen European countries have banned the use of animal meat and blood in animal feed. Farm animals there are vegetarians again.

But in the United States, the federal government has not imposed the same strict standards. And some experts worry the United States has too many holes in its Mad Cow defense. In 1997 the government outlawed most cattle remains in cattle feed. But cows are still an important ingredient in other animal feeds in this country.

That feed is supposed to be labeled "Do not feed to cows." But an investigation by the FDA found hundreds of feed makers in this country are violating the law.

CBS: The FDA's Stephen Sundlof

"We did a massive campaign to educate all the people who handle these products," says Dr. Stephen Sundlof, who is in charge of policing animal feed as the head of veterinary medicine at the FDA. "But some of them missed the message. And in most cases when we went out and inspected and asked them why they weren't in compliance, the answer we got most often was 'I wasn't aware of the rule.'"

"Our intent was to build a firewall," he says. "Remember that we're the second line of defense. The first line of defense is to keep this disease out of the United States," he says. "We certainly recognize that within our firewall, as we're building it, there are still holes in the firewall."

One of those potential holes is perfectly legal: The FDA still allows the use of cow blood in cattle feed, a practice banned in Europe.

New laboratory research suggest blood can infect sheep with Mad Cow disease, although there's no evidence in cattle so far.

"We're looking at blood in light of some new evidence that shows that blood may in fact be able to transmit the disease," Sundlof says.

It can take anywhere from 18 months to two years for a regulation to become final, according to Sundlof.

Federal Mad Cow strategy is based on the premise that there is no disease in the United States. The government believes American cattle have been safe ever since it banned the import of meat, feed and animals from countries with Mad Cow. But critics of that strategy say we may not have found Mad Cow because we're not looking hard enough.

"Germany and these other countries didn't detect their first cases until they started to do much larger screening," says Michael Hansen, who studies prion diseases at the Consumer Policy Institute, which publishes Consumer Reports magazine.

He says France tests more than 40,000 cows a week while in the United States only a tiny fraction of the national herd undergoes such scrutiny.

"We're testing about 2,000 brains out of 36 million," Hansen says. "The test we're using is an old test where you actually physically take the brain out...and you look at it under a microscope." That test takes 10 days.

In Europe, the test is done in just four hours. Europeans test 25 percent of the cattle and are finding prion disease in cows that still look healthy.

That concerns Dr. Michael Schwochert, who for seven years was a U.S. Department of Agriculture vet assigned to watch for sick animals in slaughterhouses.

The Department of Agriculture declined to comment on testing. The USDA did send 60 Minutes II a letter saying it will "ensure timely detection and swift response in the unlikely event that an introduction of (Mad Cow) were to occur" and that the agency will "continually improve the surveillance system."

But for now the United States says it doesn't need to adopt Europe's feed and testing precautions. The FDA is evaluating the latest research.

After Great Britain's big Mad Cow outbreak, France, Germany and Italy all implemented regulations that they were sure would protect them. But all three now have Mad Cow disease.

"It does worry me," Sundlof says about the European failure to contain the disease. "There was slippage in the enforcement."

"I think there's more that we can do, and I think we're committed to do that," Sundlof says.

Monk's advice to the United States would be adopt the measures of others. "That is the lesson from Europe. We had to learn the hard way," Monk says. "America should be quick on the draw and get on with this because the likelihood is it will come to America."

In the United Kingdom Mad Cow disease seems to be on the decline in cattle herds, but the number of people diagnosed with variant CJD is growing. The symptoms may take years to develop.

Only a few months after McVey buried her daughter, the specter of CJD returned to her home.

"This is a twist of fate we hadn't expected at all. About six months after Claire died I myself experienced some change and sensation in my legs and I thought psychological," says McVey. "And then we were trying to cross the road one day and I couldn't move. My legs wouldn't move at all....So I put people on dementia watch."

McVey may have multiple sclerosis but she worries she has CJD.

She watches herself everyday a slurred word or a lost memory exactly much like her daugher did.

"I don't want anybody else to go through this," McVey says. "It's preventable. When a child dies, it's terrible. When they die of variant CJD and all the things they're going to go through, that's horrendous. But when it's preventable, there's not enough containment in the world to hold the rage I feel at times."

The National Cattlemen's Beef Association says the American government is doing all the right things and in its view American beef is the "safest in the world."


21 Mar 01 - CJD - Mad Sheep Scare In Vermont



CBS News--Wednesday 21 March 2001


Feds Say Imported Sheep Could Have Mad Cow-Related Disease

Agriculture Department Seized Sheep Early Wednesday

Confiscated As Foot-In-Mouth Fears Continue Worldwide

(CBS) Livestock disease jitters - which Americans have been feeling from afar, with the Mad Cow and foot and mouth crises in Europe and elsewhere - hit home in a bigger way Wednesday as federal agents concerned about a Mad Cow-related disease swooped down on a farm in New England.

There were also new developments in the battle against foot-and-mouth disease, with cases being confirmed for the first time in the Netherlands, and the European Union recommending a ban on Dutch livestock imports.

The first cases of foot-and-mouth turned up a month ago in Britain, which has been hardest hit. Nations all over the world, including the U.S., have imposed countermeasures against the spread of the highly contagious disease, which hasn't been found in the U.S.

Spongiform encephalopathy, which is in the Mad Cow family of illnesses, is another matter.

Agents from the U.S. Department of Agriculture turned up at 6 a.m. Wednesday at the Vermont farm of Houghton Freeman, with trucks to seize sheep the government believes might be infected with a form of Mad Cow disease.

Freeman was aware of the government's concerns and had already asked his elected representatives, Senators Patrick Leahy and James Jeffords, in addition to Rep. Bernard Sanders, to intervene.

But all three refused to stop the seizure of the sheep, saying instead that public safety demands that the animals be destroyed.

Houghton Freeman's flock of 233 sheep is one of two that has been at the center of a storm of protests since the U.S. Department of Agriculture ordered that they be seized and destroyed.

The other flock of sheep is owned by Larry and Linda Faillace of Warren, Vermont.

All of the sheep are related and imported from Belgium, which is what triggered the concern on the part of the federal government.

The Agriculture Department says the sheep could have been exposed to Mad Cow disease by means of contaminated feed before they were imported from Europe in 1996.

The USDA also says four of Freeman's sheep show signs of transmissible spongiform encephalopathy.

The Faillaces have been waging a legal battle to stop the feds from seizing their flock and are currently appealing the government's victory last month in federal court.

USDA spokesman Ed Curlett says the Faillaces' 140 sheep will also be seized and they will be notified the night before, as was Freeman.

Freeman's flock is being taken to federal laboratories in Iowa for scientists there to take samples from their brains to study. The sheep will be taken to the National Sciences Veterinary Services Laboratory. They will eventually be slaughtered.

"We intend to collect the sheep," said Curlett. "We are very grateful for the owner's cooperation."


21 Mar 01 - CJD - U.S. seizes sheep with possible strain of Mad Cow disease



CBC--Wednesday 21 March 2001


GREENSBORO, VT. - Fears that a version of Mad Cow disease may have spread to the U.S. have prompted officials to seize a flock of sheep in Vermont, in spite of angry protests by the animals' owners.

The animals were loaded onto trucks in the town of Greensboro early Wednesday morning by teams of vets and government officials. Two dozen police officers stood guard.

The sheep have been at the centre of a controversy since four of the 355 animals tested positive last summer for a class of neurological disease that includes both Mad Cow and the sheep disease scrapie.

Officials maintain the sheep may have eaten contaminated feed before they were imported from Belgium in 1996. They've been under quarantine since 1998. Although no cases of Mad Cow disease have been found in sheep the government says it is taking no chances.

Their owners say the flocks are healthy and the tests are inconclusive. They've called on the government to conduct further tests. An appeals court had been scheduled to hear their objections next month.

Their lawyer calls the government's move, "sad, depressing and a rush to judgement."

Two flocks are affected. The second one is expected to be seized later today. It's the first time such fears have prompted officials to seize livestock.

On Tuesday supporters of the owners held a march to the offices of their political representatives, who have all come out in favour of the seizure.

Several prominent Vermont farm groups also support the move, saying it's better to be safe than sorry.

The human version of Mad Cow disease has killed almost 100 people in Britain.

The sheep are being taken to a national veterinary laboratory in Iowa, where scientists will study samples from their brains. The flock will eventually be destroyed.


21 Mar 01 - CJD - Vermont Sheep May Have Mad Cow

By Wilson Ring, Associated Press Writer

LYCOS--Wednesday 21 March 2001


GREENSBORO, Vt. (AP) - Federal agents early Wednesday began taking away sheep feared infected with a version of Mad Cow disease. It was the first time U.S. farm animals had been seized because of concerns over the disease.

Houghton Freeman's flock of 233 sheep is one of two that have been at the center of a storm of protests since the Agriculture Department ordered that they be seized and destroyed. The department says the sheep, imported from Belgium, could be carrying a disease akin to Mad Cow disease.

Ed Curlett of the USDA, speaking from the Freeman farm, confirmed the seizure was under way. Inspectors arrived between 6 a.m. and 6:30 and trucks arrived around two hours later, he said. The first truck was loaded by 9:45 a.m.

Teams of veternarians and other USDA officials dressed in brown coveralls were loading the sheep onto the trucks, as about two dozen law enforcement agents stood by.

There was no sign of protesters.

Freeman confirmed the agents had arrived at his farm but had no further comment.

``This is so unnecessary at this junction,'' said his lawyer Thomas Amidon, who had hoped the federal government would delay the seizure until after a federal appeals court heard arguments in the case next month.

The sheep were to be taken to federal laboratories in Iowa, where scientists will take samples from their brains to study. The animals will eventually be slaughtered.

The other disputed flock, with 140 sheep, is owned by Larry and Linda Faillace of East Warren. Those animals were to be seized later, and the owners will receive notice the night before the seizure, as Freeman did, Curlett said.

``We assume they're coming tonight,'' Linda Faillace said. She accused the USDA of failing to heed science.

``That's what makes us so angry. USDA builds up public hysteria over a species that doesn't get the disease,'' she said.

While the seizure was a first, another flock of 21 sheep that had come from the same family of sheep was voluntarily turned over to government officials last summer by their Lyndonville owner. The sheep were destroyed.

The seizure at the Freeman farm came one day after supporters of the owners held the latest in a series of protests, marching to the Vermont offices of the state's three congressional delegates. All three - Democratic Sen. Patrick Leahy, Republican Sen. James Jeffords and independent Rep. Bernard Sanders - have supported the seizure.

``Too little is yet known about this disease, but we do know that it is deadly and that it has the potential to spread quickly, widely and insidiously if not handled early. We wish there was a sound alternative to the removal of these flocks, but there is not,'' they said in a joint statement last week.

The government says the sheep may have been exposed to Mad Cow disease through contaminated feed before they were imported from Europe in 1996. The owners say the sheep are healthy and the tests are not conclusive, and they have urged more extensive tests.

After losing their case in U.S. District Court in February, the Faillaces and Freeman appealed and asked that the seizure order be put on hold until the case had worked its way through the courts. The circuit court refused to stay the seizure order last week but said it would hear the appeal.

The USDA maintains that four of the sheep culled from Freeman's flock showed signs of transmissible spongiform encephalopathy. That is a class of neurological diseases that includes both Bovine Spongiform Encephalopathy, or Mad Cow disease, and scrapie, a sheep disease that is not harmful to humans.

The government says the sheep may have been exposed to Mad Cow disease through contaminated feed before they were imported from Europe in 1996, and it has quarantined the sheep since 1998. The human version of BSE, which like the animal version has a lengthy incubation period, has killed almost 100 people in Great Britain since 1995, when it virtually wiped out the British beef industry.

Scrapie has been in the United States since at least 1947, while there are no known cases of Mad Cow disease, or Bovine Spongiform Encephalopathy. Destroying the sheep would eliminate them as a possible source of BSE, USDA says.

BSE has been transmitted to sheep experimentally through the feeding of small amounts of infected cattle brain. Testing to determine whether the Vermont sheep have scrapie or BSE would take two to three years to complete, USDA says.">

21 Mar 01 - CJD -

AnanovaHungry vultures 'attacking cows'

PA News--Wednesday 21 March 2001


Vultures in Spain are attacking live cows because anti-BSE measures have deprived them of carcasses.

Farmers in Morell have reported that groups of the birds have been attacking their herds and that two cows have been killed.

Experts say vultures only attack in the absence of corpses and more livestock are being destroyed before old age under regulations aimed at preventing BSE.

The two cows were killed near Masia de la Torreta on Tuesday, reports the Costa Blanca News.


21 Mar 01 - CJD - We have to learn CJD lessons, says butcher

Ananova

PA News--Wednesday 21 March 2001


An award-winning Queniborough butcher says today's report into vCJD in Leicestershire is fair and well-researched.

David Clarke says it highlights practices which had been commonplace but were no longer used because of what the meat industry had learned about BSE.

The report by Leicestershire Health Authority revealed the only factor to link the five people who died between 1998 and 2000 was the fact they ate beef.

"I think it is an excellent report," said Mr Clarke, 63, who has run the butcher's shop in the centre of the village since 1981.

"But the point is that the report is looking at a time when no-one knew what BSE was. The important thing is that we learn the lessons and I think in Britain we have learned the lessons."

Mr Clarke, who has been in the meat trade for 47 years, added: "It is terrible what happened to these people, not only in this area, but in other parts of Britain. Awful.

"But you can't blame anyone. Butchers were using methods that were traditional that everyone used. No one heard of BSE and no one knew there was any danger.

"Why this happened here I don't know. But it probably could have happened in other places because everyone was using the same methods in the old days.

"I was bought up with those methods. When I started there was food rationing. You needed all the food you could get. The head was regarded as a bonus. No one knew about the dangers of spinal cords and so on.

"But we have learned and methods have changed. And the British meat industry is now squeaky clean. I don't care where you go in the world. We are second to none in terms of our standards."

Dr Philip Monk, the consultant in public health who led the investigation, stressed there was no one butcher or slaughter house involved in the outbreak. He said the traditional slaughter and butchery practices were legal in the 1980s.


21 Mar 01 - CJD - U.S. Remains Free of BSE And FMD



PR Newswire--Wednesday 21 March 2001


WASHINGTON, March 20 /PRNewswire/ -- The U.S. remains free of foot-and-mouth disease (FMD) and Bovine Spongiform Encephalopathy (BSE), or what's commonly referred to as Mad Cow disease. There have been no cases of either disease in the U.S., due in large part to aggressive prevention measures designed to protect U.S. cattle herds and assure the safety of the beef supply for American consumers. Below is a summary of facts about FMD and BSE, including prevention measures for both:

Foot-and-Mouth Disease:

Background Foot-and-mouth disease (FMD) is a highly contagious viral disease that does not affect humans but has devastating effects on animals with cloven hooves, such as cattle, swine, sheep, goats and deer. The U.S. has not had a case of foot-and-mouth disease since 1929, an outbreak that was quickly contained and eradicated. There are seven types of the FMD virus, all of which have similar symptoms. Immunity to one type does not protect animals from other types. The average incubation period for FMD is between three and eight days, but can be up to two weeks in some cases. The disease is rarely fatal but may kill very young animals. Animals that survive are often debilitated and experience severe loss in milk or meat production.

Spread Foot-and-mouth disease is a highly contagious virus and can be spread by movement of infected animals, movement of contaminated vehicles, and by contaminated facilities used to hold animals. It also can infect animals through contaminated hay or feedstuffs and if susceptible animals drink from a common water source. While FMD is not considered a threat to human health, people who come in contact with the virus can spread it to animals through clothing, footwear or other equipment/materials. The virus can harbor in the human nasal passages for as long as 28 hours. Wind also can spread the virus through the air.

Economic Effects If FMD were to occur in the U.S., the degree of economic impact would depend on how quickly the disease was identified and effective control measures put in place. If it was controlled quickly and eradicated, as was the case with the last outbreak in the U.S. in 1929, the damage might be small. However, if the disease became widespread, the economic loss could easily be many billions of dollars. The most serious effects would result from the necessity of destroying animals in order to eradicate the disease. In addition, countries with FMD experience restricted exports.

Prevention The U.S. Department of Agriculture (USDA) regularly monitors for any disease among U.S. cattle herds and takes aggressive steps to prevent FMD from spreading to the U.S. whenever there is an outbreak in other countries:

-- As part of its ongoing surveillance program, the USDA conducts hundreds of field inquiries each year in an effort to detect animal diseases that might affect livestock.

-- When there is an outbreak of FMD in another country, the U.S. temporarily prohibits the importation of animals and animal products from that country into the United States. These restrictions augment those already in place on ruminants and ruminant products to prevent the introduction of BSE into the U.S.

-- The government also prohibits travelers from carrying into the United States any agricultural products, particularly animal products that could spread FMD. Passengers are required to identify any farm contact to Customs and USDA officials. All baggage is subject to inspection. Violations could result in penalties of up to $1,000.

-- A team of experts (40 federal, state and University officials) is sent to the European Union - or other country with an outbreak - to monitor, evaluate and assist in containment efforts.

-- There is heightened alert at ports of entry and airports to ensure passengers, luggage and cargo are checked as appropriate. This includes placing additional inspectors and dog teams at airports to check incoming flights and passengers.

-- USDA officials also are stationed around the globe to monitor and coordinate with the state agriculture officials.

-- USDA recently initiated an aggressive public education campaign that includes additional signage in airports, public service announcements, website, and other tools to inform the public about this important issue and steps they can take to prevent it from entering the United States. The USDA also recently established an 800 number to respond to questions from the public, industry and media about the foot-and-mouth outbreak in Europe.

Bovine Spongiform Encephalopothy Summary of Facts:

Background First diagnosed in 1986, BSE is a degenerative disease affecting the central nervous system of cattle. Commonly known as "Mad Cow disease," BSE has not been found in the United States, but it has been detected in the United Kingdom and other European countries. Research from the U.K. indicates the BSE disease agent has been found in brain tissue, the spinal cord and retina (eye) tissue of naturally infected cattle. It has not been detected in muscle meat or milk.

Prevention A surveillance program begun in 1990 by the United States Department of Agriculture (USDA) has found no evidence of BSE in U.S. cattle. In addition, the USDA, the Food and Drug Administration (FDA) and many arms of the U.S. livestock industry have taken a number of measures for more than a decade to prevent BSE from occurring in the U.S. Keeping BSE from entering or occurring in this country is a top priority for the beef industry and U.S. government.

-- The United States was the first country in the world to implement stringent feed and import bans without having BSE within its borders. No beef has been imported from the U.K. since 1985. In 1989, the U.S. banned the importation of ruminant animals (cattle, sheep, goats, deer, elk and buffalo) and at-risk ruminant products (such as meat and bone meal) from all countries where BSE had been found.

-- In 1997, the USDA banned imports of all live ruminant animals and at- risk products from all European countries.

-- Also in 1997, the FDA banned the feeding of most mammal-derived protein supplements to cattle and other ruminants because, in Europe, the disease spread through feed containing protein supplements that carried the BSE disease agent. The feed ban is a critical firewall measure and is an extra precaution against BSE. While BSE has not been found in the U.S., the feed ban assures that if BSE somehow ever did get into this country, it would be quickly isolated and eradicated.

-- While the 1997 ban is a critical firewall measure, it should be noted that just feeding mammal-derived proteins to cattle does not cause BSE in and of itself. BSE is only spread by feed if that feed is contaminated with the disease agent.

-- The USDA also continues to take stringent measures. In December 2000, it banned imports of all rendered animal protein products, regardless of species, from all European countries.

-- The U.S. beef industry urges full compliance with all federal regulations and will continue cooperative efforts with the U.S. government to keep America's cattle herds safe from BSE.


21 Mar 01 - CJD - BSE and the sheep disease scrapie have similar symptoms

Staff Reporter'Mad Cow' flock seized

BBC--Wednesday 21 March 2001


United States federal officials in Vermont have seized a flock of imported sheep that are thought to have been infected with an ovine version of Mad Cow disease (or BSE).

The flock of 233 sheep - which came from Belgium and the Netherlands in 1996 - was first identified as possibly having the disease in 1998.

BSE has caused havoc in beef production in the UK, where 100 people are thought to have died of a human variation of the disease from eating infected meat.

There have been protests in the US over seizure orders issued in July for two flocks of imported after the owners said the sheep were completely healthy.

A lawyer for owner Houghton Freeman said the seizure was unnecessary, declaring it "sad, depressing and a rushed judgment".

'Causing hysteria'

A USDA spokesman said the sheep were to be taken to federal laboratories in Iowa where they would be "humanely euthanised" and samples taken from their brains for study.

He added that it was the first seizure of any cow or sheep in the US under suspicion of having an illness related to Mad Cow disease.

A second disputed flock is expected to be seized in the coming days.

Owners Larry and Linda Faillace accused the USDA was building up public hysteria "over a species that doesn't get the disease."

Full compensation

Officials say the sheep may have been exposed to Mad Cow disease through contaminated feed before they were imported from Europe and now four of them show signs of transmissible spongiform encephalophathy, a disease group which includes BSE and scrapie, the sheep disease which is harmless to humans.

The owners will be compensated for the fair market value of the sheep, the USDA said.

"While we understand this is a very difficult time for the flock owners, USDA has no choice but to take this decisive action based on the threat the sheep pose to the health of America's livestock nationwide," said Craig Reed, administrator of the department's animal and plant health inspection service.


21 Mar 01 - CJD - CJD report 'won't help me'

BBC Environment Correspondent Tim Hirsch

BBC--Wednesday 21 March 2001


Arthur Beyless has now got the answer to what probably caused his daughter Pamela to contract the agonising brain disease vCJD, leading to her death at the age of 24 two-and-a-half years ago.

It is something he really did not want to know.

At his home in a neat suburb of Leicester, I met Mr Beyless shortly after he was briefed by the head of the investigation team looking into the mystery of why five people with close connections to the nearby village of Queniborough all came down with the human form of BSE, even though only 95 people in the whole of the UK have so far succumbed.

His telephone was ringing constantly, and he had given up answering it.

"I get calls from Swiss television, German newspapers, people from America, all wanting to speak to me about Pamela," says Mr Beyless, a milkman.

"I'm just exhausted now and I have to get up at 2am to do my rounds, so I've got to stop somewhere."

'Grieving process'

He is the only one of the relatives of the five victims of the so-called Queniborough cluster who has courageously chosen to speak publicly about the quest for answers, which could help to solve some of the remaining questions about this terrible illness.

He shows no impatience with the curiosity of journalists, and speaks eloquently and courteously.

Five people died of the disease in the village

"What I've been told has not really sunk in, but then it has not sunk in properly that Pamela has died. It sounds strange, perhaps psychologists could explain it. Maybe it's just part of the grieving process."

Unlike some bereaved relatives who feel driven to find answers to why their loved ones died, Mr Beyless has involved himself in this macabre inquiry for entirely selfless reasons.

"Now I have been given all the details of exactly how Pamela probably contracted the disease, but as a father it is not something I really wanted to know. I felt I had to co-operate with the inquiry because it could help to put up bricks in the wall of knowledge about CJD, and maybe help scientific understanding which one day could lead to a cure.

"I'd compare it with having someone killed by a gun. Someone can come along and tell you all about the ballistics of the gun, exactly which organs the bullet passed through and everything. But do you really want to know that? It isn't going to help me, but it may help others."


21 Mar 01 - CJD - Italian farmers seek government help

Staff Reporter

BBC--Wednesday 21 March 2001


Sales of beef have plummeted in Italy

Thousands of Italian beef farmers have demonstrated in Rome, calling for government assistance following outbreaks of Mad Cow and foot-and-mouth disease in Europe.

At least 20,000 farmers took to the streets demanding compensation from the government following the sharp drop in red meat sales.

They also sought to reassure consumers about the safety of Italian meat.

The European Commission estimates beef consumption has dropped by 42% in Italy since last October, with butchers being the hardest hit financially.

No foot-and-mouth

''After Mad Cow disease and foot-and-mouth... we must all work to guarantee the quality of food and food safety,'' said Massimo Pacetti, president of the Italian Farmers Confederation.

''The market needs clear, transparent rules.''

Italy has reported at least six suspected cases of BSE this year, but has no reported incidents of foot-and-mouth.

Farmers' confederation spokesman Alfredo Bernardino said the farmers wanted to reassure consumers they were committed to producing high-quality, safe foods.

''Our slogan is 'Our quality is your safety','' he said.

Relief package

The Italian government recently approved a package of measures to help farmers who have been hurt by the drop in beef consumption.

The confederation estimated losses due to the Mad Cow crisis at more than 516.5 million euros ($465m).

Under the package, farmers, meat wholesalers and retailers can delay paying tax for six months.

Italian Farm Minister Alfonso Decorator sent a message of support to the farmers for what he described as "relaunching Italian agriculture by choosing quality''.


21 Mar 01 - CJD - U.S. supplement firms pledge anti-Mad Cow steps

Reuters

CNN--Wednesday 21 March 2001


WASHINGTON (Reuters) -- U.S. dietary supplements firms said Monday they were taking steps to boost consumer confidence and make sure a Mad Cow disease scare does not taint the $16 billion a year supplement industry.

Mad Cow disease has never been tracked to dietary supplements. But some scientists worry vitamins, minerals, herbs or other products that contain brain or other tissues from cattle glands could harbor the proteins thought to be responsible for Mad Cow and its deadly human form.

An industry trade association said only 0.4 percent, or about 200 of the more than 25,000 supplements sold in the United States, contained glandular materials.

Even in those products, the chance they would contain infected material is extremely low, industry representatives said. U.S. law forbids the import of any bovine ingredients from countries such as Britain, France and Ireland where Mad Cow disease has been found, and most companies use U.S. sources, according to the National Nutritional Foods Association.

The illness, known as Bovine Spongiform Encephalopathy (BSE), has not been detected in the United States, but its human form has killed nearly 90 people in Europe.

Some supplements contain tissues such as from the cattle brain, thymus, liver or adrenal glands. People generally take the products with the goal of supporting the functions of those glands, industry representatives said.

Brain and spinal cord tissue from cows is believed to carry the highest risk of passing along the disease.

Industry groups recently directed members to closely track and document ingredient sources. But they also said they did not want to leave an impression the industry had ignored the Mad Cow crisis until now.

"Companies are taking this extremely seriously. They have procedures in place. They have audited their suppliers," said Annette Dickinson, a vice president for the Council for Responsible Nutrition.

The Food and Drug Administration (FDA) inspects about 60 supplement makers each year in part to check on the origin of ingredients, said Christine Lewis, director of the FDA's dietary supplements office.

"There is no evidence companies are using products from other countries," Lewis said. "However, there is a concern. The concern is it's possible to be careless."


21 Mar 01 - CJD - DG Bank struck by Mad Cow disease

Staff Reporter

Future Vantage--Wednesday 21 March 2001


DG reported its preliminary results with an operating profit up 17% at DM1,055m (539m 339m $485m). The profits increase was depressed by a more than a two fold increase in provisions to DM1,894m. The main reason for this was the direct and indirect consequence of Mad Cow disease. DG is a co-operative bank and is heavily dependent on the agricultural sector. Many farms had also been damaged by the late 1999 winter storms.

On the bright side it also confirmed that the proposed merger with WGZ has gained regulatory approval.


21 Mar 01 - CJD - BSE report published

By Staff Reporter

Irish News--Wednesday 21 March 2001


THE first conclusive evidence that contaminated meat is to blame for the human form of Mad Cow disease is due to be revealed today, a report claimed last night.

An investigation into Britain's first cluster of deaths from the variant form of CJD will reveal that a series of coincidences in the way meat was prepared is to blame, claimed Channel 4 News.

Five people died in the Leicestershire village of Queniborough from vCJD and Channel 4 News said it had learned that meat was responsible.

The findings of an investigation by Leicestershire Health Authority is due to be published this morning.

Channel 4 News said that the villagers died because brain and spinal cord from cattle infected with BSE found their way into the food chain.

Last night the Department of Health refused to comment ahead of today's meeting in the village.

Leicestershire Health Authority, who prepared the report, said it has discovered how the people died, but refused to reveal its findings.

Arthur Bayliss, whose 24-year-old daughter Pamela died of the disease, told Channel 4 News: "I've got to come to terms with it, my wife has got to come to terms with it and so has my family.

"Knowing it is meat is not going to help us come to terms with it."

The investigation was launched in July last year and a progress report in November said lines of inquiry had been narrowed and were concentrating on the meat supply chain as "the only remaining factor".

Channel 4 News claimed doctors in Doncaster are looking at a similar scenario which is believed to be responsible for three deaths in a nearby area.