Document Directory

21 Nov 00 - CJD - CJD fears could lead to blood donor ban
21 Nov 00 - CJD - Europe wakes to BSE alarm
21 Nov 00 - CJD - No British ban on French beef
21 Nov 00 - CJD - French beef ban not necessary, says No 10
21 Nov 00 - CJD - France 'plays politics' with CJD victims
21 Nov 00 - CJD - Blair pressed to ban unsafe French beef
20 Nov 00 - CJD - Tories to demand facts on French beef
19 Nov 00 - CJD - Fourth French person may have CJD
19 Nov 00 - CJD - Doctor denies French CJD fears
19 Nov 00 - CJD - Italian farmers blockade French beef
19 Nov 00 - CJD - Déjà vu for BSE as French fail to learn from British mistakes
19 Nov 00 - CJD - Butchers Call For Ban On French Beef
18 Nov 00 - CJD - Italy bans French beef because of crisis over BSE
18 Nov 00 - CJD - French families sue Britain over vCJD
18 Nov 00 - CJD - French may charge the Tories over CJD deaths
17 Nov 00 - CJD - Ex-ministers May Face Charges In French CJD Cases
17 Nov 00 - CJD - French victims of CJD to sue Britain and EU
17 Nov 00 - CJD - Italy bans French beef imports
17 Nov 00 - CJD - Governments sued over French CJD
17 Nov 00 - CJD - Mad Cow To Be Discussed at EU Summit
16 Nov 00 - CJD - Nobody's genes can protect them from BSE
27 Oct 98 - CJD - Phenotype-genotype studies in kuru: implications for vCJD
16 Nov 00 - CJD - French Families To Sue UK After Men Develop CJD



21 Nov 00 - CJD - CJD fears could lead to blood donor ban

James Meikle, health correspondent

Guardian ... Tuesday 21 November 2000


Mad Cow Correspondent's note: readers will observe that the Phillips report whitewash has already led to the demise of the much vaunted "new government openness" which was spun by "New" Labour after the release of the report. Blood donation investigations are described as "secret", recipients of blood donated by CJD victims are kept in ignorance, and what appears to be a major review of the UK blood supply has been uncovered by a journalist rather than disclosed by the government. Nothing has changed.)

The NHS is considering banning anyone who has received blood transfusions from giving blood themselves amid rising concerns that they may unknowingly pass on the fatal human variant of BSE.

Such a move could cut Britain's 1.9m volunteer donors by up to 10% and create such huge shortages that transfusion services would struggle to meet demand.

The possibility, first secretly investigated two years ago, is being re-examined as EU scientists and officials decide whether guidance should be offered on blood safety throughout the union.

But some insiders are concerned that such a drastic step could create a level of panic among the whole donor population that would threaten more lives through shortages than might be saved from what is, at present, regarded as only a theoretical risk.

Seven of the 85 British victims of variant CJD, the human equivalent of BSE, have been identified as donors before they showed obvious symptoms of the disease. Recipients of their blood have not been informed but the Department of Health and ethics committees on health authorities are seeking to develop protocols for doing so in the future.

There is as yet no test for detecting vCJD and many patients might not wish to know whether they had been exposed to the risk of a condition for which there is not even at present a treatment to moderate its effects, let alone cure it.

White blood cells are already filtered out of blood donations because they have been thought most likely to carry the agent responsible for vCJD and there is a ban on most British-sourced plasma products .

But the government is anxious to test whether red blood cells carry the agent too. Importing enough blood to meet needs in operating theatres in this country - 2.5m units a year, each about a pint - would be impossible because of its short shelf life so all options that might minimise the threat of contamination are now being reassessed.

The health department confirmed last night that it had ordered the review as to whether those who had received transfusions should be eligible to be blood donors. "The whole issue of cleanliness [of blood] is under review. This is just part of that."

A spokesman for the national blood service said: "We have been working on the basis [that] between 5% and 10% of blood donors have received transfusions . If it were the case that 10% were knocked off our donor data base, we would be struggling to meet the needs of the country."

At present, potential donors are not routinely asked whether they have received blood themselves. Some donors may not even know because by no means all operations involve transfusions.

But the service is to question new donors among the 10% of volunteers it has to recruit each year to replace those who can no longer donate blood, or choose not to, in an effort to assess the position more accurately.

Belgium , Austria and some German states, as well as Switzerland , are already following the example of the United States , Australia , Canada and New Zealand in banning donations from anyone who spent more than six months in the UK between 1980 and 1996 , the years in which exposure to BSE through infected meat , vaccines , cosmetics or other possible routes was highest.

The blood authorities in those countries considered they could stand the loss of such donors.

It is also understood that the NHS is in discussions with Jehovah's Witnesses about their experience with recycling of patients' own blood during operations and other blood-saving techniques, as officials and surgeons step up their search for alternatives to traditional donation so they can eke out supplies.

Witnesses have already raised funds for several machines in NHS hospitals that wash and process blood lost in surgery for transfusion back into the same patients.


21 Nov 00 - CJD - Europe wakes to BSE alarm

21 November 2000

Independent ... Tuesday 21 November 2000


Mass testing set to be revealed

European Union agriculture ministers today agreed on a major package of measures to halt the spread of Mad Cow disease .

The agreement followed 16 hours of reportedly intense negotiations and was reached shortly before 7am.

The measures include testing on a massive scale of the European beef herd , involving millions of animals, but there is no EU-level clampdown on French beef.

The EU Commissioner for Health and Consumer Protection David Byrne said that all "at risk" cattle over 30 months old would be checked. .

Mad Cow disease, or bovine spongiform encephalopathy (BSE), destroys cows' brains. Scientists believe that the human equivalent of BSE, new variant Creutzfeldt-Jakob Disease, is contracted through infected meat.

* Meat from older French animals could already have entered the UK food chain , according to a report of a letter from the Agriculture Minister Nick Brown to the Prime Minister.

* Morocco has banned imports of live cattle and frozen beef from Europe to prevent the spread of Mad Cow disease.


21 Nov 00 - CJD - No British ban on French beef

Staff Reporter

Independent ... Tuesday 21 November 2000


Britain today refused to ban the sale of French beef despite increasing concerns over its safety.

Downing Street said there was no scientific justification for banning its import into the UK.

The announcement came after EU agriculture ministers agreed a new BSE testing programme after marathon talks in Brussels.

A number of countries have already banned the beef because of disturbing levels of Mad Cow disease in France.

A Downing Street spokesman said there were no plans to stop its sale in Britain.

He also claimed Agriculture Minister Nick Brown had not called for a ban and neither the Food Standards Agency or EU scientists thought it necessary.

But shadow agriculture minister Tim Yeo accused ministers of putting British consumers at risk to placate France.

(Mad Cow correspondents note: The UK needs French support in the forthcoming EU negotiations, a few more CJD deaths are obviously considered a worthwhile price to pay by the government)

Renewing calls for a ban on beef reared across the channel, Mr Yeo said he would not eat it.

And he challenged Mr Brown to make a statement to MPs clarifying the risk after the Agriculture Minister apparently sent the Prime Minister a letter setting out the risks last week.

"On Thursday Nick Brown told the House of Commons that French beef was safe, then we learn that on Friday he warned Tony Blair that it posed a health risk to British consumers," Mr Yeo said.

"Which one is it to be? While there is doubt, French beef should be banned . I would not eat French beef myself."

It is illegal to sell British beef from cows aged over 30 months for human consumption in the UK.

However, in Mr Brown's letter - copied to Health Secretary Alan Milburn - he warned meat from older animals reared in France and other EU countries could be imported to the UK , The Times newspaper said.

Downing Street today said spot checks had failed to show "any evidence" that potentially risky French beef was coming into the country.

"There is no evidence older beef is getting through," the No 10 spokesman said.

French officials gave assurances that no meat banned in their own country would be exported to Britain during a meeting of European agriculture ministers in Brussels overnight, he said.

French imports account for just 1.25% of the 800,000 tons of beef eaten in Britain each year, he added.

But he refused to say whether the Government would implement a unilateral ban if it was urged by the Food Standards Agency against European Commission advice.

Ben Gill, president of the National Farmers Union, also played down fears about the safety of French beef calling on consumers not to be "unduly alarmed".

After speaking with Mr Brown, he said: "This is about a hypothetical case in the future.

"If things get out of hand in France, if stocks of beef become untraceable, if a strong pound leads to attempts in the future - it's all about what would happen if somebody tries to abuse the law in the future.

"That's what the minister was trying to do - not ban French beef. It is precautionary planning."

Millions of cows will be tested for Mad Cow disease under the European Commission's biggest anti-BSE programme.

All "at risk " cattle over 30 months old will be checked for BSE from next January under the deal agreed by agriculture ministers this morning after 16 hours of negotiations.

The new EU measures will make little difference to the UK's anti-BSE measures, acknowledged as already more comprehensive than those in any other member state.

Random testing of animals over 30 months destined for the food chain is already carried out in Britain (Mad Cow correspondents note: it is this testing which is responsible for the extra cases of BSE that have been found in France. At least the French government, unlike its UK counterpart, releases the results of the tests) , and extending the system to all cattle over the age limit will involve a "negligible" extra burden, according to officials in Brussels.

The Consumers' Association welcomed the programme but called for country of origin labelling to be introduced.

Spokeswoman Mona Patel said: "This is an important measure taken on a precautionary basis, especially in the light of scientific uncertainty that still exists about BSE.

"Hopefully this is a sign that the EU is learning lessons from the UK BSE crisis."


21 Nov 00 - CJD - French beef ban not necessary, says No 10

Staff Reporter

Times ... Tuesday 21 November 2000


There is no scientific evidence to support a ban on French beef, Downing Street said today.

The assurance followed reports that Tony Blair was given warning that a legal loophole could leave Britons at risk of inadvertently eating French beef infected with BSE .

The Agriculture Minister, Nick Brown, apparently highlighted the risk in a letter to the Prime Minister on Friday.

But Mr Brown had not called for a ban on beef reared across the channel and neither the Food Standards Agency or EU scientists thought it necessary, a No 10 spokesman said.

Asked directly if there were plans for a ban, he said: "No, for the simple reason that the scientific advice is not there to justify it."

However, the Shadow Agriculture Minister, Tim Yeo, has called on Mr Brown to face MPs and clear up confusion over the safety of French beef.

It is illegal to sell British beef (Mad Cow Correspondents note: but not French beef) from cows older than 30 months for human consumption in the UK.


21 Nov 00 - CJD - France 'plays politics' with CJD victims

By Patrick Bishop in Paris

Telegraph ... Tuesday 21 November 2000


The families of French CJD victims have rebuked President Chirac and his Prime Minister, Lionel Jospin, for trying to make political capital out of the BSE crisis .

Relatives of the country's only two officially identified victims of the human form of Mad Cow disease met both men yesterday. Earlier, the body representing sufferers condemned the political slanging match . The General Secretary of the Association for the Victims of Creutzfeldt-Jakob disease (AVMJC), Jeanne Goerrian, said the point-scoring was "indecent ".

The myth of mutual respect and co-operation between the easy-going Gaullist president and the stern Socialist prime minister has been exploded by the bitter exchanges as they scrambled for cover in the face of public anger .


21 Nov 00 - CJD - Blair pressed to ban unsafe French beef

By Valerie Elliott, Consumer Editor

Times ... Tuesday 21 November 2000


Tony Blair was under pressure last night to ban French beef products after a senior Cabinet minister warned him that BSE-infected beef from France could have disappeared into the food chain.

The alarm was raised with the Prime Minister by Nick Brown , the Agriculture Minister. He told him that there was a risk that beef aged over 30 months from France and other EU states could have reached the food market through lack of sufficient checks and controls.

Mr Brown wrote last Friday to Mr Blair and Alan Milburn, the Health Secretary. He warned them that older beef can be legally imported into Britain even though it is illegal to sell it for human consumption.

Legal loopholes have been identified which make it possible for older French meat to be cut and packed in other EU countries. It can then be imported and sold without British customers knowing its true origin.

Pressure for a ban is expected to grow because the Government cannot guarantee that older French beef is not reaching the dinner plate . The sale of old British meat for consumption is banned.

Some farmers and meat industry sources are convinced that this old meat could be reaching the poorer end of the market in pies and burgers , Chinese and Indian meals.

A senior government source confirmed that Mr Brown had raised the issue in talks with a number of colleagues privately and believed that he had to raise formally his concerns within the Government.

The Food Standards Agency (FSA) said last night that it had not been informed of Mr Brown's letter. A spokesman insisted, however, that it had investigated complaints and not yet found any evidence of any suspect meat being sold or fed to the public.

The agency admitted , however, that it had already asked local authorities to instruct trading standards and environmental health officers to step up checks on beef used at processing plants and on sale at small wholesale companies.

It also urged anyone involved in the beef trade to ensure that they held accurate documentation to show the origin of the meat and its age.

Last night Hugh Leman, of Cumberland Meat Packing, in Coventry, who raised concerns about the old meat to the FSA six months ago, reiterated his claim that more than a million tonnes of beef from old dairy cattle has been imported into the UK from the EU in the past five years .

Mr Leman was in contact with the FSA again yesterday but an agency spokesman said that he had so far refused to name any company involved in selling this illegal meat.

The Prime Minister has asked Mr Milburn, the Government's main BSE experts, the Spongiform Encephalopathy Advisory Committee, and the FSA for urgent advice on the risks to human health.

Legal options include a ban on all beef aged over 30 months entering Britain from France and the rest of the EU. If Sir John Krebs, chairman of the FSA, and scientific advisers say that there is a risk to human health the Government can ban or suspend imports without the consent of the European Commission.

Despite assurances from the FSA that there was no risk to human health because of the low incidence of BSE in France, Mr Brown was not satisfied and wrote to Mr Blair.

The Agriculture Minister has maintained his boycott of French beef and other products because of French refusal to accept British beef exports under the date-based export scheme.

But he is concerned that there is a law in Britain that states that beef aged over 30 months should be banned from human consumption and yet there are no rigid enforcement controls in place .

The FSA has admitted that it had no idea where this older meat is going and has also told ministers that checking the age of the meat is difficult, especially if it has been processed into pies , burgers or ready meals .

Mr Brown believes that the public would be appalled if they were being sold meat that failed British safety checks. He has made clear to other ministers that he believes that diplomatic niceties should be ignored if there is any risk to consumers.


20 Nov 00 - CJD - Tories to demand facts on French beef

By Benedict Brogan, Political Correspondent

Telegraph ... Monday 20 November 2000


Ministers face a challenge under open government rules this week if they fail to publish the advice they have received from the Food Safety Agency on the risks posed by French beef .

The Tories said last night that they would apply to the Whitehall Ombudsman for access to all the confidential information passed to the Ministry of Agriculture about the dangers of the BSE outbreak in France.

Tim Yeo, the shadow agriculture minister, has written to Sir John Krebs, the head of the FSA, to urge him to publish full details of the advice he has given the Government in the wake of the beef-on-the-bone ban imposed by France, and Italy's announcement on Friday that it will block imports of French beef.

Mr Yeo said he would press ministers to live up to the transparency they promised when the FSA was introduced earlier this year.

In the Commons last week Nick Brown, the Agriculture Minister, said that the FSA had ruled that there were "no health reasons" for banning French beef imports. But Mr Yeo said last night that the advice could be out of date in the light of the French decision to ban the sale of T-bone steaks and the consumption of beef in schools.

He said unless up-to-date information was released he would apply for access to minutes and briefing papers under the terms of the Code of Open Government introduced under John Major.


19 Nov 00 - CJD - Fourth French person may have CJD

Ananova

PA News ... Sunday 19 November 2000


A fourth person in France may have caught the human version of so-called Mad Cow disease, a newspaper has reported.

The Journal du Dimanche quoted a doctor at Wertheimer Hospital in the south eastern city of Lyon as saying that a seriously ill 45-year-old female patient was being tested for the disease.

The woman, who is in a semi-comatose state, has been hospitalised for six weeks under the care of Dr. Guy Chazot, the hospital's chief of neurological services. Chazot identified the first case of Mad Cow in a human being in France in 1996.

According to Chazot, the diagnosis could not be confirmed until a post-mortem examination was conducted, the newspaper said.

Experts believe people contract the human form of Mad Cow disease, or variant Creutzfeld Jakob Disease, by eating infected beef. Two people have died from the disease in France and a third is believed to be dying from it.

Both the cow form, bovine spongiform encephalopathy, and the human form are varieties of a rare group of brain-wasting diseases known as transmissible spongiform encephalopathies. Such illnesses cause microscopic holes in the brain. There is no cure .

The Journal also published a full-page advert by the French government assuring the public of the safety of beef. The ad included a hotline number that people may call for information on the disease. According to Europe-1 radio, the hotline received 700 calls in the first 13 hours of operation.


19 Nov 00 - CJD - Doctor denies French CJD fears

Ananova

PA News ... Sunday 19 November 2000


A French doctor has denied newspaper reports that a fourth person in France may be suffering from CJD but admitted he said the patient had showed some initial symptoms of the disease.

The Journal du Dimanche reported that a seriously ill 43-year-old female patient was being tested for the disease at Lyon's Pierre Wertheimer Hospital, where she has been hospitalised for six weeks and is in a comatose state.

Dr Guy Chazot, the hospital's chief of neurological services, said at a press conference that the patient initially showed problems involving memory and behavior and has been tested for the disease.

However, Chazot said he did not believe the patient had contracted the deadly brain-wasting illness and that such a diagnosis could not be confirmed in any event until a post-mortem examination was conducted.

Chazot identified the first case of Mad Cow in a human being in France in 1996.

Experts believe people contract the human form of Mad Cow disease - or variant Creutzfeld Jakob Disease - by eating infected beef. Two people have died from the disease in France and a third is believed to be dying from it.

Both the cow form, bovine spongiform encephalopathy, and the human form are varieties of a rare group of brain-wasting diseases known as transmissible spongiform encephalopathies. Such illnesses cause microscopic holes in the brain. There is no cure.

The Journal also published a full-page advert by the French government assuring the public of the safety of beef. The ad included a hotline number that people may call for information on the disease. According to Europe-1 radio, the hotline received 700 calls in the first 13 hours of operation.


19 Nov 00 - CJD - Italian farmers blockade French beef

Ananova

PA News ... Sunday 19 November 2000


Dozens of Italian farmers are heading to the border with France to make sure no French beef crosses into their country after most imports were banned to prevent the spread of BSE .

Agriculture Minister Alfonso Pecoraro Scanio has called on the protesters to stay calm .

Italy imported some 40 percent of its beef from France until Friday, when Rome banned most beef imports following an increase in detection of the brain-wasting animal disease in France.

Some 50 farmers and cattle raisers from the Piedmont region which borders France headed to the frontier point of Ventimiglia to hand out leaflets and be on guard for any trucks that might be arriving with meat from France.

The Italian news agency ANSA, reporting from the border town, said the protest was calm and that few trucks of any kind had crossed the border because most trucks don't make their runs on Sundays.

"In the middle of all this confusion, we maintain that a blockade of border crossings is the only useful tool to safeguard both the interests of consumers and those of cattle raisers," said Marco Favaro, head of a group of Italian beef producers.

Italian cattle have largely been spared the Mad Cow problem, with the only two cases that of two animals imported from Britain a few years ago.

The protesting Italians invited the agriculture minister to join them in their demonstration but the minister said in a statement he would do better to go to Brussels, where a European Union meeting of farm ministers was set for Monday.

"I understand the agitation" of the breeders, the minister said, "but law enforcement agencies and authorities will apply the order of the health ministry" Friday banning the French imports.


19 Nov 00 - CJD - Déjà vu for BSE as French fail to learn from British mistakes

By John Lichfield in Paris

Independent ... Sunday 19 November 2000


On Monday and Tuesday, both lunchtime and evening, the Hippopotamus Grill in the Place de Ternes near the Arc de Triomphe was deserted. "En plein psychose de boeuf" (in the midst of the beef panic) no one wanted to eat in a child-friendly restaurant famous for its succulent steaks.

On Wednesday night, we took the entire family. I was the only one to insist on eating steak. But, surprise, surprise, the restaurant was three quarters full. I asked the waitress to explain the difference.

"Yesterday, the Prime Minister announced some new measures on BSE. It seems to have persuaded some people that they can eat beef again . None of the new measures will take effect for days." She might have added that they won't affect the food chain for years . "It is bizarre but..." She shrugged her shoulders.

Bizarre ? But then the whole crisis is bizarre. French families are suing British ex-ministers; the Italians are banning French beef. We are suing the French for not admitting British beef (which the Italians are, in theory, eating).

The French public is eating 50 per cent less beef than it did three weeks ago. Why? Because 12 animals were declared to be Mad Cow suspects after they had already reached the supermarket shelves. They were, in fact, almost certainly healthy.

The French President, Jacques Chirac, accuses the Italians of bad faith and demagoguery; the French Prime Minister, Lionel Jospin, accuses President Chirac (who helped to foment the BSE panic in France when it suited him to embarrass Jospin 10 days ago) of demagoguery and bad faith.

Tomorrow EU agriculture ministers meet in Brussels to try to sort out this poisonous - and irrational - beef stew. Good luck to them.

The crisis is irrational to the extent that, in Britain or in France, you probably have less chance of being infected by a Mad Cow than at any time in the past 12 years. This is a political crisis rather than a health crisis.

The damage that was done to the known, and yet to be discovered, CJD victims was almost certainly done years ago, before the potentially damaging parts of cows were removed from the food chain. The average incubation periods of both BSE and CJD are unknown but most scientific findings point to an average period of 10 years .

Nonetheless; the Third European Mad Cow Crisis - after those of 1996 and 1999 - may turn out to be the most politically explosive of them all.

French farmers are not like British farmers. They will not suffer in silence the kind of - mostly undeserved - suffering imposed on beef and dairy British farmers in the past six years. If the Italian boycott holds, or spreads, and French domestic beef sales fail to pick up, the next stage in the crisis is predictable: there will be French farmer barricades on the Italian and Spanish borders and, possibly, at the Channel ports and the tunnel.

The crisis will be all the more difficult to resolve because it is based on a series of misunderstandings , half-truths and frequent misreporting of Mad Cow science which is barely understood by the scientists. It is also rooted in a bizarre approach to the BSE crisis by the French government, which imposed rules that were stricter than were necessary in some areas but weaker than was sensible in others.

For instance, Paris refused, until last Tuesday, to impose a complete ban on the use of ground-up cattle in all animal feeds, despite evidence, from our own mistakes in the early 1990s, that a partial ban (allowing cattle to be fed to pigs and poultry) would be useless. The steady but still slow increase in BSE in France is traced by the government's own vets to the "cross-over" use of pig and poultry feed to fatten cattle.

On the other hand, the French government insists on slaughtering all the animals in a herd where a case of BSE has occurred, despite the evidence that this is pointless. BSE is not infectious like foot and mouth disease.

There have been 181 cases of BSE in France to date - just more than 100 this year. In all the thousands of animals in the 181 massacred herds, only one extra cow has been found to have BSE.

It is this unnecessary rule which started the present crisis. An animal tested positive for BSE at a Norman abattoir last month. Its 12 herd-mates had already been butchered and sent out to the shops. The evidence suggested that the other animals were healthy but French officials insisted that the meat had to be recalled. One French newspaper ran the irresponsible headline "Mad Cows in our supermarkets " and the crisis began.

Serve the French right for banning limited imports of UK beef, declared safe by the EU, a year ago? Maybe. But who gave BSE to France in the first place? It is accepted, on both sides of the Channel, that the BSE in French cows came originally from infected cattle feed imported from Britain. Given the incubation period for both BSE and new variant CJD, the three (and possibly four ) CJD victims in France probably caught the disease from low-quality British hamburger beef , which was exported to France in large quantities before 1996.

The legal action brought by two families of French CJD victims on Friday seeks to blame British , French and EU officials in the period from 1986 to 1996 for allowing BSE to take hold in the French cattle herds. The families point out that the British government banned cattle feed containing cattle remains in 1988. But British firms were allowed - or allegedly encouraged - to dump the feed cheaply on the continent, especially in France.

Evidence to substantiate such claims may be difficult to find. The chances of Conservative ex-agriculture ministers such as John Gummer and Douglas Hogg appearing in a French court are virtually nil . But the court case serves as a reminder that the rampant Schadenfreude in some parts of the British press, and especially in the Conservative party, is utterly misplaced.

The French epidemic, still growing, is part of the 700-times larger British epidemic , now, thankfully, fading.


19 Nov 00 - CJD - Butchers Call For Ban On French Beef

From Ananova

Guardian ... Sunday 19 November 2000


Butchers in Scotland are calling on the Government to ban meat imports from France following the discovery of dozens of cases of Mad Cow disease.

Wilson Ferguson, outgoing president of the Scottish Federation of Meat Traders' Associations, says the billions of pounds spent on eradicating BSE in the UK could be wasted if French beef is allowed into the country.

Countries including Italy and Spain have imposed unilateral bans on imports of French beef after tests discovered 80 cases of the brain-wasting disease in cattle.

On Friday the families of two French victims of variant Creutzfeldt-Jakob disease (vCJD), the human form of BSE, filed lawsuits accusing authorities in London, Paris and Brussels of causing their infection by failing to stop the disease entering the human food chain.

Speaking at the organisation's Annual General Meeting, Mr Ferguson said: "Our industry spends an enormous amount of time and money complying with legislation to ensure that a safe and wholesome product is available for consumers, and yet this Government continues to allow imports of meat from countries who do not supply with the same standards of legislation we do .

"It would be common sense to ban all imports of meat and meat products from countries in Europe and further afield who do not have in place the legislation and controls in existence in this country."

Mr Ferguson also criticised the Food Standards Agency Scotland for not consulting his organisation before introducing new licensing rules for butchers' shops.

The Butchers' Licensing Regulations, which require all butchers to have a government license before they can sell raw and cooked meats on the same premises, came into force at the start of October.

Mr Ferguson said: "The enforcers, be they government, Food Standards Agency or local authority, have seen fit to take little advice from the Federation, for whatever reason.

"They have bickered and dithered amongst themselves and the trade has been caught in the middle."


18 Nov 00 - CJD - Italy bans French beef because of crisis over BSE

By Bruce Johnston in Rome

Telegraph ... Saturday 18 November 2000


Italy banned the import of French beef last night because of the BSE crisis .

Italy is France's biggest export market for beef and the authorities described the ban as a selective one. The latest move includes a ban on adult French cattle, and French beef on the bone, as well as a ban on the use of feed that contains animal derivatives.

In recent weeks, diplomatic relations between Rome and Paris have been frosty , particularly after the Italian authorities urged the European Union to take concrete action over the beef crisis. Alfonso Pecorario Scanio, the Agriculture Minister, said after a cabinet meeting that the ban would take immediate effect and last three months.

In addition, all cattle in Italy originating from France which were under 18 months old will now have to be slaughtered , after first being tested to determine whether there were traces of prion, which indicated a future BSE infection. A spokesman for Mr Scanio said that, from next January, Italy would begin testing all 800,000 cattle in the country for signs of prion.

It was estimated that the operation would cost Italy at least £30 million . The ministry spokeman added: "Italy will also be reiterating its request for Brussels to take steps to contain the BSE problem, by banning the export of beef from those countries in Europe."

It is thought that Italy may be followed by Germany in the event that agriculture ministers fail to decide on concrete steps next week. Italy imports about half of its beef from France, and in view of BSE developments there, concern has mounted in the light of official Italian figures, which show that deaths from Creutzfeldt-Jakob disease in the country have doubled from 36 to 72 since 1993.

In the wake of French measures to address the BSE crisis there, hundreds of towns and cities in Italy, including Rome, Milan, Florence, Venice and Siena, banned the use of beef in school meals earlier this week. The measures prompted an immediate and, according to butchers and shopkeepers, unjustified collapse in sales of beef in general in shops and supermarkets throughout the country.

In Florence, which like all of Tuscany has a strong tradition of eating beef, the Esselunga supermarket chain complained that beef sales were 50 per cent down, and that French beef could "not be given away ". Italian authorities maintain that the problems experienced in France could never be repeated in Italy, where there are said to much stricter controls, although an inspection team sent by Brussels was of a different opinion.

The European Union yesterday granted special financial aid to private beef stocks amounting to £275 a ton to support beef markets against a possible Mad Cow scare. The aid will be re-evaluated after three months.


18 Nov 00 - CJD - French families sue Britain over vCJD

Jon Henley in Paris

Guardian ... Saturday 18 November 2000


The families of two French victims of CJD, the fatal human variant of Mad Cow disease or BSE, filed a lawsuit in Paris yesterday against French , British and European authorities , alleging poisoning and involuntary manslaughter .

As Italy announced a partial ban on French beef, the parents of Laurence Duhamel, 39, who died of new variant CJD in February, and Arnaud Eboli, 19, a suspected sufferer, said Britain, France and the EU had failed to adequately protect their citizens.

"People who smoke and drink do so by their own choice. All my son did was eat meat, and he is going to die," said Dominique Eboli outside the court. "What we want now is for measures to prevent this from happening again."

The 100-page suit, the first of its kind related to CJD in France, was lodged "against persons unknown", a legal procedure that requires judicial experts to establish whether there is a case to answer, and if so, who should appear in court as defendants. Lawyers said Britain was targeted because of claims that it had effectively exported BSE, and hence nvCJD, to France. French farmers claim at least 14,000 tonnes of meat-and-bone meal banned in Britain were imported into France in the early 1990s. A spokesman for Tony Blair said there had been no formal notification of any action against the government.

The French government this week banned T-bone steak and other cuts of beef on the bone, and suspended the use of meat-and-bone meal in all animal feed. Sales have fallen 40% since three supermarket chains admitted last month that they had unknowingly sold potentially contaminated beef.

A hospital in Paris said yesterday that it had detected a fourth possible CJD case, a woman of 40 . At least 80 cases have been diagnosed in Britain.

Three French ministers stood trial last year after allowing the transfusion service to use blood that eventually infected some 4,000 people with HIV.


18 Nov 00 - CJD - French may charge the Tories over CJD deaths

By John Lichfield in Paris

Independent ... Saturday 18 November 2000


French families have filed a ground-breaking lawsuit in Paris which, in part, accuses British Conservatives of spreading BSE

The families of two French victims of the human variant of Mad Cow Disease yesterday brought a legal action for manslaughter and "poisoning " against two former British Conservative governments.

If a French magistrate decides that there is a criminal case to answer, British ministers or senior officials , from the Margaret Thatcher and John Major governments, could, in theory, be summoned before a French court .

British officials in Paris believe it is unlikely that the case will go that far, but legal documents on the case have been sent to London for expert advice.

The families of the French victims of CJD have formally accused unnamed British , French and European Union officials, from the period between 1986 and 1996, of "manslaughter, poisoning and placing the lives of others in danger". They accuse the "agents of British institutions " of allowing, or even encouraging , the export of UK cattle meat and bone meal to France, after it was banned in Britain in 1988 as the near-certain cause of the British BSE epidemic. They also accuse the Thatcher and Major governments of encouraging the export of British beef offal to France, even though its human consumption had been forbidden in the UK.

French and EU officials are accused of allowing both trades to continue , even though they knew, or should have known, that there was a serious risk to human health.

The case, if accepted, would break new legal ground in France. There is no precedent for a French criminal action against decisions taken by another European government. French courts have, however, allowed criminal actions against non-European governments, including, recently, an accusation that the Libyan leader Col Moammar Gaddafi ordered a terrorist attack on a French airliner.

During the time covered by the French legal action, John Gummer and Stephen Dorrell were the agriculture ministers in the Thatcher and Major governments.

The legal action marks a further stage in the anxiety , bordering on hysteria , surrounding BSE in France over the last two weeks, which is now communicating itself to other EU countries. The Italian government, faced with its own consumer revolt, defied EU rules yesterday and imposed a unilateral ban on imports of French beef on the bone. Since most beef is transported on the bone, this amounts to a near-total ban on French beef.

Spain and Austria have already banned imports of French live cattle.

The French government is hardly well-placed to object, having taken an equally illegal decision to ban the limited imports of "young" British beef, which were declared safe by the EU a year ago.

Nonetheless, President Jacques Chiirac protested against the Italian decision yesterday, accusing Italy of having BSE in its own cattle herds without admitting it. He said that there were no Italian cases because there were no Italian tests.

The suit calling for criminal action against British, French and EU officials for their actions - or failures to act - in 1986-1996 has been brought by the families of two of the the known French victims of new-variant CJD. (A fourth possible case in France is under investigation.) Lawyers for the families of Laurence Duhamel, 36, and Arnaud Eboli, 19, base their case on the steep increase after 1988 in the sale to France of British animal feed, containing the ground-up remains of British cows.

Although this feed had been identified as the likely cause of BSE, and banned in Britain, 12,500 tonnes were exported to other EU countries (mostly France) in 1988 and 25,000 tonnes in 1989. The trade continued, nominally to feed pigs and poultry, up until 1996.

British officials point out that there was nothing illegal about these exports. There was no French ban, at that time, on imports of meat and bone meal from Britain.

The moral case against the UK is stronger . It is generally accepted that British exports of suspect cattle fodder - both before and after it was banned in the UK in 1988 - caused the smaller epidemic of BSE now causing intense consumer anxiety in France. A rash of cases this week has brought the number of BSE attacks in France this year to just more than 100 , compared to 31 last year. At the height of the UK epidemic in 1992, there were 32,000 cases , or 80 each day .

*First it was only mad dogs and Englishmen, but now even Germans who might have eaten too many British hamburgers have been branded a health risk. The German Red Cross confirmed yesterday that Germans who have spent six months in Britain between 1980 and 1996 may not donate blood .

The decision, welcomed by the Health Ministry in Berlin, follows recommendations by the prestigious Paul Ehrlich and Robert Koch Institutes. Their scientists based their verdict on fresh research in Britain indicating that CJD could be transmitted through the blood.


17 Nov 00 - CJD - Ex-ministers May Face Charges In French CJD Cases

From Ananova

Guardian ... Friday 17 November 2000


British former government ministers may find themselves facing charges in a French court after the families of two victims of the human form of BSE filed a suit accusing Britain of causing their illness.

The families hope that a judicial inquiry will be carried out by the judge who put France's ex-Prime Minister Laurent Fabius in the dock for manslaughter after a health scandal involving HIV-contaminated blood.

The suit, filed at Paris County Court, alleges that the British , French and European authorities all bear responsibility for poisoning , involuntary homicide and endangering the lives of others .

The case comes amid growing concern over the spread of BSE in France, with Italy following Spain in announcing a unilateral import ban on most French beef, and Germany and Greece considering similar action . Berlin also threatened to reimpose its import ban on British beef products.

European Union farm ministers meet on Monday to consider moves to halt the spread of BSE, following the discovery of more than 100 cases in French cattle and the announcement by a Paris hospital of the country's fourth suspected case of variant CJD, the human form of the illness.

Both Germany and Greece have threatened unilateral embargoes on French beef if no EU-wide export ban is imposed on Monday.

But Agriculture Minister Nick Brown yesterday rejected a Tory call for a precautionary ban, telling MPs the Food Standards Agency had advised that there were "no health reasons" for it.

The court case is brought by the families of Laurence Duhamel , a 36-year-old woman who died from vCJD in February, and 19-year-old Arnaud Eboli , who is critically ill.

The families' lawyer, Francois Honnorat, presented a 100-page dossier alleging that authorities in London, Paris and Brussels were to blame for failing to stop BSE entering the human food chain.

Downing Street has refused to comment on the case, saying that the British Government had not yet been officially notified of any action.


17 Nov 00 - CJD - French victims of CJD to sue Britain and EU

By Steve Connor, Science Editor

Independent ... Friday 17 November 2000


The families of two French victims who developed the human form of "Mad Cow" disease plan to sue Britain for its role in allegedly allowing bovine spongiform encephalopathy (BSE) to enter the human food chain.

François Honnorat, lawyer for the families of 36-year-old Laurence Duhamel, who died last February, and 19-year-old Arnaud Eboli, who is critically ill, said he would file his case today alleging that Britain, France and the European Union were all culpable.

The 100-page lawsuit accuses Europe and the two governments of failing in their duty to ensure food safety and of opposing changes to the law governing the controls over beef during a 10-year period between 1986, when BSE was first identified, and 1996, when it evidence emerged that it had crossed into humans.

Honnorat is filing a "complaint against persons unknown" that judicial experts will study to establish whether there is a case for a full-blown legal inquiry that might be brought to the courts.

"The plaintiffs' suit covers a whole range of issues showing the lack of action taken to limit the BSE epidemic and the human consequences, at the level of the French authorities, those of the European Union and also in Britain," Honnorat said.

France was the biggest export market for British beef before it was banned in 1996. It is widely accepted that many cows infected with BSE were sent abroad at the height of the epidemic at the end of the 1980s, although France is now known to have its own indigenous BSE.

The ground-breaking legal action comes as France's political leaders struggle to contain further panic over BSE, which scientists believe has caused the variant form of Creutzfeldt-Jakob disease, a deadly brain-wasting disorder of humans.


17 Nov 00 - CJD - Italy bans French beef imports

Staff Reporter

BBC Online ... Friday 17 November 2000


Italy has banned imports of adult cows and beef on the bone from France as fears over "Mad Cow" disease spread.

The move follows revelations that several tonnes of meat from a BSE-infected herd had gone on sale in French supermarkets. Italy is France's biggest customer for meat and dairy exports, importing about one million head of cattle a year.

France has itself banned beef on the bone and Spain , Russia , Poland , Hungary and Austria have announced their own restrictions on French beef, but the European Union has not so far placed restrictions on exports.

Italy's move came as relatives of two French victims of the human form of the brain-wasting disease, or variant Creutzfeldt-Jakob disease (vCJD), began legal action on Friday against the French and UK governments and the European Commission.

According to officials in Rome, no case of BSE has been detected in Italian cattle, nor were there any cases of vCJD in humans.

Nevertheless, Italy now plans to test cattle aged over 24 months for BSE, said Farm Minister Alfonso Pecoraro Scanio.

The French beef scare has already led more than 30 Italian cities to take beef off their school menus .

SALES SLUMP

Beef sales have dropped 10% , according to Italy's butchers' association.

Hundreds of French schools have already dropped beef from their menu, while sales of beef have slumped by 40% .

Germany said it had not yet made a decision on whether to join Italy, but said "all options were open ".

In the UK, where an outbreak of BSE led to a foreign embargo on British beef in 1996, the government was under pressure from the Conservative opposition to bring in restrictions.

In Paris, a lawyer acting jointly for 19-year-old Arnaud Eboli, who is suffering from vCJD, and Laurence Duhamel, who died from the disease last February aged 36, filed a suit in a civil court.

It accuses Britain of exporting possibly contaminated material , and France and the European Commission of failing to take the threat of disease seriously enough.

So far only two people are confirmed to have died of the human variant of BSE in France, compared with more than 80 deaths in Britain over the past decade.

But the number of BSE cases recorded this year in France has more than tripled since last year to 101 .

The President of the European Commission, Romano Prodi, on a visit to Athens, has tried to play down fears over the disease.

"Mad Cow disease exists and it's serious. One must take precautions but not panic," Mr Prodi said.

He said the commission had asked European Union member-states to conduct tests for BSE and would keep insisting on this, but it could not impose testing on national authorities.


17 Nov 00 - CJD - Governments sued over French CJD

Staff Reporter

BBC Online ... Friday 17 November 2000


Relatives of two French victims of new variant CJD - the human form of "Mad Cow disease" - are suing the French and British Governments and the European Commission .

A lawyer acting jointly for 19-year-old Arnaud Eboli, who is suffering from vCJD, and Laurence Duhamel, who died from the brain wasting disease last February aged 36, filed a suit in a Paris civil court.

It accuses Britain of knowingly exporting possibly contaminated material, and France and the European Commission of failing to take the threat of disease seriously enough.

A spokesman for the Commission said "it considers itself not guilty of any improprieties," but said a defence case would be drawn up.

FRENCH CASES INCREASE

France has just banned beef on the bone and suspect animal feed because of renewed fears about the disease.

Three times as many cases of BSE have been discovered in French herds this year as in 1999.

Consumer panic broke out after the revelation last month that eight tonnes of meat from a BSE-infected herd had gone on sale in French supermarkets.

Hundreds of French schools have already dropped beef from their menu, while sales of beef have slumped by 40% .

So far only two people are confirmed to have died of the human variant of BSE in France, compared with more than 80 deaths in Britain over the past decade.

But the number of BSE cases recorded this year in France has more than tripled since last year to 101 .

ITALIAN BAN

The BSE scare is already having repercussions across Europe .

Italy introduced a ban on Friday on imports of French beef from high-risk animals and of beef on the bone.

The German Farm Minister, Karl-Heinz Funke, also said he would take steps to ensure French animal feed "which is not considered safe in its own country" was not sold in Germany.

Meanwhile the Netherlands discovered the country's first case of BSE for 20 months on Thursday. An Agriculture Ministry spokeswoman said parliament had been informed.

The President of the European Commission, Romano Prodi, on a visit to Greece, tried to play down fears over the disease.

"Mad Cow disease exists and it's serious. One must take precautions but not panic," Mr Prodi said.

He said the Commission had asked European Union member-states to conduct tests for BSE and would keep insisting on this, but it could not impose testing on national authorities.


17 Nov 00 - CJD - Mad Cow To Be Discussed at EU Summit

Associated Press

Guardian ... Friday 17 November 2000


Paris (AP) - With fears over Mad Cow disease crippling beef sales in France, President Jacques Chirac promised Thursday to push the issue of food safety at next month's European Union summit.

The European Parliament, meanwhile, followed France's lead and urged the 15 EU members Thursday to ban feeds for all livestock containing meat from mammals - not just feeds for cattle.

France imposed a similar ban Tuesday. It also banned the T-bone steak, the second specialty to be slashed from the nation's menus in a week amid what many commentators describe as public hysteria over so-called Mad Cow disease.

France and the EU are hoping to protect the food chain from bovine spongiform encephalopathy, or Mad Cow disease. The brain-wasting ailment is suspected by scientists to be linked to a similar human malady, variant Creutzfeldt-Jakob disease.

Earlier EU measures banned giving cattle any feeds containing ground meat and bone meal from mammals. Scientists believe Mad Cow disease originated in Britain when cattle were given feed containing the ground remains of sheep infected with a brain ailment.

Under the agreement passed Thursday by the 626-member EU assembly in Strasbourg, France, giving such feeds to sheep , goats , poultry , fish and pigs would also be barred. The parliament decision is not binding on EU governments.

The EU summit Dec. 7-8 in Nice, southern France, marks the end of France's six-month presidency of the 15-nation group. What should have been a showcase for French diplomacy has been marred by the Mad Cow scandal.

Last month, it was discovered that potentially infected meat made it onto supermarket shelves . Many school districts have banned beef from their canteens, and sales have slumped about 40 percent in a nation renowned for its love of meat.

There has been a sharp rise in the number of cases of Mad Cow disease found among animals in France this year - some 90 compared to 31 last year. The EU has said that part of the reason is more rigorous testing of animals.

The French public's fears are all the stronger because of a string of recent food scares, including an outbreak of listeriosis connected to pork tongue in gelatin.

Many have also been reminded of the so-called ``tainted blood affair' ' of 1985, in which more than 4,000 people contracted the AIDS virus from blood transfusions. Many have since died, and several government officials stood trial over the affair.

Chirac met with farming leaders Thursday and promised to make food safety a key talking point at the summit, his spokeswoman Catherine Colonna said.

The head of France's largest farming union, Luc Guyau, said Chirac backed farmers' calls for an ambitious Europe-wide plan to supply enough vegetable proteins to replace animal-based feeds.

Also Thursday, a lawyer for the families of two French victims of variant Creutzfeldt-Jakob disease said the families planned to sue authorities in France and Europe for alleged poisoning.

The families argue that not enough was done to warn people of the dangers of beef, or to ban animal-based feeds as soon as the risks were apparent. One of the victims has died and one is very ill.

One other person has died of the disease in France, but is not involved in the court action. In Britain, more than 80 people have died from the disease.


16 Nov 00 - CJD - Nobody's genes can protect them from BSE

Staff Reporter

New Scientist ... Thursday 16 November 2000


Disturbing new evidence suggests that nearly three times as many people may be at risk from BSE than previously thought. So far, all victims of vCJD, the human form of BSE, have been from a minority of the population with a particular genetic make-up. This raised hopes that most of the population was immune to the infection.

But the discovery that a closely related disease called Kuru is more widespread among people in Papua New Guinea than previously thought, suggests vCJD may pose a threat to everyone exposed to the infectious agent that causes it.

According to vCJD expert and British government adviser John Collinge of St Mary's Hospital, London, this could indicate that the mean incubation period in people is 30 years or more . 'We may well see [cases] well into the second half of the century ,' he says.

So far, all the 88 victims of vCJD are believed to have inherited identical copies of a critical gene from both parents. The critical gene makes a protein called PrPC. This is the normal version of the rogue 'prion' protein which causes BSE in cattle and vCJD in humans.

At a particular site in the gene called codon 129, the victims have DNA which loads the amino acid methionine into the PrPC protein. Victims inherited the same gene variant from both parents, giving them this 'MM' combination. About 37 per cent of the population share the MM combination.

In other people, at least one of their two prion genes builds the amino acid valine into the PrPC protein instead of methionine. Most have the 'MV ' combination, while the rest are 'VV '.

But tests on 11 elderly victims of a different form of human CJD, called Kuru, suggest otherwise. Presented last week by Collinge at the Millennium Festival of Medicine in London, the findings show that all 11 of them had the 'heterozygous' or 'MV' combination, which is considered most protective .

Although Kuru is not the same as vCJD, the findings suggest that people with the MV combination can't assume they're immune to prion diseases. 'All these latest [cases] are heterozygous, and we're seeing lifetime incubation periods,' says Collinge, who is a member of the British government's Spongiform Encephalopathy Advisory Committee (SEAC).

The implication is that people with the 'protective' gene combinations are not immune from the disease. They'll simply have to wait longer for the disease to strike. Peter Smith, who chairs SEAC, agrees the new findings are cause for concern . 'It doesn't rule out the possibility that the incubation period is longer in people of that genotype,' he says.

Kuru has killed over 2500 members of the Fore tribe in Papua New Guinea in the past 100 years. The practice of eating dead relatives' brains as a mark of respect caused the rapid spread of Kuru from a single individual who probably developed it through a spontaneous gene mutation.

The practice was banned in 1957, and the number of cases had dwindled-until the latest, worrying emergence of new cases among the elderly.


27 Oct 98 - CJD - Phenotype-genotype studies in kuru: implications for vCJD

Cervenakova L, Goldfarb LG, Garruto R, Lee HS, Gajdusek DC, Brown P

Proceedings of the National Academy of Science ... 27 October 1998


Proceedings of the National Academy of Science U S A 1998 Oct 27;95(22):13239-41

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.

The PRNP polymorphic (methionine/valine) codon 129 genotype influences the phenotypic features of transmissible spongiform encephalopathy. All tested cases of new variant Creutzfeldt-Jakob disease (nvCJD) have been homozygous for methionine, and it is conjectural whether different genotypes, if they appear, might have distinctive phenotypes and implications for the future "epidemic curve" of nvCJD. Genotype-phenotype studies of kuru, the only other orally transmitted transmissible spongiform encephalopathy, might be instructive in predicting the answers to these questions. We therefore extracted DNA from blood clots or sera from 92 kuru patients, and analyzed their codon 129 PRNP genotypes with respect to the age at onset and duration of illness and, in nine cases, to detailed clinical and neuropathology data. Homozygosity at codon 129 (particularly for methionine) was associated with an earlier age at onset and a shorter duration of illness than was heterozygosity, but other clinical characteristics were similar for all genotypes. In the nine neuropathologically examined cases, the presence of histologically recognizable plaques was limited to cases carrying at least one methionine allele (three homozygotes and one heterozygote). If nvCJD behaves like kuru, future cases (with longer incubation periods) may begin to occur in older individuals with heterozygous codon 129 genotypes and signal a maturing evolution of the nvCJD "epidemic." The clinical phenotype of such cases should be similar to that of homozygous cases, but may have less (or at least less readily identified) amyloid plaque formation.

The distribution of homozygous methionine, homozygous valine, and heterozygous genotypes according to age at onset of kuru symptoms is shown in Fig. 1. It is evident that compared with the series as a whole, homozygous methionine patients are overrepresented in the younger age groups, and heterozygous patients are overrepresented in the older age groups. When the genotype frequencies of the group of children and young adolescents (<15 yr of age) were compared with adults >30 yr of age, the difference between homozygotes and heterozygotes was statistically significant (P = 0.0001), Table 1. A less impressive but still statistically significant correspondence also exists between the codon 129 genotype and the duration of illness, shorter illnesses being associated with homozygosity and longer illnesses being associated with heterozygosity (P = 0.006).

Nine specimens in the series were matched with patients whose brains had earlier been the subject of extensive neuropathological examinations (4). An extract of these data is presented in Table 2, along with clinical summaries and codon 129 genotypes. Irrespective of the age at onset or duration of illness, or of the codon 129 genotype, all nine patients had a clinical picture dominated by progressive locomotor ataxia associated with the shivering tremor characteristic of kuru. The occurrence of extrapyramidal signs, strabismus, dysphagia, and mutism was also unrelated to age at onset or duration of illness, or to the codon 129 genotype.

In regard to neuropathology, widespread gliosis occurred in all cases, associated with a variable pattern of neuronal abnormalities and vacuolation that was unrelated to genotype. However, amyloid plaques were noted only in the four patients with at least one methionine allele, and not in the five patients with only valine alleles. Except for the presence of plaques, the lone heterozygote was neuropathologically indistinguishable from either the methionine or valine homozygotes.

One of the more interesting features of the chromosome 20 PRNP gene is the phenotypic influence of polymorphic codon 129. Although not in itself pathogenic, it has been shown to influence susceptibility to iatrogenic and sporadic forms of TSE and to affect age at onset and duration of illness in familial TSE; in association with a pathogenic mutation in codon 178, the entire disease phenotype is altered (511).

Because all cases of nvCJD recognized to date have tested homozygous for methionine at codon 129, it is not known whether other genotypes (should they occur) will change the nvCJD phenotype, possibly even to the point of obscuring its distinctive clinical and neuropathological features.

One clue that this may not happen comes from experience with iatrogenic CreutzfeldtJakob disease, resulting from peripheral injection by contaminated human growth hormone, which shows that, other than a longer period of latency between infection and the onset of symptoms, the clinicopathological picture of CreutzfeldtJakob disease in patients with a heterozygous codon 129 genotype does not differ from that in patients with either methionine or valine homozygosity (ref. 12; D. Dormont, personal communication; M. Preece, personal communication).

An even more appropriate comparison comes from phenotype-genotype studies in kuru patients, in whom infection occurred, at least in part, by the oral route. Three recent studies comparing the neuropathological features of kuru and nvCJD include kuru cases in which the genotype of codon 129 was determined. In one study, the brains from two valine homozygotes showed a tendency for "proper plaque formation" in PrP-stained immunohistochemical sections (13). In another study, the brain from a valine homozygote showed numerous plaques by both histological and immunohistochemical staining (14). In the third study, brains from three valine and two methionine homozygotes had plaques visible by both histological and immunohistochemical staining, and no genotypic correlations were noticed (15).

In contrast to these studies, our series of nine cases showed a distinct correlation between the presence of the methionine allele and the presence of histologically recognizable amyloid plaques. It is possible that these differing results are a consequence of chance or of undefined differences in tissue fixation and staining methods. It is also possible that as a group, kuru cases with a valine allele at codon 129 have a comparatively reduced potential for plaque formation, and, by analogy, nvCJD cases with a codon 129 valine allele might not always show the prominent daisy plaque formation observed in methionine homozygotes.

Our finding that homozygotes have an earlier age at onset (and thus probably a shorter incubation period) than heterozygotes, but show a generally similar clinical phenotype, is in keeping with the effect of codon 129 homozygosity in other forms of TSE, and, if applicable to nvCJD, would predict (i) that codon 129 nvCJD heterozygotes will not pose a problem in recognition or diagnosis and (ii) that the appearance of an increasing proportion of heterozygotes may signal that the nvCJD epidemic is already evolving beyond its "leading edge," and thus provide a more solid foundation for predictive modeling studies of its overall extent and duration.


16 Nov 00 - CJD - French Families To Sue UK After Men Develop CJD

PA News

Guardian ... Thursday 16 November 2000


Families of two French victims of variant CJD are planning to sue the British Government for allegedly causing their infection, according to French newspapers.

In a civil case backed by the French Association of CJD Victims, the families plan to accuse the British, European and French authorities of "poisoning ", reports Le Monde.

They will charge the authorities in London, Brussels and Paris with having failed to do enough to ensure the safety of beef over the past 10 years. Papers prepared for the case allege that the British authorities bear a "heavy responsibility" for allowing the export of cattle and meat and bone meal.

It is also alleged that the French government undermined public safety by trying to persuade its people that BSE, Mad Cow disease, was confined to the UK.

The case is being brought by the families of Laurence Duhamel , who died aged 36 in February this year, and Arnaud Eboli , 19, who is currently critically ill with vCJD the human form of BSE.