Victim Status Update
Unusual UK victims of CJD in the past three years
CJD in the UK from 1985 to May 1996
English physicians still out of the loop on v-CJD
Bad News on Mouse Bioassay -- more beef organs tainted?
Percent drops in beef consumption in Europe since BSE crisis
French villagers cheer farmers on mad cow march
Protesters' cows graze on Champ de Mars
French send calves to British abattoirs for EU handout
Retailers balk at BSE-free beef plan
Sheepish about safety ... unsustainable agriculture
Farmers in France stage night raids to block British beef
BSE in Germany
Prion accumulation in spinal cords of patients with sporadic and growth hormone CJD
Tissue handling in suspected CJD
Transplantation of corneal tissue from donors with diseases of the CNS

Victim Status Update

Listserve correspondent ... 29 Aug 96

"I've been going through some newspaper articles trying to collate information about the vCJD cases that were the basis for the government's statement on March 20. (I assume everyone recalls that date.) The reports I've seen show some inconsistencies and don't add up to 10 names. They include:
 
Vicky Rimmer, 18, North Wales
Stephen Churchill, 19, Wiltshire
an anonymous 16-year-old girl of Turkish-Cypriot extraction
Michelle Bowen, 29, Manchester
Jean Wake, 38
Fonnie Van Es, 44, Banbury
Christine Hay, 46, Australia
Maurice Callaghan, 30, Belfast, Ireland
Anna Pearson, 29, Kent
Ann Richardson, 41
Ken Sharpe, 42, Liverpool
David Tipping, Wiltshire
Ann Harness, Suffolk
Gwendoline Lawrence, 64, Clwyd
Kevin Morrison, 25, Aberdeenshire
Peter Hall, 20
This adds up to 16 names. They were all mentioned in newspaper reports about vCJD in the days after March 20. However, other newspaper reports indicate that David Tipping actually contracted his case of CJD from treatments of human growth hormone, and Fonnie Van Es is apparently considered a normal "spontaneous CJD" case rather than a case of vCJD. After eliminating them, that still leaves 14 names.

Can anyone comment to me on this list? Which of the names listed don't belong among "the 10"? Which ones have been confirmed as vCJD by the government? Also, what's the current status of Vicky Rimmer? The last report I've seen indicated that she was still alive. Is this still the case? Finally, are there other confirmed or suspected vCJD cases not on this list, or not among "the 10"?"


Unusual cases of CJD in the past three years

(* = variant - CJD)(? = CJD unconfirmed) (# = occupation)(i=iatrogenic)
#1. Dairy farmer Peter Warhurst. Died March 1993. #2. Dairy farmer Mark Duncan Templeman Died July 1993. *3. Vicky Rimmer, 18-year old kennel worker from North Wales. Fell ill in 1993. CJD diagnosed by brain biopsy. Still in a vegetative coma. First known British teenage victim of CJD. i4. Stewart Smith, a plumbing salesman from Pattishall, Northamptonshire. Died October 1993, aged 30. Contracted CJD through growth hormone treatment received as a child. *5. Stephen Churchill, aged 19. Died May 1995. #6. Unnamed farmer, died September 1995. *7. Michell Bowen, 29, dies November 1995, six weeks after giving birth; CJD suspected. #8. Jean Wake, a 38 year old meat-chopper in a pie factory from Washington, Tyne and Wear. Died November 1995. *9. Maurice Callaghan, 30, of Belfast died 1995. *10. Unnamed 17-year-old girl who had eaten cow's brain in Cyprus, died 1995. *11. Ann Richardson, 41, health care assistant and mother of one died January 1996. #12. Abattoir worker Leonard Franklin died February 1996. *13. Peter Hall, 20, enviromental studies student at Sunderland University, died February 1996. *14. Anna Pearson, 29, solicitor from Canterbury, died February 1996. *15. Businessman Ken Sharp, 42 of Childwall, Liverpool, started showing signs of CJD in April 1995. Died March 1996. Suspected new cases being investigated [through 1 May 1996 only]: *? 16. Unnamed 27-year-old, still alive *? 17. Middle-aged man in North Yorkshire, still alive *? 18. Unnamed 36-year-old, still alive *? 19. Unnamed 42-year-old *? 20. Barry Baker, 29, woodcutter from High Halden nr Ashford, Kent, d. 2 June 96 *? 21. Graham Brown, 36, fireman from Ashford, Kent ? 22. Betty Bottle, 58, from Ashford, Kent. Her sister is thought to have died of CJD aged 51; the cause may have been genetic. *? 23. Helen Rutherford, 15, Glasgow area; Calif Tech test diagnosis, still alive # 24. A fourth dairy farmer who had been in contact with cattle affected by BSE has died of Creutzfeldt-Jakob disease. The man, 59, was admitted to hospital last September suffering from disturbance of his vision and died a month later, the Aug 30 1996 Lancet reports. A post-mortem examination confirmed he had a form of sporadic CJD which is distinct from the new variant of the disease that has been linked with BSE in cattle.

CJD in the UK from 1985 to May 1996.

From July/August's Public Health News
Year Referrals * Deaths of definite and probable cases
Sporadic Iatrogenic Familial GSS V-CJD Total
1985 ----- 26 1 1 0 ----- 28
1986 ----- 26 0 0 0 ----- 26
1987 ----- 23 0 0 1 ----- 24
1988 ----- 21 1 1 0 ----- 23
1989 ----- 28 2 1 0 ----- 31
1990 52** 26 5 0 0 ----- 31
1991 75 32 1 3 0 ----- 36
1992 96 44 2 4 1 ----- 51
1993 78 37 4 2 2 ----- 45
1994 115 53 1 2 3 ----- 59
1995 79 33 4
1 2 3 43
1996 (end of May) 46 12 0 0 1 6*** 19

* A simple count of all cases referred from a variety of sources, including suspected cases. About 50% of cases referred in the past have turned out not to be CJD. There is no read-across between this column and the deaths columns.

** The CJD Surveillance Unit was set up by the Department of Health and the Scottish Home and Health Department in May 1990.

*** Plus 2 definite cases of V-CJD still alive; total number of confirmed cases of V-CJD: 11.


CJD families win court battle In the UK families of people who died of CJD after receiving growth-hormone treatment as children won their 6-year battle against the Department of Health. The High Court judge ruled that the Department of Health should have acted on warning of CJD being contracted and stopped using the treatment after July 1977, when it took over responsibility for it from the Medical Research Council (MRC). The judge ruled that the Department had not been negligent between 1958 and 1977 and cleared the MRC of any blame. Of 2000 children who received growth hormone treatment a total of 16 have died and 3 are known to have the disease. Another 200 actions are pending, some of which claim mental injury because of fear of the disease. A damages claim was mounted for those treated after 1977 and a campaign was launched for ex-gratia payments form public funds for those treated before 1977. Financial Times 20-21.7.96, p4

Bad News on Mouse Bioassay

CHARLES ARTHUR
Science Editor, The Independent for 30 Aug 1996 [not used]

I think that the most interesting point in the latest Nature was a throwaway line in David Skegg's article: saying that the mouse bioassay is not sensitive enough. This is self-evident, he says, because it can't determine which tissue is the cause of maternal infection. But maternal infection happens. Therefore the mouse is not a sufficient indicator of tissues' infectivity.

People generally look to science to offer an unequivocal answer to questions. How inconvenient, then, to have to say that in the matter of the culling of cattle aged over 30 months, science can offer powerful arguments both for and against halting the scheme right now.

The arguments in favour appear in the careful calculations of the team of Oxford scientists, who yesterday reported that the BSE epidemic will die out in 2001 if there is no cull; and at virtually the same time if there is. For every case of BSE averted by the cull, they reckon, 45 cows will die needlessly. The government estimates that the cost of the scheme will be 600 million in the first year, and 500 million in each subsequent year.

But some disturbing data has also emerged in the past month. BSE can be transmitted from cow to calf. How? We don't know. This, says David Skegg, of the University of Otago in New Zealand, pushes us towards some uncomfortable facts. None of the tissues that might carry the disease from the mother cause BSE when injected into mice. But some must carry it; otherwise the calf could not catch it.

Therefore, injecting cow tissues into mice is not a sensitive enough way of finding out whether the BSE agent is present. However, all the government's assurances about the safety of beef and other tissues such as liver and lungs - yesterday's latest putative BSE-carrying villain - are based around the assumption that mice are just like humans in their sensitivity to BSE. Other experiments with monkeys imply they are not. The scientists advising the government have tried to make this point repeatedly, but the politicians don't want to hear them. "It's true," says Jeff Almond, of the Spongiform Encepalopathy Advisory Committee. "If al the tissues are genuinely BSE-negative, then you couldn't have maternal transmission." But you do: up to 10 per cent of calves born to BSE-infected mothers develop the disease.

We can hear the wind of logic blowing through this house of cards. Beef might not be "safe" after all - though the rapid drop in the incidence of the disease means that at least fewer cows harbouring the disease are reaching our dinner plates. And if there is one measurement in all this which is even harder to fathom than the risk posed by BSE to humans, it is the extent to which British consumers - ostensibly the object of all these policies - are really worried.

In all of this, my sympathies lie with the farmers. They have often been depicted as the creators of the BSE epidemic, who somehow "fed cows to cows" - as if they hung the carcasses up in their farmyards to be consumed by the herd, like a fox before hounds. Quite untrue; many are people vainly trying to make a living from this 10,000-year-old profession, who are beset by the pressures of the modern world with its insistence on economy, efficiency and productivity. Nobody could blame the farmers for taking the cheap option of using the protein cakes from the meat and bone meal manufacturers. Those are the companies to blame, if blame has to be laid. They too will feel the pinch if the cull goes ahead.

The number of infected cows consumed, by the way, averages out to about 1 in every 50 over the past ten years in Britain. Before the SBO ban, it was slightly higher - about 3 per cent.

Dealler's figure, from p.295 of his book, for the years 1985-1995 of "cattle eaten that were incubating BSE at the time of slaughter", totals to 1.71 million. In the average year, a total of 3.2 million cows are slaughtered, so multiply by 10 to get your decade total of slaughtered cows.


Percentage change in beef consumption in Europe since beef crisis.

From July/August's Public Health News

Country 29 April 25 March
% %
UK -15 -40
Ireland -30 -37
France -40 -40
Germany -30/50 -70
Netherlands -10 -50
Italy -30 -50
Spain -10/20 -30
Denmark -10 -20
Scandinavia -10 -20


French villagers cheer farmers on mad cow march

THE TIMES: FOREIGN NEWS
FROM BEN MACINTYRE IN PARIS ... August 28 1996

A HERD of French cows, flanked by police motorcycle outriders, plodded along the slow lane of a busy highway towards Paris yesterday in the latest, and maddest, protest at the dire effects of mad cow disease on the French beef industry. The cattle and their owners set off from the south of Poitiers on August 11 to draw attention to plunging beef prices and, as they approach the end of their 220-mile odyssey, they have caught the imagination of the public and brought new pressure to bear on the French Government.

"We want to see President Chirac, and we will," declared Jacques Tourenne as he rounded up his cows on a football field near Rambouillet, 30 miles south of Paris, for the morning cattle-drive. The herd and herdsmen, originating in one of the poorest agricultural regions of France, will arrive in the capital on Saturday when the animals will be assembled on the Champ de Mars, which has not seen grazing cattle since the siege of Paris in 1870.

What began as a quixotic protest by a handful of farmers from the Vienne region has gradually swollen to 36 cows and more than 80 people. Teams of six cows at a time take it in turn to amble along the tarmac, behind a tractor with a placard reading: "We are marching to avoid annihilation", while the rest of the herd follows in trailers.

Passing through small towns and villages along the way, they are greeted by the ringing of church bells and spontaneous offers of food and money from sympathetic locals. Beef consumption in France has dropped by a third since the start of the mad cow crisis in March, and the protesters say they face financial ruin unless the Government and the European Union steps in with massively increased compensation and a fixed price for beef.

The protest now occupies a regular slot on the evening news and the progress of the "Mad Cow March" is relayed daily by national newspapers. Politically and otherwise, a prolonged occupation of the Champ de Mars, in the shadow of the Eiffel Tower, could prove extremely messy. Jacques Chirac has often emphasised his roots in the cattle-producing region of the Corrèze and one senior Elysée official said President Chirac would probably agree to meet a delegation of the marchers at the weekend.

The troop headed north along the four-lane N10 highway yesterday at a steady 2mph, causing huge tailbacks but prompting honks of support from lorries passing in the opposite direction.

Roland Fontaneau said he did not regret giving up his summer holiday to walk to Paris. "This was the best way we could think of to show how serious things have got," he said. Like many French farmers, he holds Britain to blame for the health scare that has undermined his livelihood, but he also suspects a more sinister conspiracy. "I think the United States put pressure on Britain to destabilise the European beef market so that American suppliers could monopolise the market," he said.

At night the walkers sleep on straw in the cattle trailers or accept whatever local hospitality is offered. "It's amazing how much help we've had," M Fontaneau remarked, as he whacked the ample rump of Marguerite, a Limousin heifer and the self-appointed herd-leader. The walkers say that Marguerite has become wholly committed to the protest, often refusing to get back into the trailer at the end of her stint on the road. Herding cows on a dual carriageway is technically illegal, yet the police have not only allowed the march to continue but have provided a motorcycle escort. They have warned the herdsmen, however, that as traffic becomes heavier on the approach to the capital, the cows will have to travel on the trailers for some stretches and avoid rushhours.

After their strenuous efforts, the cows will not end up at the Rungis foodmarket outside Paris, but will be returned to the Vienne, leaner and a good deal fitter. "Sometimes we bathe their feet because the road makes them hot," said Jean Grolleau, owner of Marguerite, but otherwise the cows appeared to be enjoying their forced march.


Protesters' cows graze on Champ de Mars

BY BEN MACINTYRE
THE TIMES: FOREIGN NEWS: August 31 1996

A HERD of more than 30 cows grazed nonchalantly alongside the Eiffel Tower yesterday as President Chirac met their owners and promised to support cattle farmers after the BSE crisis. The protesters, bovine and human, left Charroux in central France on August 11 to walk 220 miles to Paris, and were cheered by Parisians as they plodded on to the grassy Champ de Mars yesterday.

The day after farm unions mounted a huge operation to intercept lorries suspected of importing beef from Britain and outside the European Union, M Chirac welcomed a delegation of demonstrators, who entered the Elysée Palace clad in farming clothes and carrying staffs. "The farmers must know that we support cattle breeding and that we will not let them down," the President said after the meeting. M Chirac's power base is in the cattle-producing Corrèze region and the former Agriculture Minister spent an hour and a half listening to the grievances of the farmers, who have seen beef consumption drop by up to a third since March. "He talks like a farmer," Pierre Grolleau said, adding: "The President told us he would not let us down, but he had no magic wand."


French send calves to British abattoirs for EU handout

The Times: Britain August 31 1996
BY MICHAEL HORNSBY, AGRICULTURE CORRESPONDENT

NEWBORN French calves are being shipped to Britain in their hundreds to be slaughtered under a subsidy scheme intended to compensate British dairy farmers for income losses caused by "mad cow" disease.

The Labour Party and animal welfare groups said they were appalled by the trade and called on the Government to stop it. The Ministry of Agriculture is raising this matter with the European Commission. But a spokesman said there was nothing illegal about the imports under the free-trade rules of the single market.

Several hundred French calves have arrived through Dover over the past few weeks in a reversal of the trade in British veal calves to the Continent which last year brought thousands of protesters on to the streets. But the French calves are not for rearing or eating. They are taken straight to an abattoir to be slaughtered.

The carcasses are rendered and incinerated. Provided the animals are less than 20 days old, they qualify for an EU subsidy of £103 a head. The subsidy was introduced in April after British beef exports were banned in response to the Government's disclosure of a possible link between Creutzfeldt-Jakob disease in humans and eating beef contaminated by BSE. Before the ban, dairy farmers had been exporting up to 450,000 calves a year to the Continent for veal production, a trade worth about £45 million.

The animals were mainly surplus male calves for which there is no market here. British farmers, with the export market closed, have sent some 151,000 calves to slaughter under the Calf Processing Scheme to claim the subsidy, which is shared with the abattoir operator.

The man behind the import of French calves is David Muir, a leading livestock dealer based near Oxford. He says he is merely trying to save a business virtually destroyed overnight by the beef ban. "Some people are trying to portray me as an unscrupulous cattle dealer, but I have to make living," he said. "Since the ban I have had to lay off the 18 people I had working for me and get rid of seven of my ten lorries. I desperately need the work."

Mr Muir said that he got £5 of the £103 subsidy per animal as commission. About £80 went to the French farmer and the rest to the abattoir. He began shipping calves several weeks ago and his latest consignment of 200 arrived at Dover from Dunkirk yesterday. "The French have just as much need of this subsidy as the British," he said. "French cattle prices have been driven down by the BSE scare and £80 is a better price than they can get on their local markets."

Elliot Morley, Labour's spokesman on rural affairs and animal welfare, said the Goverment must act to stop the trade. Philip Lymbery of Compassion in World Farming, which led last year's protests against the veal trade, said he was "appalled at this slaughter of the innocents".

Mr Morley said: "The subsidy was put in place to help British farmers. It is being funded very largely by British taxpayers and it is not designed to line the pockets of people dragging calves over here from mainland Europe."

The Ministry of Agriculture said the subsidy came out of the EU budget and would be mostly paid for by taxpayers in other states.



Retailers balk at BSE-free beef plan

By Alison Maitland

The Financial Times ... Wednesday August 28 1996

The government yesterday came under pressure to ease the strict conditions for its BSE-free beef scheme after Marks and Spencer found that none of the 500 Scottish farms which supply it could meet the criteria.

The food retailer said many of the farms had had no cases of bovine spongiform encephalopathy - or mad cow disease - but failed to meet other anti-BSE criteria set by the agriculture ministry for the beef assurance scheme. These cover the type of feed used, and its storage, and outlaw the common practice of adding dairy cows to beef herds.

Mr Edward Goodman, an M&S food technologist, said the retailer had sent its findings to the ministry in the hope it would change the criteria. "We were very disappointed," he said.

The scheme is designed to provide an outlet for specialist, late maturing breeds aged up to 42 months, mainly from Scotland and Northern Ireland. Beef from cattle over 30 months has been banned from the food chain since March.

The government hopes the scheme, launched last week, will be a "foot in the door" to an eventual lifting of the European Union export ban on British beef. It expects 2,500 to 3,000 herds to be involved in the scheme, which cost (UK pounds) 700,000 to establish.

But most supermarket groups are shunning the scheme. Tesco is one of the few that has not ruled out joining. "We need to review whether the claims will stand up as far as the customer is concerned," said Mr Andrew Batty, meat trading director.

J. Sainsbury said it had no plans to change its policy of stocking meat only from cattle under 30 months. Waitrose said: "There are so few farms taking part in it that we won't necessarily be able to get the standard and quantity we need." Somerfield said the scheme was "of no interest to us at all".

Even independent butchers, whom the government sees as the most likely initial outlet, are unsure the scheme will work.

Mr Roger Kelsey, president of the National Federation of Meat and Food Traders, which represents about 10,000 high street butchers, said: "The problem is whether there's enough supply to satisfy the demand and whether distribution is going to be up to scratch."

Sheepish about safety

Michael Jacobs
The Guardian ... Wednesday, July 24, 1996

THE announcement yesterday that it may be possible for BSE to be transmitted through sheep as well as cattle deals a further body blow to Europe's agricultural industry, still reeling from the original BSE crisis.

The sheep and cattle cases differ in vital respects. The precautionary measures to be announced today to remove the potentially infected parts of sheep from the food chain contrast markedly with the slothful reaction of the authorities to the incidence of BSE in cattle in the 1980s. We may be grateful that at least something has been learned. But the underlying issues are the same.

Not that you would understand this from listening to the reactions of the politicians. Once again, the deeper character of an environmental problem seems to escape comprehension.

Take "risk". We shall no doubt be told today that we are more likely to be run over on our way to the supermarket than to catch CJD from the lamb we buy there. Risk, say the experts, is a question of probability; and the probability of being infected by beef (let alone lamb) is minuscule. Given that people take hundreds of other risks every day that are statistically far larger (such as smoking and driving), it is irrational for consumers to stop buying meat. But this fails to see the problem from the consumer's perspective. As an individual lay person, none of us has any idea what the statistical probability is of catching CJD (or indeed of many other risks we take). We are not scientists conducting experiments or compiling statistics. All we have to go on is what the experts tell us. So to the consumer, risk is not actually about probabilities at all. It's about the trustworthiness of the institutions which are telling us what the risk is. Do we believe them?

Unfortunately, that doesn't leave the Government in a very strong position because, frankly, people don't trust it. They particularely don't trust the Ministry of Agriculture, Fisheries and Food, which has a long record of supporting the agricultural industry whatever harms it proposes to inflict on the consumer. In these circumstances it is perfectly rational to stop eating meat; since they've got it wrong before, there is every chance they'll have it wrong this time. Better be safe than sorry.

Underlying this consumer concern is actually a much deeper anxiety. The Government will no doubt tell us this week that the fall-off in sales of lamb will not last long. A temporary panic, they will say, whipped up by the media scare.

Look at beef sales, now back to within 80 and 90 per cent of their pre-BSE levels after falling through the floor in March. This shows how fickle public concerns are about environmental issues: certainly there is no call here, they will say, for a deeper reappraisal of the agricultural industry.

But this mistakes symptoms for causes. There is no question that expressed public concern about individual environmental issues rises and falls in reaction to media exposure. But the underlying anxieties remain even when the particular topical issue has passed.

This is clear from public attitude research. In qualitative surveys, the most common reaction of members of the lay public to the revelation that herbivorous cattle have been fed with sheep meat - and now, we learn, that sheep have likewise been eating dead cattle -is that it is "unnatural". Others describe the practice as "perverted". These phrases capture a form of moral disgust which in turn manifests a far more profound anxiety about the nature of industrial society than the political system is wont to recognise.

RECENT studies confirm a deep concern amongst people in all social groups about "the environment" and what is being done to it. But this anxiety is not of the kind with which politicians (and indeed pressure groups) are very comfortable. It's not a specific concern about particular issues. It's a more general common feeling that the riches of industrial society have been bought at a price; and the costs are now "coming home to roost," (a phrase frequently used by respondents to survey interviews and in discussion groups). Fear that one's children will experience much worse environmental problems in the future is particularly widespread.

These general feelings attach themselves to whatever environmental subjects emerge into the news. Specific issues - BSE, veal calves, Brent Spar - simply act as vehicles propelling a more amorphous environmental anxiety to the surface of public life.

The problem is that, once they get there, there is nothing to hang on to to ensure that they stay in public debate. British politics lacks an ideological framework through which environmental issues like the BSE crisis can be given proper political expression. It is clear that modern conservatism cannot do this. The conservative party's traditional ties to the farming lobby now comes attached to an anti-regulatory, free market zeal; a combination which makes it impossible for the problems of the agricultural industry to be properly addressed.

The Labour Party has quite rightly highlighted the responsibility of the Govenrment's de-regulation policies for allowing the BSE problem to develop. Environmental issues are questions of regulations and dealing with them almost always requires state intervention in markets for the public good. Labour is well placed to frame the issues ideologically in this way. But the deeper question of the kind of agricultural industry which such intervention should seek to promote seems to be beyond it.

This is not just because Labour's electoral tactics dictate that the European beef ban must not become a solely Tory campaign. It is becaue the answer relates to the more fundamental issue of the relationship between inudustrial society and the natural environment.

Like other spheres of economic life, the present methods of the agricultural industry are fundamentally unsustainable. The environmental costs that have now become apparent - not just BSE, but pesticide residues on vegetables, nitrate pollution of water supplies, loss of natural habitats, greenhouse gas emissions from vast transport distances - arise from the very structure of the industry. These are not problems which can be dealt with simply by add-on regulatory measures; as we discovered again yesterday, as soon as one appears to be "solved" another emerges. They can only be dealt with at a fundamental level, by changing the basic processes of the industry towards less intensive, and less chemicalised methods.

Such a re-structuring will not be easy; there will be substantial transitional costs in doing so, and food prices are certain to rise. Public anxiety about the environmental effects of present agricultural methods offers a base of public support on which such a programme might be built. But so long as the issues continue to be framed within conventional political categories, it is hard to see where the momentum for reform might come.

The absence of a properly politicised green movement in the UK is keenly felt here. Agricultural policy reform is an issue which clearly presents the green case; where the language of political ecology-in particular, the concept of "environmental sustainability" finds its natural resonance. The environmental pressure groups do their best. But their focus is on policy, when the real need is to articulate the issues at an ideological level, framing the issues in terms of the fundamental environmental imbalance of the present system. If this could be done, there is a significant wellspring of public concern waiting to be tapped.

Michael Jacobs is a research fellow at the Centre for the Study of Environmental Change at Lancaster University.

Farmers in France stage night raids to block British beef

THE TIMES: FOREIGN NEWS August 30 1996
FROM BEN MACINTYRE IN PARIS

THOUSANDS of angry French farmers erected barricades and intercepted lorries suspected of carrying beef from Britain or countries outside the European Union in an all-night protest over the dire effects of "mad cow" disease. The display of muscle by farm unions, organised in strictest secrecy and carried out with military-style precision, helped to push the franc yesterday to its lowest level for five months.

Using portable telephones and faxes, union leaders mobilised more than 15,000 demonstrators to block main roads, frontier posts and toll gates from late Wednesday night, while squads of farmers carried out spot-checks on lorries carrying meat into France. Simultaneous protests were held in several French towns and cities. Unions said the "nocturnal operation" was intended to reassure French consumers that no British beef was being smuggled into France in defiance of the import ban, and to ensure that all imported meat conformed to health safety standards. But the protest was also aimed at cheap meat from East Europe, which French farmers say has helped to force down prices after the BSE crisis. Beef consumption in France has dropped by a third since March.

More than 2,000 lorries were intercepted illegally before the barricades were dismantled yesterday morning. The unions said at least 15 lorries were found to be carrying "suspect" cargo.

More than 40 British lorries were stopped on arrival at the Channel ports of Dieppe and Le Havre between 10pm and 4am. Four of these, carrying British poultry and lamb, were searched thoroughly and then allowed to continue. "We know it's illegal, we have no right to intercept vehicles, but we felt it was important for the sake of French consumers," Arnold Puech-Dalissac, an official of the National Centre of Young Farmers in Normandy, said. "The British lorry drivers always look frightened when they see a hundred French farmers waiting for them on the dock, but we treated them correctly." The police made no attempt to restrain the farmers. But union officials said police had diverted some lorries away from the roadblocks.

The demonstration, on the eve of an EU meeting to discuss the BSE crisis, was jointly organised by France's two largest agricultural unions, the FNSEA and the young farmers' union, which have long threatened an "explosive" end to the summer. Farmers are demanding increased compensation to counter the collapse of the beef market. "There will be more union operations in the next few days if our calls for help are not heard," a statement said. Luc Guyau, head of the FNSEA, said: "We will be inflexible." He added that the aim of the protest was not to close borders, but to ensure that "both producers and consumers benefit from complete openness on the origin of meat". The operation was carried out in secrecy in order to intercept "rogue importers from outside the EU". In other protests, farmers dumped veal on the streets of Laval, invaded local government offices in the Creuse region and occupied an abattoir near Grenoble. A Dutch lorry driver on the Belgian border who refused to allow an inspection had his tyres slashed and his cargo handed over to customs officials.

Philippe Vasseur, the Agriculture Minister, insisted last night that he had not been warned in advance of the unions' demonstration, but avoided criticising the farmers. "We will do what we have to do to ensure the farmers are compensated for their losses and to safeguard their future." President Chirac has agreed to meet a delegation of farmers from the Vienne region today. The group, driving a herd of more than 30 cows ahead of them, are due to arrive on the Champ de Mars in central Paris this morning, a day ahead of schedule. "We have some simple truths to tell him, particularly about the effects of BSE disease," a spokesman for the group said.


BSE in Germany

(Extracted from Animal Pharm No 349, 24 May, 1996

The German ban on imports of Swiss beef and beef products must remain in place until the BSE problem is cleared, Agriculture Minister Jochum Bochert has stressed. To date Switzerland has had 211 cases of BSE. Speaking at a meeting of the German central agricultural marketing board (CMA) last month, the minister sought to reassure consumers by stressing that German beef comes only from animals slaughtered in Germany and veterinarian checked. However, compared with its European neighbours, the German reaction to BSE has been out of proportion to its incidence.

To date there have been 4 cases of BSE reported in Germany. All were imported breeding stock from the UK and held to have been "infected" prior to entering Germany. There is no definite proof however of when and how that "infection" took place, notes Arzte Zeitung. Moreover, all four were of breeds usually grassfed and reared extensively. Perhaps as few as 200 of the 160000 British BSE cases have stemmed from cattle reared in this manner, believes Dr Gotz Anhalt, a veterinarian with the Agriculture Ministry of Lower Saxony.

The 4 cattle were sold at about 2 years old to German cattle traders. It is assumed that they were fed up before export to enhance weight gain and make them more attractive to dealers. Their ages, 3 were born in 1988 and 1 in 1986, would indicate that that any such feeding with meat and bone meal would have occurred after the UK's July 1988 ban on mammalian feed, thus illegally. Arzte Zeitung has thus speculated on the possibility that they became infected within Germany. The animals were sold through cattle traders in Lower Saxony and elsewhere. There is a dispute for at least one as to the herd of origin. A veterinarian claimed that for the index case in February 1992, UK documentation showed clearly that the animal came from a herd that until then had been BSE-free. Another veterinarian also involved in the diagnosis claims that the precise origin was far from clear and possibly through "dubious channels".

The issue of BSE and its effects in Germany have incensed the national farmers' union (DBV) whose president has lambasted the UK for its "scandalous negligence" in past BSE control efforts. He asserts that German farmers are innocent victims and have suffered a very severe backlash - surveys show that German beef consumption is lagging at under half pre-crisis levels. The DBV is adamant that mammalian feed has never been fed to German cattle and that "Germany is BSE free".


English physicians still out of the loop on v-CJD

A Franks & M Schweiger, British Medical Journal, 1996, 312(May 25), 1358

A survey of 50 physicians was conducted in Leeds, England, to evaluate their awareness of the national surveillance system for Creutzfeldt-Jakob disease. Of the 38 physicians who responded 4 neurologists were aware of the system (3 of whom had seen cases), no neurologist responded who was unaware of the system; 3 psychiatrists were aware but 7 were not aware of the system; 5 specialists in geriatrics were aware and 2 were not; 8 other medical specialists were aware but 9 were not. Cases of variant-Creutzfeldt-Jakob disease, who may not initially present with obvious neurological symptoms, may at first be referred to psychiatrists, a group that appears to be less aware of the surveillance system. The researchers call for the existence of the CJD Surveillance Unit in Edinburgh to be made more widely known and for working case definitions for provisional, possible and confirmed cases to be available.


Prion protein accumulation in the spinal cords of patients with sporadic and growth hormone CJD

Goodbrand IA; Ironside JW; Nicolson D; Bell JE National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, UK
Neurosci Lett, 183: 1-2, 1995 Jan 2, 127-30
An immunohistological study of the spinal cord in 20 cases of sporadic and 4 iatrogenic (growth hormone) cases of Creutzfeldt-Jakob (CJD) diseasepatients was performed to detect the presence of disease specific prion protein using a number of different antisera. Prion protein was present in all thegrowth hormone recipients and in 11 of the 20 sporadic CJD cases. Plaque-like deposits of prion protein were found in all the growth hormone cases andthree of the sporadic cases. This is the first demonstration of the topographic immunolocalisation of prion protein in the spinal cord of CJD patients, afeature which could help elucidate some important aspects of the pathogenesis of CJD.

Tissue handling in suspected CJD

Budka H; Aguzzi A; Brown P; Brucher JM; Bugiani O; Collinge J; Diringer H; Gullotta F; Haltia M; Hauw JJ; et al Institute of Neurology, University of Vienna, Austria
Brain Pathol, 5: 3, 1995 Jul, 319-22
Despite many sensational and intimidating reports in the mass media, transmissible spongiform encephalopathies (prion disease) are not contagious in theusual sense. Successful transmission requires both specific material (an affected individual's tissue, from or adjacent to CNS) and specific modes (mainlypenetrating contact with the recipient). Nevertheless, specific safety precautions are mandatory to avoid accidental transmission and to decontaminate anyinfectivity. Autopsy is essential for definite diagnosis of these disorders. Recommendations are given here for performance of the autopsy, forneuropathology service and appropriate decontamination; they are based on the current literature and on precautions taken in most laboratories withexperience in handling tissue from transmissible spongiform encephalopathies. In particular, special care must be taken to avoid penetrating wounds,possible contamination should be kept to a minimum, and potential infectious material must be adequately decontaminated by specific means.

Transplantation of corneal tissue from donors with diseases of the CNS

Hogan RN; Cavanagh HD 
Department of Ophthalmology, University of Texas Southwestern Medical Center at Dallas 75235-9057, USA. 

Cornea, 14: 6, 1995 Nov, 547-53 
A great deal of controversy and concern exists over potential transmission of central nervous system diseases by corneal transplant. The purpose of this study was to evaluate the available data relative to this question, pertaining especially to transmission of infectious dementia. From these data, determination of conveyance risks are possible, and rational policies for donor inclusion criteria can be constructed. Retrospective analysis of available published data regarding transmission of infectious dementias was performed. Risk of disease transmission was calculated from population data. Of the various forms of dementia, only rabies, hepatitis B, and Creutzfeldt-Jakob disease (CJD) have been transmitted by corneal transplantation. Transmission of the first two viruses is preventable by serologic testing. Prevention of CJD transmission relies on clinical history. Despite the possibility of transmission and the lack of available testing, slow virus disease (CJD) has been transmitted only once. That this case represents an extremely rare event is supported by a lack of successful transmission via corneal transplant in monkeys; lower levels of infectious agent in cornea than in brain; lack of successful transmission of similar human dementias, including Alzheimer's disease to primates; the apparent requirement for homozygosity at codon 129 of chromosome 20 for transmission; lack of transmission in 5-10% of CJD cases even after brain inoculation; and low numerical risk of transmission based on population data. Only 0.5-4 CJD infected donors per year would be expected. Current Eye Bank Association of America criteria for donor exclusion based on suspicious history are adequate to protect against accidental conveyance of transmissible dementia.