Prions
Mad Cow Home or Best Links

Mad cow link to CJD said proved
Britain may face 2nD and 3rd waves of CJD cases
New evidence underlines CJD link with beef
New Evidence Dispels Doubt on Mad Cow Disease
Collinge val/val mice dead
More evidence links mad cow disease to human disorder
Families demand compensation
Caution urged on Bruce study -- Roland Heynkes
France: "Thousands of tons of British beef are being sold illegally"
USDA to inspect another plant after E. coli found in ground beef
Incinerators ... are operators following the rules?
Britain suffers setback on beef ban
$200 billion spent per year on Alzheimer's
UK Chicken 'poisoning 500,000 a year'

Mad cow link to CJD said proved

Sunday Times, September 28, 1997  by Steve Connor  Science Correspondent
SCIENTISTS have proved that "mad cow" disease has caused Creutzfeldt-Jakob disease (CJD) in humans. The final confirmation comes after years of uncertainty and will bolster calls for a full judicial inquiry and compensation for victims' families.

Experiments on laboratory mice injected with human brain tissue have revealed the unequivocal evidence scientists have been waiting for. They can now say definitively that the infectious agent causing the outbreak of bovine spongiform encephalopathy (BSE) in cattle is the same as that causing the epidemic of new-variant CJD in humans.

Although senior scientific advisers to the government had repeatedly said that BSE was the most likely cause of the new CJD there was enough doubt for them to draw back from stating categorically that eating infected beef products was responsible. The new research dispels the doubt.

David Churchill, whose son Stephen was the first and youngest victim of the new variant of CJD, said this weekend: "If they come out and say it is definitely BSE it's a dreadful result because it means that whoever mismanaged the food chain killed our son." Churchill, the chairman of the association set up to help the 21 families so far affected by the disease, called for a judicial inquiry and compensation.

Scientists have carried out the mice experiments over the past two years and although the research is not finished, their preliminary results - expected to be published this week [available Wednesday 4:00 pm PST] by the science journal Nature - demonstrate the strongest link yet between BSE and new-variant CJD.

The study, led by Dr Moira Bruce at the government-funded Neuropathogenesis Unit in Edinburgh, compared the new variant, which has mainly affected younger people, with the "classical" CJD that usually strikes older victims and was known about long before the current BSE epidemic in cattle.

She took brain tissue from three patients who had died of new-variant CJD, and from six people who had died of classical CJD, including two farmers and two patients who died before the BSE epidemic.

Different genetic strains of laboratory mice were used because each is known to have a precise incubation period for BSE. One strain, called R3, dies within a year of being injected with BSE and shows similar neurological symptoms. Another strain, called C57, dies about 100 days after the R3 group.

The latest results show that the R3 mice injected with brain tissue from new-variant CJD patients have died at precisely the same time as they would have done had they been injected with BSE material. Deaths in the C57 strain of mice injected with new-variant CJD are also following the predicted incubation period for BSE.

This did not happen with the R3 and C57 mice injected with brain tissue from classical CJD patients. Bruce's conclusion is that the new variant of CJD in humans is caused by the same agent that causes BSE in cattle, whereas the classical CJD agent is not the same.

Her conclusions are supported by a separate series of experiments by Professor John Collinge at Imperial College School of Medicine at St Mary's hospital in London. He found molecular differences in genetically engineered mice injected with new-variant CJD material which match BSE.

Dr James Ironside, the head of pathology at the National CJD Surveillance Unit in Edinburgh, said that if the results were correct, it was the closest scientists would ever come to formally proving that BSE had caused new-variant CJD. Another senior scientist said the results were the final confirmation needed to prove that BSE had caused new-variant CJD.

   "This is bound to cause a great stir and will put enormous pressure on the
   government to agree to a judicial inquiry."
Dr Chris Bostock, director of the Institute of Animal Health, which overseas the work of the Neuropathogenesis Unit, refused this weekend to confirm the results of the mice experiments.

Britain 'may face new wave of CJD cases'

 Electronic Telegraph, September 30, 1997    By Roger Highfield, Science Editor
EVIDENCE that Britain faces a second and perhaps a third epidemic of new variant of Creutzfeldt-Jakob disease will be published by scientists this week. It is too early to say whether the 20 cases seen to date are at the peak of the first epidemic or are a "tail" announcing that greater numbers will result from exposure to BSE - mad cow disease - in coming years. "It may take 20 years for the second epidemic to materialise, and longer for the third," said Prof John Collinge, who carried out the research.

This week's issue of the journal Nature will publish his results along with those from the Neuropathogenesis Unit (NPU) in Edinburgh. Together, they mark the strongest evidence to date that the agent that causes BSE also led to the emergence of new variant CJD. Both remove any doubts that measures that have been put in place to protect the population were necessary, said Dr Chris Bostock of the Institute of Animal Health, head of one of the teams in London, Edinburgh and Newbury.

His colleague, Dr Moira Bruce, is carrying out what is seen as the definitive strain-typing experiments that have shown, in only one strain of mouse so far, that the incubation time and brain damage caused to mice by BSE is identical to that caused by new variant CJD. This is supported by a different experiment - on mice that contain human brain proteins - conducted by Prof Collinge and colleagues at St Mary's and the Institute of Psychiatry, London.

The studies fall short of direct proof because scientists have not yet identified the agent responsible for the disease, though it is linked to a brain protein called the prion protein, one described by a gene 800 letters long.

The work of Prof Collinge suggests two more epidemics of the incurable brain disease may strike in coming decades because of differences in the prion protein found in the population. We inherit two prion genes, one from each parent. To complicate matters, there are two versions of the prion gene, dubbed M and V, which differ by a single genetic "letter".

From the prion perspective, then, there are three types of people: 38 per cent are MM, the only ones affected by the current epidemic, 11 per cent VV, and 51 per cent MV, the latter being the most resilient. "Compared to the MMs, a similar or lower proportion of VVs will probably get the disease, and probably a lower proportion again of MVs," said Prof Collinge. However, there is plenty of room for error, he concedes.

The findings were made as part of a comparison of the effects of contaminated tissue from six cases of new variant CJD, 20 cases of conventional CJD caused by a range of causes - from transplants of contaminated tissue to sporadic causes - and five sets of BSE.

The study showed that the new variant disease is much more deadly to wild type mice than conventional CJD and "behaves like BSE", said Prof Collinge. Infected mice "do something very odd - they walk backwards - which we have only seen with new variant CJD and BSE". Unlike the wild mice, only half of the humanised mice are killed when exposed to new variant CJD, which is "remarkable," said Prof Collinge, suggesting that another type of agent responsible for the variant disease has been produced in these experiments.

The resistance suggests that the infectious agent responsible for new variant has to overcome a barrier, partly because the mice are VV, while all the samples of new variant CJD came from MM patients. "This suggests that if BSE transmits to humans who are VV, the disease is likely to look different." Evidence that a novel strain of the disease is produced has come from analysis of prion protein fragments from the humanised mice: they reveal a unique "type 5 pattern". Detection of this pattern in the general population would reveal that the second, VV type of disease had broken out, he said.

Support for the link between variant CJD and BSE also came from another approach taken by Dr Bruce and colleagues: she took four pure-bred strains of mice and injected them with contaminated tissue, eight BSE and three new variant. So far, only one strain, dubbed RIII, has succumbed to both variant CJD and BSE, with similar incubation periods and patterns of damage to the brain.

The Government's spongiform encephalopathy advisory committee said: "This new research provides convincing evidence that the agent which causes BSE is the same as that which causes new variant CJD. SEAC's advice to the Government has always been based on this assumption. The most likely explanation of the new variant cases to date remains exposure to BSE before the introduction of the specified bovine offal ban in 1989."

Confirmation of the results comes as the parents of Britain's first victim of new variation CJD, Stephen Churchill, prepare to lobby Euro MPs in their campaign for a public inquiry into the BSE crisis. Dorothy and David Churchill, of Devizes, Wilts, are meeting members of the European Parliament's BSE committee during their fact-finding visit to abattoirs and meat plants in Britain today. Mrs Churchill said:

"We've been waiting 18 months for these test results. It's the nearest thing we're ever going to get to proof that CJD and BSE are one and the same. We hope this will help our campaign to put pressure on the Government to set up a full judicial inquiry into the BSE crisis. And we want to see money made available for help nursing victims of CJD.
" They are also campaigning for compensation for victims, many of whom had left children and families. "The Government was quick to compensate farmers for cows that got BSE." she said. "They should make some sort of compensation award to the families."

Studies show link between mad cow disease, human variant

Agence France-Presse September 29, 1997
LONDON - Two British research studies show that the strain of prion disease that causes the new variant of a brain-wasting disease in humans is identical to mad cow disease in cattle, Nature magazine reported Monday. It said this made it highly likely that humans with the Creutzfeldt-Jacob disease variant (vCJD) contracted it by eating meat from cattle infected with bovine spongiform encephalopathy (BSE).

Until now researchers have only spoken of the possibility of a link between BSE and the CJD variant. This led the European Union to decree a worldwide embargo on British beef. Nature referred to two experiments carried out by the Institute of Animal Health in Edinburgh, Scotland, and the Prion Disease Group at the Imperial College School of Medicine in London.

The Scottish researchers, led by Moira Bruce, injected several breeds of laboratory mice with infectious brain samples from cows, from patients with the CJD variant, from patients with sporadic CJD and from farmers who died of CJD after working with animals with BSE. The researchers looked at how long it took for the injected mice to fall ill, the type of brain damage caused and the areas of brain damage. Their research showed that the symptons and course of the CJD variant in mice were identical to that of BSE in mice, but distinct from other forms of CJD.

The London research team, led by John Collinge, used a different approach that led to the same conclusion, says the magazine. They used a biochemical assay that showed that the BSE and CJD variant agents were the same, but distinct from other forms of CJD in humans. Their research showed that the disease agent that causes BSE can convert human prion protein. Genetically engineered or transgenic mice which have had their native mouse prion protein gene replaced with a human one do eventually contract spongiform encephalopathy after injection wtih BSE contaminated material.

Their original research, published in Nature in 1995, had shown that mice did not become ill, even after 200 days after inoculation. But the researchers waited longer, and now report that the mice succumb after 500 days.

Commentary on Bruce study -- Roland Heynkes

Listserve 9.28.97  Roland Heynkes ... Web site
My reaction to the Sunday Time story:
SCIENTISTS have proved that "mad cow" disease has caused Creutzfeldt-Jakob disease (CJD) in humans. The final confirmation comes after years of uncertainty and will bolster calls for a full judicial inquiry and compensation for victims' families.
I strongly doubt that this story in the Sunday Times, September 28, 1997, is true. In my opinion it can not be proved definitively that BSE is the origin of vCJD. But the evidence is strong enough to call for a judicial inquiry and compensation for victims' families.
Experiments on laboratory mice injected with human brain tissue have revealed the unequivocal evidence scientists have been waiting for. They can now say definitively that the infectious agent causing the outbreak of bovine spongiform encephalopathy (BSE) in cattle is the same as that causing the epidemic of new-variant CJD in humans.
...
She took brain tissue from three patients who had died of new-variant CJD, and from six people who had died of classical CJD, including two farmers and two patients who died before the BSE epidemic.
They can not reach such a conclusion until all variants of Creutzfeldt-Jakob disease, Gerstmann- Straeussler-Scheinker syndrome and fatal familial insomnie have been tested.
The study, led by Dr Moira Bruce at the government-funded Neuropathogenesis Unit in Edinburgh, compared the new variant, which has mainly affected younger people, with the "classical" CJD that usually strikes older victims and was known about long before the current BSE epidemic in cattle.
The repeatedly so called "classical" CJD does not exist. There is a broad spectrum of vCJDs, more or less similar to the new variant CJD which of course is not the newest variant of CJD.
The latest results show that the R3 mice injected with brain tissue from new-variant CJD patients have died at precisely the same time as they would have done had they been injected with BSE material. Deaths in the C57 strain of mice injected with new-variant CJD are also following the predicted incubation period for BSE.

This did not happen with the R3 and C57 mice injected with brain tissue from classical CJD patients. Bruce's conclusion is that the new variant of CJD in humans is caused by the same agent that causes BSE in cattle, whereas the classical CJD agent is not the same.

In my opinion this is nonsense, because the incubation time does not depend solely on the infective agent and the strain of recipient. It also depends on the dosis of infectivity and the amounts of infective agents from cow and human are not comparable until we can not count them.
Her conclusions are supported by a separate series of experiments by Professor John Collinge at Imperial College School of Medicine at St Mary's hospital in London. He found molecular differences in genetically engineered mice injected with new-variant CJD material which match BSE.
This is further evidence, but far from a proof.

Incinerators ... are operators following the rules?

Sun, 28 Sep 1997  Correspondent opinion
"There is evidence that the Government is pushing through planning applications and relaxing environmental laws on the 9 operators who are licenced to incinerate cattle. Rufforth near York is fighting a planning application in the green belt of all places!

Planning Officers are recommending refusal but the Labour controlled Council are set to approve the installation - there is talk of bribes, backhanders, etc. to council members.

The EA is turning a blind eye to it's own regulations as it is proving non cost effective for the operators to burn the cattle comply with the regs and make a profit --and of course profit ALWAYS comes first irrespective of peoples health."

France: "Thousands of tons of British beef are being sold illegally"

 Agence France-Presse September 28, 1997
PARIS - Thousands of tons of British beef are being sold illegally on the European market, including in France, the French Journal du Dimanche weekly reported Sunday. It said the British Minister of Agriculture, Jack Cunningham had confirmed as much on Aug. 28 in a telephone conversation with a socialist member of the European Parliament whom the paper did not name.
"In the past year, tens of thousands of tonnes of British beef have been exported fraudulently from Britain," Cunningham was reported to have said, adding that "a very significant part" of the meet was destined for sale "on the internal European market."
British beef has been under an international embargo since March 1996 because of fears that it could be contaminated with bovine spongiform encephalopathy (BSE), a fatal brain disorder thought to be transmissible to humans who eat meat from infected animals. British herds were worst affected by the so-called "madcow disease." The Journal du Dimanche said one of the routes used by beef traffickers went through Northern Ireland where "with the complicity of British Army reservists in Ulster, the meat is loaded onto small boats for Frence and Belgium."

Another route was through the Irish Republic which is not under embargo. There, the meat was stamped with a bogus Irish or Belgian origin before being exported to the continent. The paper said French customs authorities had sent a report to magistrate Edith Boisette, who is specialised in financial crime, aout four French firms involved in the traffic.

Drugs extend home life for Alzheimer's patients

September 28, 1997  Reuter Information Service --By JOHN ACHER, Reuters
HELSINKI - Though Alzheimer's disease remains incurable, symptom-suppressing drugs are keeping patients out of hospitals for longer, experts at a world Alzheimer's congress said on Sunday.
"We are making real progress in treatment of patients," Paavo Riekkinen, professor of neurology at Finland's University of Kuopio, told Reuters on the sidelines of the conference that officially begins on Monday. "The drugs now available wind the cognitive clock back by six to 12 months," said Dr Kari Alhainen of the University of Joensuu. "They delay the stage at which patients must go into institutional care."
Alzheimer's is a brain disease resulting from progressive degeneration of nerve cells in areas of the brain essential for learning, language and memory. It is the main cause of dementia which affects an estimated 22 million people worldwide -- including four million in the United States and three million in Europe -- and a major cost burden on health care, experts say. Two drugs, tacrine and donepezil, have been approved for treatment of Alzheimer's, and others are on the way.
"Three more drugs are in the approval phase," Riekkinen said, but added that long-term studies would be needed to show whether drugs can actually slow down progression of the disease or only alleviate the symptoms. The drugs that are available now, or that will be in a few years, are symptomatic drugs that give patients more time at home and improve their daily lives," Alhainen said. "They work on memory loss and the behavior problems that begin to affect Alzheimer's patients in the moderate phase."
Tacrine and donepezil raise the level in the brain of a substance called acetylcholine, which Alzheimer's patients typically have in reduced supply and which facilitates communications among the brain cells responsible for memory. Other drugs in the pipeline function differently, for instance by protecting nerve cells from the damage caused by the increase in Alzheimer's disease of a protein fragment called amyloid or by enhancing memory formation, experts say.
"In the future, we will be using cocktails of drugs, including drugs that enhance acetylcholine and those that protect neurons," Alhainen said.
Keeping patients out of institutions for up to a year longer than would be possible without drugs may not seem like much, but it means a lot to society, experts say.
"In Scandinavia, postponing institutionalization by just one year can save $50,000 per year (per patient)," Riekkinen told a news conference.
With people everywhere living longer, estimates of the scale of the medical and social challenge of Alzheimer's vary and are sketchy, especially for the developing world. According to some estimates, 100 million people worldwide are already affected by mild to advanced stages of disease, and that figure is expected to rise to 160 million by 2030, Riekkinen said.
"Alzheimer's is one of the three most expensive diseases in the world, alongside cancer and heart disease," said Dr Anders Wimo, associate professor of family medicine at the University of Umea in Sweden.
West European countries spend $94 billion to $122 billion a year on treating and caring for dementia patients, and the United States spends at least $100 billion a year on treatment and care for Alzheimer's patients alone, Wimo said. Costs of the voluntary help provided by families of Alzheimer's sufferers -- not to mention the emotional costs of the disease that affects care-givers as well as patients -- often go uncounted, experts say.
"It is a problem that we have hidden. Now we are opening the closets and seeing the magnitude of it," said Jan Marcusson, head of medicine and surgery at Sweden's Linkoping University Hospital. "The challenge now for health care is to build a system that will help find people (with the disease) before they are demented," he said.

USDA to inspect plant after E. coli found in ground beef

Associated Press September 29, 1997
NORFOLK, Neb. -- An inspector from the U.S. Department of Agriculture was expected Monday at the BeefAmerica plant after it was disclosed that a routine federal inspection turned up E. coli in meat at a Virginia supermarket. Records indicate the tainted beef came from the Nebraska packing plant. The company is cooperating with the investigation, said Jacque Knight, a USDA spokeswoman in Washington. The bacteria was found in fresh ground beef in Emporia, Va., earlier this month as part of the USDA's random sampling program, she said. No illnesses traced to the tainted meat have been reported, Knight said. The Great Valu Supermarket in Emporia is conducting a voluntary recall of any fresh ground beef sold in the store Sept. 3 or 4. No other recalls have been implemented.

E. coli can cause serious illness or even death if the meat is not cooked properly. The bacteria was blamed for a recall of 25 million pounds of ground beef from a Hudson Foods plant in Columbus, Neb. The Virginia supermarket's records indicate the beef originated from the Norfolk plant, Knight said. It appears the meat was shipped from the plant to a distributor in Richmond, Va., then to the supermarket. BeefAmerica does not think its plant is the source of the latest contamination, said Keith DeHann, a company spokesman in Omaha. In the Columbus case, the contamination was thought to happened before the beef reached the Hudson packing plant; the source has not yet been identified.

Chicken 'poisoning 500,000 a year'

The Times 29 Sept 97    BY MICHAEL HORSNELL
THE scientist who investigated the deadly E.Coli outbreak caused by contaminated meat in Scotland said yesterday that at least a third of uncooked chickens on sale were infected by campylobacter. The organism, which triggers gastroenteritis and can cause complications including paralysis, is now more common than salmonella and accounts for more cases of food poisoning. The danger lies in eating chicken that has not been thoroughly cooked and in failing to wash hands after touching the raw meat.

Hugh Pennington, who was called in by the Government as E.Coli killed 20 people, says he believes that up to 500,000 people are poisoned by campylobacter through chickens each year.

He is writing to the Ministry of Agriculture, for which he is conducting a two-year study, asking it to introduce a phased programme of campylobacter-free flocks among the country's 2,300 poultry farmers. Each year 730 million chickens are killed for consumption in Britain where the flock stands at about 76 million at any one time, of which the professor believes 25 million are contaminated. "We have a serious health problem that needs tackling," Professor Pennington said.

More evidence links mad cow disease to human disorder

The Associated Press September 29, 1997 
NEW YORK -- Scientists have found more evidence that a brain-wasting disease that has struck 21 people in England was caused by eating contaminated beef. Last year, the British government warned that cattle with so-called mad cow disease were the most likely cause of a variant of Creutzfeldt-Jakob disease in people. Both conditions are blamed on infectious proteins called prions.

Previous laboratory studies have supported the idea that prions from cattle caused the human disease. Further evidence appears in two new studies from England and Scotland that will appear in next Thursday's issue of the journal Nature. The work makes a convincing case that the cow and human diseases are caused by the same strain of germ, Jeffrey Almond of the University of Reading in England and Dr. John Pattison of the University College London Medical School write in an accompanying commentary.

For one paper, scientists injected mice with ground-up brain samples from three people who had died from the variant of Creutzfeld-Jakob disease and from six people who had had the standard form of that disease. Mice exposed to the CJD variant have gotten sick, with symptoms and brain abnormalities like those seen in mice exposed to mad cow disease. In contrast, the mice injected with extracts from the standard CJD haven't shown any outward sign of disease, though brain exams showed they did get infected.

The other paper, which also worked with infected mice, found chemical evidence that the CJD variant and mad cow disease are caused by the same kind of prion.

New evidence underlines CJD link with beef

September 30 1997 BY NIGEL HAWKES, Times SCIENCE EDITOR 
SCIENTISTS have found compelling evidence that BSE can be transmitted to humans. Their studies confirm that 21 people who contracted a new form of the brain disease CJD almost certainly did so from eating infected beef. By dispelling remaining uncertainties about the link, the findings seem certain to help calls by victims' families for a judicial inquiry.

Scientific groups from Edinburgh and London have been able to show that the new variant form of Creutzfeldt-Jakob disease is indistinguishable from BSE in its effects when injected into mice. Not only did the mice develop the disease in the same period of time after injection, but extracts of infected brain were identical to those found in BSE-infected cattle. Other forms of CJD developed at a different speed and produced different molecules in the brain.

John Collinge, the leader of the team from St Mary's Hospital, Paddington, says that he has reached the "inescapable conclusion" that the new variant of CJD is the human equivalent of BSE and that eating beef is probably to blame.

His findings, and those of a team led by Moira Bruce of the Neuropathogenesis Unit in Edinburgh, are published in this week's Nature. The findings were released early after a report of them appeared in The Sunday Times. Dr Bruce took brain tissue from victims of the new variant of CJD and from six patients who had died of the classical form of the disease, and injected it into different strains of laboratory mice. One strain, called RIII, died 300 to 350 days after being injected.

Dr Bruce's results show that the RIII mice injected with the new variant known as nvCJD have died at the same time as if injected with BSE. The appearance of their brains was the same as mice brains infected by BSE.

"Our data provide strong evidence that the same agent is involved in both BSE and nvCJD," the team concludes. When taken with the epidemiological data, the evidence becomes "compelling", they say.
A second strain of mice, called C57BL, have also been injected with the brain extracts. They tend to live about 100 days longer but are beginning to show signs of disease, as would be expected if BSE and nvCJD were identical.

The two new studies do not change assumptions about nvCJD and BSE. The Government was advised by the Spongiform Encephalopathy Advisory Committee in March 1996 that there was probably a link and accepted the advice. Some scientists remained sceptical, but this latest evidence is as convincing as it is likely to get. The advisory committee said yesterday that the new research was convincing but did not call for a change in policy. Measures to protect the public and in animal health were in place already, it said.

"Strictly speaking, formal proof that BSE causes new-variant CJD cannot be provided as this would require experimental infection of humans," Dr Collinge said yesterday. "But we believe that the combined weight of the evidence leads to the same inescapable conclusion: new-variant CJD is the human counterpart of BSE."

Families demand compensation

RELATIVES of victims of the new strain of CJD called yesterday for compensation and a public inquiry. Dorothy Churchill, whose son Stephen died in May 1995 at the age of 19, said:
"We have been waiting for 18 months for these test results. We hope this will help our campaign to put pressure on the Government to set up a full, judicial inquiry into the BSE crisis. "We also want to see money made available to help in the nursing of victims of CJD. The Government was quick enough to compensate farmers for cows that got BSE. We feel that they should make some sort of compensation award to the families."
Mrs Churchill and her husband, David, of Devizes, Wiltshire, pressed their case last night with a group of Members of the European Parliament who are on a three-day visit to inspect abattoirs. The couple hope the European Parliament will put pressure on the Government to provide no-fault compensation.

The call for an inquiry was backed by a solicitor representing relatives of ten CJD victims. David Body, of the Sheffield firm Irwin Mitchell, said that legal aid certificates had been granted to investigate their claims. He added, however: "The families want to find out the facts about why their relatives died. They are not desperate to embark on litigation unless they have to." Mr Body said an inquiry under a judge with power to subpoena documents and witnesses, and sitting in public, was needed.

Britain suffers setback on beef ban

September 30, 1997  Nando.net
BRUSSELS 08:04 a.m. EDT http://www.nando.net) - Britain's bid to have a European ban on its beef exports relaxed hit a snag Tuesday when the European Court of Justice's Advocate General pronounced the ban valid.
"The (European) Commission's decision prohibiting the United Kingdom from exporting live cattle, meat and other products obtained from bovine animals to other (EU) member states or to non-member countries is valid," said Advocate General Giuseppe Tesauro said.
Though the advocate general's opinion is advisory and non-binding on the court, in most cases his opinion is followed by his fellow judges. The European Court is the top legal authority in the 15-country European Union while the Commission is the EU executive body. Explaining his opinion, Tesauro said:
"The Commission's intention was to avoid the twin risks of the disease's transmissibility and the attendant concern, widespread among consumers. The reasoning is thus consistent."
The Commission banned exports of British cattle and beef products in March 1996 in an attempt to prevent the spread of bovine spongiform encephalopathy (BSE) and its human equivalent Creutzfeldt-Jacob disease, after new evidence on BSE was released.

Tesauro's opinion was handed down Tuesday on two separate but related cases, one brought to the European Court by the British government against the Commission and the other by Britain's National Farmers Union against British authorities. Britain asked the court last year to stay the ban against exports of beef and beef products until the court could fully consider the financial damage that would hit the British beef market. The court turned down that request, arguing that health safety issues outweighed any potential harm to the beef industry. It also rejected Britain's argument that the Commission had no authority to prevent beef exports to non-EU countries.

"The ban on exports to non-member countries is an indispensable tool for ensuring that the ban is truly effective for preventing the ban on exports to other member states from being thwarted by transit through non-member countries," Tesauro said. "The strict controls already placed on imports from the United Kingdom had proved to be insufficient" he said, referring to the recent discovery that British beef had allegedly been sold into the Union through other member states.

New Evidence Dispels Doubt on Mad Cow Disease

September 30, 1997 By SANDRA BLAKESLEE New York Times
Scientists in Scotland say they have found the first "compelling evidence" that mad cow disease -- a mysterious brain ailment that has killed thousands of cattle in Britain -- has been transmitted to people who apparently contracted the disease somehow after eating beef or being exposed to cattle.

So far 14 people have died from the cow disease -- bovine spongiform encephalopathy, named for the spongelike holes that it eats into the brain -- and another seven are seriously ill and expected to die from it. The research fuels fears that still others may become ill with the disease.

The researchers said their findings, which will be published in the Oct. 2 issue of the British journal Nature, remove all doubts that mad cow disease is in any way distinct from the disease that struck the people. It resembles an ailment called Creutzfeldt Jacob disease and has been called new variant CJD or nvCJD.

The researchers, led by Moira Bruce of the Institute of Animal Health in Edinburgh, Scotland, said they injected mice with tissue samples from the brains of people who died of the mysterious disease and the mice developed the same pattern of brain damage they developed when injected with brain tissue from sick cows. These findings were regarded as proof that people and cows were afflicted by the same ailment.

Dr. Robert Rohwer, another CJD expert at the Veterans Administration Hospital in Baltimore, said, "This nails it. We are about as close as we can get to having proof that people get the disease from contact with bovine products."

Until now, some mad cow experts in Britain have been arguing that the new variant of CJD, which differs from CJD in that it strikes young people, had existed in the population but had simply been overlooked, said Dr. Christopher Bostock, director of the Institute of Animal Health in Edinburgh, a government-funded agency that is looking into how mad cow disease is transmitted to other species. But that possibility is now ruled out, he said.

To discover the source of this new human disease, Dr. Bruce and her colleagues at the Institute of Animal Health carried out a series of experiments in mice. First, they took brain tissue from various sheep infected with scrapie and injected it into the brains and abdominal cavities of four strains of mice. The mice all developed holes in their brains but in distinct ways.

Incubation periods for the disease and the pattern of holes in brain tissue varied, leading to the discovery of 14 distinct strains of scrapie. That is, sheep have many different versions of the disease.

In the second experiment, the scientists infected mice with brain tissue taken from mad cows. This time, the incubation period and pattern of holes in the brain were identical in the various strains of mice. In other words, cows have only one version of the disease, which the researchers called the "BSE signature."

In the next set of experiments, brain tissue was taken from three domestic cats, a greater kudu, a nyala and a pig, all of which developed holes in their brains after eating contaminated meat or, in the case of the pig, being injected with infected cow-brain tissue. The various tissues were again injected into mice. Again, all the mice developed the BSE signature. The cow disease agent was unchanged as it passed among species.

Finally, in experiments described in this week's Nature, the researchers took infected human brain tissue, ground it up and injected it into mice. Three samples were taken from patients who died from new variant CJD. Six samples were culled from the brains of people who died from conventional CJD.

The mice injected with new variant CJD tissue are all developing the "BSE signature," Bostock said. One strain has full-blown disease, whereas the others are just now getting sick. But there is no doubt, he said, that the mice have mad cow disease that was passed on to them through human brains. The mice infected with conventional CJD brain tissue show no clinical signs of disease, Bostock said.

Mad Cow Home or Best Links