Document Directory
23 Jul 00 - CJD - Top scientist says BSE is in sheep flocks
23 Jul 00 - CJD - Top scientist says BSE is in sheep flocks - original text
22 Jul 00 - CJD - Young woman's death blamed on CJD
22 Jul 00 - CJD - Ulster beef ban may be lifted
20 Jul 00 - CJD - BSE alarm after cow's head is found on sale
19 Jul 00 - CJD - Plan drawn up to avert food panic if BSE found in sheep
18 Jul 00 - CJD - Scientists warn of 30% rise in human BSE
18 Jul 00 - CJD - Beef imports 'a BSE risk'
18 Jul 00 - CJD - BSE measures 'not tight enough'
17 Jul 00 - CJD - US check on sheep in new BSE alert
17 Jul 00 - CJD - School food 'may have spread CJD'
17 Jul 00 - CJD - Bid to export Ulster beef
17 Jul 00 - CJD - CJD cluster 'cannot be blamed on baby food'
17 Jul 00 - CJD - Baby food theory may explain CJD deaths
16 Jul 00 - CJD - US sheep may be infected with BSE from British feed
16 Jul 00 - CJD - School meals linked to CJD deaths
16 Jul 00 - CJD - Expert: School Meals May Have Spread CJD
15 Jul 00 - CJD - Village shaken to find itself CJD blackspot
15 Jul 00 - CJD - Tonsil and appendix checks could show spread of CJD
15 Jul 00 - CJD - Eating habits may explain the link between victims
15 Jul 00 - CJD - The five deaths that link a village in middle England to a CJD epidemic
15 Jul 00 - CJD - How human BSE emerged
15 Jul 00 - CJD - Village CJD investigation centres on abattoirs
15 Jul 00 - CJD - Britain is facing 'long, slow epidemic' of CJD
15 Jul 00 - CJD - Queniborough, where adverts for beef look like local indignation
23 Jul 00 - CJD - Top scientist says BSE is in sheep flocks
Jonathan Leake, Science Editor
Sunday Times ... Sunday 22 July 2000
A Nobel prize-winning scientist has warned that there is increasing evidence that BSE, so-called "mad cow" disease, is endemic in British sheep .
Research by Stanley Prusiner suggests that the infective prion agent that causes BSE is found in sheep but at levels which have until now been undetectable.
Prusiner, associate professor of neurology at the University of California in San Francisco (UCSF), won the Nobel prize in 1997 for discovering prions. Last week he said: "The implication of our latest work is that BSE is endemic throughout the British national sheep flock."
Prusiner's original work described how prions form when proteins that occur naturally in the brains of all mammals become deformed. The altered protein then acts as a template, changing other molecules in a chain reaction that devastates the brain.
If an infected beast is eaten, the prions start a similar chain reaction in the animal that ate it.
However, although this mechanism has been well described, the BSE prion has never been found in sheep.
Last week Prusiner said he and Professor Mike Scott, a Scottish researcher, appeared to have produced BSE in mice by infecting them with material from sheep suffering from scrapie , which is another prion disease.
Prusiner said: "Our initial data suggests that these sheep were producing more than one type of prion. One was the scrapie prion that killed them, but we believe some were also making the BSE prion."
The research uses "bovinised" mice, in which the gene that makes prion proteins is replaced by the same gene from a cow. Such mice react to BSE prions just like cows, but take less than 10 months to develop the disease, compared with more than three years for cows.
When such mice were inoculated with BSE prions the resulting disease was identical to that caused by variant CJD prions from humans - evidence that the prions are the same.
The mice were then injected with material from sheep with scrapie and, again, the incubation and symptoms were close to those of BSE. This was a powerful indicator that sheep can produce BSE prions.
Fred Cohen, professor of pharmacology at UCSF, said there was strong evidence that cattle developed BSE because of changes in the way sheep carcasses were rendered into animal feed .
In their latest work, Cohen, Prusiner and Scott replicated the changes in the rendering process in the laboratory and injected the resulting material into mice. Cohen said early results suggested that the theory was correct .
"When scrapie-infected sheep were slaughtered, the rendering process destroyed the scrapie prions but left behind the tougher BSE prions - to which cattle were vulnerable," he said.
If the unpublished results are confirmed, it could have a serious impact on the sheep industry. There is no evidence that people can catch BSE from eating sheep, but most research has focused on cattle, so the possibility cannot be ruled out.
The main damage to the industry would come from a loss of consumer confidence in sheep products. The government has already prepared contingency plans for testing and slaughtering if BSE should be found in sheep.
23 Jul 00 - CJD - Top scientist says BSE is in sheep flocks - original text
Jonathan Leake, Science Editor
Sunday Times ... Sunday 22 July 2000
A leading scientist has warned that BSE, so-called mad-cow disease is endemic in British sheep - and that people could be at a low but real risk from eating lamb.
New research by Professor Stanley Prusiner strongly suggests that the infective "prion" agent that causes BSE is found in sheep but at levels which have, until now, been undetectable.
Prusiner, professor of neurology at the University of California in San Francisco, won the Nobel prize for discovering prions. His laboratories are among the world leaders in such research. Last week he said: "The implication of our latest work is that BSE is endemic throughout the British national sheep flock ."
Prusiner's original work described how prions form when proteins that occur naturally in the brains of all mammals become deformed. The altered protein then acts as a template, changing other molecules in a chain reaction that devastates the brain. If an infected animal is eaten the prions start a similar chain reaction in the animal or person that ate it.
However, although this mechanism has been well described the BSE prion has only been found in cattle, never in sheep - until now . Some British researchers argue that BSE prions arose through a genetic mutation in cattle and spread because material from dead cows was put in cattle feed.
Last week Prusiner said he and Professor Mike Scott, a Scottish researcher, appeared to have produced BSE in mice by infecting them with material from sheep suffering from scrapie, another prion disease. Prusiner, who has given up eating all sheep products because of his work , said: "Our initial data suggests that these sheep were producing more than one type of prion. One was the scrapie prion which killed them - but we believe at least some were also making the BSE prion."
The research uses "bovinised" mice in which the gene that makes prion proteins is replaced by the same gene from a cow. Such mice react to BSE prions just like a cow - but take less than 10 months to develop the disease, compared with more than three years for cows - so speeding up research. When such mice were inoculated with BSE prions the resulting disease was identical to that caused by variant CJD prions from humans - powerful evidence that the prions are the same .
By contrast, material from animals and humans with different prion diseases took longer and damaged the brain differently.
The mice were then injected with material from sheep with scrapie - and again the incubation and symptoms were very close to those of BSE. This was powerful evidence that sheep can produce BSE prions . If they had died of scrapie the disease should have looked different.
Fred Cohen, professor of pharmacology at UCSF, said this was strong evidence that cattle got BSE because of changes in the way sheep carcases were rendered into animal feed .
These changes involved cheaper solvents and lower temperatures - which Cohen believes still killed scrapie prions - but had no effect on the tougher BSE prions.
In their latest work, said Cohen, he, Prusiner and Scott were replicating the changes in the rendering process in the laboratory - and injecting the resulting material into mice. Cohen said that early results, so far unpublished, suggested that the theory was correct. Material subjected to the earlier, tougher regime, seemed not to produce disease.
"When scrapie-infected sheep were slaughtered the rendering process destroyed the scrapie prions but left behind the tougher BSE prions - to which cattle were vulnerable," he said.
Such theories have been advanced before. Research by Moira Bruce at the Neuropathogenesis Unit in Edinburgh has confirmed that sheep can produce a range of prion particles but finding the one that causes BSE has eluded researchers until now.
If the results are confirmed - and many more experiments are needed to be sure - it will be a devastating blow to the British sheep industry. There is no evidence that people can catch BSE directly from eating sheep but most research has focussed on cattle so the possibility cannot be ruled out . Such a discovery would also devastate consumer confidence .
22 Jul 00 - CJD - Young woman's death blamed on CJD
By Valerie Elliott, Consumer Editor
Times ... Saturday 22 July 2000
A Young woman from Cheshire was named yesterday as the latest probable victim of variant CJD, the human form of BSE.
Kirsty Garven, 20, from the farming village of Waverton, near Chester, died two weeks ago after 14 months of the fatal brain illness which left her unable to eat, speak or walk.
Her parents, Alex, 59, and Jenny, 53, attacked politicians yesterday for not doing more to protect the public. Mrs Garven said: "It's greed and profit . That's why we cannot let them get away with it."
She recalled the image of John Gummer, then Agriculture Minister, feeding his daughter a burger in front of television cameras to persuade people that beef was safe. "I would like to say, 'Excuse me, would you come and see this? This is what it has done to my child.' I cry every day when I think about her," she said.
The couple have no idea how Miss Garven contracted the disease and did not tell her that she had it. For the last seven months of her life she needed 24-hour care . Mrs Garven said: "By the end she was a skeleton. We were watching our child fade away."
Experts in vCJD at the Edinburgh surveillance centre are carrying out tests to confirm that Miss Garven was a victim of the disease. News of the death comes after concern from scientists that cases - 69 deaths so far and seven probable cases, including Miss Garven - are to rise by up to 30 per cent a year . Professor Peter Smith, acting chairman of the Spongiform Encephalopathy Advisory Committee, which advises the Government, said this week that he believes that thousands could die from the disease, but he did not believe it would become an epidemic.
Normal beef exports from Northern Ireland to the Continent could resume "within weeks" as a result of the low incidence of BSE in the Province, David Byrne, the EC Food Safety Commissioner, said yesterday.
22 Jul 00 - CJD - Ulster beef ban may be lifted
By David Brown, Agriculture Editor
Telegraph ... Saturday 22 July 2000
Restrictions on exports of beef from Northern Ireland may be lifted by the European Commission within a couple of weeks , David Byrne, the EU Commissioner for Health and Consumer Protection, said yesterday.
Only six cases of BSE have been reported in Ulster this year compared with nearly 500 in Britain
Speaking in London, Mr Byrne said that he was keen to remove the restriction on exports of beef from animals more than 30 months old in view of the very small number of BSE cases in Northern Ireland. Currently no cattle in the United Kingdom which are more than 30 months can be used for beef. However, Mr Byrne said that measures to ensure that British beef was not shipped to Ulster, and then transferred onwards, would have to be put in place first.
Farmers in Ulster have argued that they should be treated equally to producers in the Republic of Ireland who have also had relatively few cases of BSE and do not have to comply with the over-30 months rule. Only about six cases of BSE have been reported in Ulster this year, compared with nearly 500 in Britain.
The restriction has damaged Ulster - and other UK beef exports - because traditional beef breeds often reach prime slaughter condition after 30 months. Slaughtering them earlier affects beef quality at a time when top quality is essential to recapture export markets.
Scottish farmers have also pressed for this dispensation but Mr Byrne signalled that they would be refused. Although Scotland has had fewer than 20 cases of BSE this year there are concerns in Brussels that there is a health risk from the regular trade in cattle between Scotland and England where most BSE cases still arise.
20 Jul 00 - CJD - BSE alarm after cow's head is found on sale
By Valerie Elliott And Steve Bird
Times ... Thursday 20 July 2000
An African delicacy containing the cheeks, snout and lips of cattle has been found on sale in Britain , breaking strict food safety rules on BSE.
The illicit trade in cattle heads has triggered an urgent investigation by the Food Standards Agency (FSA) to halt their supply for human consumption. The meat in the pomos - a Nigerian and West African beef speciality - contains possible BSE-infected material and could be harmful to health. The heads are thought to have come from British cattle .
Food safety officers in London uncovered the illegal market in pomos a few weeks ago and alerted the FSA. A head of a cow was found packed in a box and stored in a fridge at a butcher's shop in Tooting, South London. Two other cases have since been reported to the agency.
The Times found the banned meat on open sale in five ethnic food shops in Brixton, South London, yesterday. The meat was selling at £1.50 per lb. All shopkeepers removed the meat from sale yesterday when they learnt that it was illegal, and Lambeth council launched an investigation.
Local authorities are now working with the agency to establish the scale of the trade and to identify the source . Heads are routinely removed from beef carcasses in Britain, rendered and then stored for incineration. Officials now fear that heads are being smuggled out of an abattoir and that cow heads are being deliberately sold to supply this speciality food market.
A senior FSA official last night said that pomos contained specified risk material and there was a risk of BSE. He said: "We are trying very hard to identify the source and to halt the supply. Cases are being sought by local authorities and we have had at least three firm reports from London. Other authorities have been alerted to watch for the meat."
Senior officials in Whitehall are concerned about the illegal trade given the latest rise in confirmed cases of the human form of "mad cow" disease and the forecast that the cases could rise by up to 30 per cent in future years . Peter Smith, acting chairman of the Government's BSE advisory committee, the Spongiform Encephalopathy Advisory Committee, said that there had been a "statistically significant" increase in the number of cases of variant Creutzfeldt-Jakob disease. Professor Smith said it could claim thousands of lives but did not think that it would now be an epidemic .
The trade in pomos was uncovered by food safety officers working for Wandsworth Borough Council. A council spokesman said: "The butcher has been issued with a formal written warning."
All the butchers selling the meat yesterday said that they had bought it in good faith. Riaz Muhammad Friez, the leaseholder of Brixton Foods, at 39 Electric Avenue, said: "If the face is illegal we will remove it from the shelves."
Other shops selling the meat were Kashmir Halal Butchers, M. M. Quality, Faiz Quality Meat and Fab Fresh.
19 Jul 00 - CJD - Plan drawn up to avert food panic if BSE found in sheep
James Meikle
Guardian ... Wednesday 19 July 2000
Contingency plans to prepare for the disastrous consequences of a food panic if BSE is found in sheep are being drawn up by the government and meat industry.
Detailed advice on changing slaughterhouse and butchery practices is being considered to help save the £385m a year sheepmeat trade from collapse even though no evidence has yet been found that the disease is occurring naturally in the 40m national flock.
But officials of the food standards agency warned yesterday that evidence "might emerge at any time in the next year or two from studies under way".
They said that present controls designed to prevent the theoretical risk of human infection from mutton and lamb, introduced after the crisis that hit beef in 1996, would not be enough if a dangerous BSE-like agent was in sheep as well. Some even sugested it would be "very difficult" to remove all risky material from meat before it went on sale because of the way BSE has been shown to work in sheep in the laboratory. It spread far more widely through the carcass than it did in cattle.
The ministry of agriculture last night insisted that its scientific advisers were keeping the problem under constant review. "Any action, if BSE was to be found in sheep, would depend on evidence at the time. We are not going to pre-empt what advice they would give," a spokeswoman said.
David Croston, of the meat and livestock commission, the government-backed industry body, confirmed it had been studying changes but added: "Until science tells us what we should be removing, we can't act. There is no strong evidence as of yet about what we should be doing. If someone says we have to remove x, y and z, we know how to do it and we will then advise the industry in an appropriate manner."
It was difficult to quantify the impact on the industry or the extent of changes that might be needed.
Ian Gardiner, deputy director general of the National Farmers' Union, said: "It would be entirely improper if experts in such agencies did not develop contingency plans." But he too was keen to stress that the risk of BSE appearing in sheep did not appear to have increased in recent months. (UK Correspondents note: If the only three sheep herds exported from Europe to the US are infected, it is inconceivable that UK sheep are not infected as BSE is far more prevalent here)
Some of the concerns were outlined in a "working document" provided by agency leaders for discussion with consumer groups, the meat industry, vets and families of victims of the human form of BSE, known as vCJD, whose deaths have been linked to eating infected material from cows before risky parts of the carcasses were banned from food between 1989 and 1995.
Scientific advisers to the government have so far decided not to widen controls, although they have commissioned an assessment of risk to public health .
Heads of sheep are already banned from human consumption, a move introduced in 1996 particularly to protect ethnic minority groups. In addition, the tonsils and spleen of all sheep and the spinal cords of sheep over a year old are banned.
But intestines are still used for sausage casings , and lymph tissue , also infected in laboratory experiments, is wide spread through other meat on lambs and sheep.
The main fear is that the presence of scrapie , a BSE-like disease in sheep not known to have endangered human health, may be disguising the BSE agent , which may have transferred to sheep through now-banned cannibalistic feeding regimes in the 1980s. Scrapie-infected sheep brains are being tested using mice to detect whether a BSE-like strain is evident. The agency document suggested one might be identified "at any time" .
"Due to the limitations of existing studies and available methods, failure to detect BSE in the national flock will not be conclusive evidence that it is not present." The tone of the document contrasts with that of scientists on Seac, the advisory committee for BSE and its human form, which suggested last February that tests on sheep brains up to then "do not have the characteristics associated with BSE".
The agency hopes genetic breeding techniques will increase sheep resistance to BSE and scrapie over the next 10-20 years. Meanwhile, the agriculture ministry has contacted US counterparts who ordered the destruction of sheep imported from Europe because they displayed signs of a BSE-like disease. It appears satisfied that some were suffering from a form of scrapie.
18 Jul 00 - CJD - Scientists warn of 30% rise in human BSE
James Meikle
Guardian ... Tuesday 18 July 2000
Government scientists yesterday warned of a sharply accelerating trend in the incidence of human BSE after studying the pattern of the disease so far.
They said the number of reported cases may in fact be rising at between 20% and 30% a year despite the apparently varied annual death rates over the past five years.
The prediction came as it was revealed that the death toll from the incurable condition officially known as vCJD had risen by a further two in the past fortnight to a total of 69 , and 14 so far this year.
The scientists said that there was now a "statistically significant rising trend " in the number of victims since the first casualties first displayed signs of the disease in 1994, although it was still too early to forecast the ultimate number of deaths caused by vCJD.
This year's toll is already equal to that for the whole of last year when the number dropped. A further seven people still alive are thought to be suffering from the condition. The scientists have come to their conclusion about the progress of the disease after analyses of monthly figures, including studying the dates at which friends, relatives or doctors first noted symptoms.
The period between this and eventual death has varied between seven and 38 months , with an average of 14 months , although the incubation period before symptoms become evident is believed to be several years longer.
Stephen Churchill was the first known death from the disease in May 1995, although it was not formally identified or officially linked to the eating of beef in the late 1980s until March 1996. Three people died in 1995, 10 in 1996, 10 in 1997, 18 in 1998 and 14 last year.
Members of the government's spongiform encephalopathy advisory committee took the unusual step of publishing the figure immediately after their meeting in London yesterday because of the recent interest in a cluster of five cases around Queniborough in Leicestershire.
These included three victims dying within a few of months in 1998, a fourth who died in May and another patient , still alive, who is thought to be suffering from the same disease.
The scientists said this was "unlikely to have occurred by chance but this cannot be completely ruled out" and they would be closely informed about local investigations.
The Department of Health last night said it could not elaborate on the significance of the new analysis until ministers and officials had considered the scientists' new advice.
The figures came amid reports that sheep imported by the US from Europe were showing signs of a disease which could be linked to BSE in cattle. Government scientists are to hold talks with their US counterparts after the US agriculture department ordered the destruction of three flocks of sheep which were in quarantine in the state of Vermont.
18 Jul 00 - CJD - Beef imports 'a BSE risk'
By David Brown, Agriculture Editor
Telegraph ... Tuesday 18 July 2000
Tighter controls on imported beef to protect consumers from mad cow disease are to be considered by the Food Standards Agency.
Sir John Krebs, the chairman, said in London yesterday that the agency would have to deal with the issue of imported beef from other European Union countries with a history of BSE "one way or another ".
Sir John admitted at a public hearing of the agency's "stakeholder group" at central hall, Westminster, that action would have to be taken after hearing complaints from farmers and vegetarians that the Irish Republic and France sent thousands of tons of meat to Britain from cattle which were not subjected to the tight controls applied here.
About 193,000 tons were imported last year and around 185,000 are expected to be imported this year, according to Meat and Livestock Commission figures. Sir John moved quickly to reassure consumers after Jim Walker, the president of the National Farmers' Union of Scotland and member of the stakeholder group, protested that a report by the agency dealing with anti-BSE controls in British abattoirs devoted "only a few lines " to the question of imported meat.
The meeting heard that meat from the cheeks of cattle slaughtered in Ireland was being used in processed foods sold in Britain. Meat from the heads of cattle in Britain is banned from human consumption as a precaution against BSE.
Mr Walker said: "We want to see spot checks on imported beef. We want imported beef to meet the same standards as our own."
18 Jul 00 - CJD - BSE measures 'not tight enough'
By David Brown, Agriculture Editor
Telegraph ... Tuesday 18 July 2000
Consumers will need stronger and greater protection against mad cow disease if research shows that BSE has spread to sheep , according to advice given to the Food Standards Agency.
A report released today by the Food Standards Agency's stakeholder group will warn that current measures to destroy "high risk " material from sheep as a precaution are not tight enough to prevent the BSE agent from entering the human food chain in a variety of lymphoid tissues.
Evidence that BSE has entered the national flock "might emerge at any time in the next year or two", it says. BSE has been linked with 75 known or probable British cases of new variant Creutzfeldt-Jakob disease (CJD), its human equivalent.
Three flocks of sheep imported to the United States from Europe are to be destroyed after displaying signs of a fatal brain disease. Scientists are trying to establish whether the sheep contracted BSE or a novel strain of scrapie.
From next January, all sheep will have to be tagged under European Union directives aimed at tracing "tainted" livestock.
17 Jul 00 - CJD - US check on sheep in new BSE alert
James Meikle
Guardian ... Monday 17 July 2000
Government scientists are to hold urgent talks with their counterparts in the United States over the discovery that sheep imported from Europe are showing signs of a disease that may be linked to BSE in cattle.
Three flocks quarantined in the state of Vermont are being destroyed under the orders of the US agriculture department which has begun tests to establish whether the disease believed to have spread to humans has also transferred to sheep.
Scientists in Britain have long feared this is possible, although the disease has been successfully introduced to sheep only in laboratory tests.
Now experts working for the government have commissioned a risk assessment to quantify the threat to human health if BSE were found in Britain's 40m sheep .
From January, all sheep will have to be tagged as part of European Union measures to track major diseases. The Ministry of Agriculture said last night: "There is nothing to suggest that what they have discovered in America is BSE at the moment. But whatever they discover we will look at with interest." (UK correspondent's note: par for the course)
Government officials are trying to quantify the costs of a human epidemic of variant CJD, linked to eating beef from infected cattle years ago, which has so far claimed 67 lives in Britain with at least eight other victims probably suffering from the fatal condition.
Experts are worried by a rise in cases in recent months, and are investigating an apparent cluster involving five people, four of whom have died, around the Leicestershire village of Queniborough.
Members of the main scientific advisory committee in BSE and variant CJD are meeting to discuss both issues today.
News of the US investigations emerged as Robert Will, head of the CJD surveillance unit in Edinburgh, reiterated his fears that the high proportion of young victims resulted from their exposure to foods containing mechanically recovered meat used in baby foods , pates, beefburgers, pies, sausages and other dishes often on school meal menus .
Many risky materials from cattle were banned from food in 1989, although enforcement of the restrictions is thought to have been poor. But mechanically recovered meat , known as MRM, machined off animal bones, was not banned until 1995 .
Professor Will told the Independent on Sunday: "Foodstuffs which contained that material could be particularly dangerous. One possible explanation for the age distribution is that young people tend to eat these products more than the adult population."
Prof Will was not available yesterday but the Department of Health said: "There is nothing new in this. Everyone has been well aware for a long time that reprocessed meat was a potential means of transmission.
"Scientific research continues. Reprocessed meat was used in a very wide variety of feedstuffs, certainly not babyfood and school meals. Nobody should be in any doubt there is no risk now from these things."
(UK correspondents note: this is simply not true, MRM was used in babyfood and fare used in school meals. The privatisation of school meals services led to the used of the cheapest products available. Prior to 1989, MRM included the brains and spinal cords of cattle, the most most infected parts)
The US investigations involving 376 sheep could take several years. Four were found to have a BSE-like disease which could be a new form of scrapie, a sheep disease long endemic in Britain, but not known to be dangerous to humans.
The tests will be conducted at a secure facility at Plum Island, near New York. The animals were dairy sheep imported from Belgium and the Netherlands in 1996 and their milk, and cheese made from it, has been sold for some time. Their lambs have also been sold for food.
Britain has banned some parts of sheep, including the head, from entering food for some years but is slightly relaxing controls as a trade-off for EU-wide measures.
Scrapie is increasing in Britain, with nearly 600 cases reported last year. The actual incidence may be eight times as high. It is mostly identified in older sheep.
17 Jul 00 - CJD - School food 'may have spread CJD'
By David Derbyshire, Medical Correspondent
Telegraph ... Monday 17 July 2000
The arrival of self-service school meals in the 1980s could have played a part in the spread of the human form of mad cow disease, a leading scientist said yesterday.
Prof Bob Will, director of the Government's CJD surveillance unit in Edinburgh, said the use of mechanically recovered meat in school meals and baby food may have contributed to the relatively high incidence of new variant CJD in young people .
Although minced beef had been a staple in school dinners for decades, the arrival of a daily choice of burgers , meat pies and sausages with the growth of self-service canteens may have increased children's exposure to cheaper and riskier meat .
The families of CJD victims called for more research into how the disease spread from cattle to people in the years before infected meat was removed from the food chain. However, the Health Department insisted last night that there was "nothing new" in the suggestion that school meals and baby food may have been contaminated with BSE in the 1980s.
The suggestion came as scientists continued to investigate the Leicestershire cluster of five cases of confirmed and suspected nvCJD. Two of the victims were in their teens. The possible link between BSE and school meals is not a new one. By 1990, six years before the Government announced that BSE infected meat could be a health risk, schools had begun to remove beef from their menus. (UK correspondents not: this was against the express wishes of the Government, who incorrectly advised that beef was safe)
Scientists have also suspected for some time that children were at greater risk because of a preference for burgers and processed food. Only the cheapest meat, such as offal, spinal cords, brain and spleen, are thought to have been contaminated in the 1980s. Before they were banned in 1989, they were used in burgers, sausages, pies, mince and baby food.
Doctors have long debated why nvCJD is relatively common in young people. Before the new strain was identified, CJD was a disease of the middle aged and elderly, rarely affecting people below 50. The average age of the first 10 victims of nvCJD was 27.5 years.
Although the incubation period of the disease is usually about seven to 10 years, the youngest two victims showed symptoms by the age of 14 . Dr Will said: "One possible explanation for the age distribution is that young people tend to eat these products more than the adult population."
Another could be that baby food and school dinners in the Eighties contained mechanically extracted meat. These are leftovers from the carcass removed using water jets (UK correspondents note: steam jets actually) and which may have contained infected spinal cords.
But Prof Wills's team at Edinburgh is not ruling out other possible reasons. One could be that developing brains are at greater risk of infection. The Government's Spongiform Encephalopathy Advisory Committee is due to discuss the outbreak around the village of Queniborough, Leicestershire, today.
At the weekend, the investigation focused on local abattoirs and their slaughtering practices. Four local people have died from the disease: 19 -year-old Stacey Robinson, Pamela Beyless, 24 , Glen Day, 35 , and an unnamed 19 -year-old man. Another is suspected to be dying.
17 Jul 00 - CJD - Bid to export Ulster beef
Staff Correspondent
Times ... Monday 17 July 2000
Nick Brown, the Agriculture Minister, is to press European ministers to allow normal exports of beef from Northern Ireland because of the low incidence of BSE (Valerie Elliott writes).
The special status for Northern Ireland beef will trigger calls for Scottish beef to be included in the new terms.
Others believe that if routine Northern Irish exports are restored, exports from the rest of the UK will follow.
Mr Brown may signal his intentions this week at the Agriculture Council in Paris.
The EC agreed to restore exports from Britain in August last year provided it met the regulations of the Date Based Export Scheme, under which only one abattoir in Cornwall and one in Scotland is licensed to export beef to the continent.
Northern Ireland does not have such an abattoir and no beef from there has been exported under the DBES.
17 Jul 00 - CJD - CJD cluster 'cannot be blamed on baby food'
By Valerie Elliott, Countryside Editor
Times ... Monday 17 July 2000
The man leading the investigation into the Leicestershire cluster of deaths from the human form of BSE, variant CJD, last night denied that it was linked to infected baby food or school dinners . Dr Philip Monk, the county consultant in communicable diseases, was emphatic: "Baby food and school dinners are not a factor in our cluster."
He spoke out after one of Britain's leading experts on the fatal brain disease suggested that contaminated beef in baby food and school meals could account for the number of cases in young people.
Dr Robert Will, director of the CJD surveillance unit in Edinburgh, said that one explanation for the disease among the young could be the amount of mechanically extracted meat in children and babies' food in the 1980s.
Such meat, widely used in baby foods and common in school meals , was also used for cheap products such as sausages , pies , burgers and ready-cooked meals . After investigating the cases in Leicestershire for three weeks, Dr Monk is convinced the answer will be a different one.
He was not prepared to speculate on his findings so far. The Times has, however, established that officials are focusing attention on the movement of cattle into the county and, in particular, the sale of elderly dairy cattle to the meat industry. The prime cuts of this dairy cattle meat were largely exported abroad, but the cheaper ends were used in the UK for mince and other ready-made products.
Officials are investigating all local abattoirs and meat- processing plants in the area and trying to establish whether the meat was sold regularly to local outlets. Initial findings about the cluster are expected to be discussed today at the regular six-weekly meeting of the Spongiform Encephalopathy Advisory Committee. Last night Dr Harriett Kimbell, a lawyer and the consumer representative on that committee, said that it may take several months to identify the source of the cluster. "I shall press for the fullest information to be made public. Rest assured, if a source is found it will be disclosed," she said, but added that the committee would probably not make a statement until next month.
Frances Hall from Chester-le-Street, Co Durham, whose son, Peter, died from vCJD aged 20 in 1996, said last night that she was concerned by Dr Will's remarks about baby food and school dinners. "We have, of course, discussed these things before. But it really is quite horrific and it makes me physically sick to think I could have been spoon-feeding poison to my baby ."
She said that Peter occasionally ate school meals but had also taken packed lunches.
Nick Brown, the Agriculture Minister, is to press European ministers to allow normal exports of beef from Northern Ireland because of the low incidence of BSE. The move will trigger calls for Scottish beef to be included.
17 Jul 00 - CJD - Baby food theory may explain CJD deaths
By Severin Carrell
Independent ... Monday 17 July 2000
The families of variant Creutzfeldt-Jakob disease victims called for further research yesterday after a warning that meat in baby food and school meals may be linked to deaths from the disease.
The CJD Support Network said the theory put forward by Professor Robert Will, the head of the CJD Surveillance Unit, could explain why so many young people contract the deadly variant of the disease.
Clive Evers, a spokesman for the network, which helps relatives of the victims, said: "Dr Will is not saying this is a certainty but through the research he knows about and has done he has reached the point where they have eliminated many [other] theories."
Dr Will's views were revealed by The Independent on Sunday as leading scientists on the Spongiform Encephalopathy Advisory Committee (Seac) prepared to meet in London today to discuss the discovery of a cluster of five vCJD cases linked to the Leicestershire village of Queniborough, which scientists believe are related.
An inquiry by the Department of Health, the Ministry of Agriculture, Fisheries and Food, local health experts and Dr Will's unit has been launched into the cause of the cluster. Two of the victims were 19 , two were 24 and the fifth was 35 .
Residents in the village, north of Leicester, have been asked to complete questionnaires on their diets. Ten local abattoirs are also being questioned over the techniques they used in the early 1980s.
Seac could suggest examining 10,000 tonsil and appendix samples stored by local hospitals to help uncover further data. Scientists have already tested 3,000 samples from across the UK but found no traces of vCJD.
Dr Will's theory could explain why a large proportion of the 75 vCJD victims so far detected are in their teens and 20s unlike people with other forms of the disease, who are generally in their 60s. He said it was possible that baby food and school meals which used "mechanically extracted meat" were the cause.
The Department of Health said yesterday that Dr Will's theory was well-known, and was addressed when mechanically derived meat was banned in 1989 because it could contain BSE-infected spinal cord. Its re-emergence as a theory could spark off "scare stories", a spokesman said.
16 Jul 00 - CJD - US sheep may be infected with BSE from British feed
Jonathan Leake, Science Editor
Sunday Times ... Sunday 16 July 2000
Vets have found the first evidence that sheep could have become infected with BSE. Three flocks in America were this weekend being taken to laboratories for slaughter and examination after tests showed signs of the disease.
The flocks of Friesian milking sheep were imported from Europe to Vermont within the past four years. Checks have shown that their parent flocks had eaten British-bought feed that was likely to contain material from BSE-infected cows.
Linda Detwiler, the United States Department of Agriculture vet responsible for monitoring BSE and related diseases in America, said they would be taken to Plum Island near New York, the country's most secure biological facility .
"Four sheep were confirmed positive on July 10 for a transmissible spongiform encephalopathy [TSE] - the class of diseases that includes BSE. Tests for scrapie, the TSE normally found in sheep, have proved negative so there is a distinct possibility they have BSE," she said.
Scientists know that, under laboratory conditions, sheep can catch BSE from eating infected bovine material. However, there has never been any clear evidence of this happening on farms. Some experts believe, however, that sheep with BSE would have been misdiagnosed as suffering from scrapie, which has identical symptoms.
Four tests were used on the Vermont sheep. The first looked at the brains of culled animals and found clear signs of the lesions seen in sheep with BSE-like diseases.
Samples were sent to other scientists. One said: "It could be a new form of scrapie, or BSE."
The evidence in favour of BSE is strengthened by the animals' European parent flocks having been certified free of scrapie .
Detwiler said it would take a year for further tests to confirm whether or not the sheep have BSE.
16 Jul 00 - CJD - School meals linked to CJD deaths
By Geoffrey Lean, Environment Correspondent
Independent ... Sunday 16 July 2000
Infected baby food and school meals have helped to cause the human equivalent of BSE, Britain's top watchdog on the disease fears.
Dr Robert Will, director of the government's CJD surveillance unit, believes that those foods may be responsible for the alarmingly high proportion of young people among those dying from the incurable illness, which now appears to be increasing sharply .
He was speaking to The Independent on Sunday as ministers launched an investigation into a cluster of four cases of the disease - including two teenagers - in three villages north of Leicester. The government's scientific advisory panel will discuss the cluster tomorrow.
Dr Will believes one of the greatest mysteries of the disease is why "new variant CJD", the human BSE, disproportionally hits young people.
The youngest two victims first showed symptoms at the age of 14 , and many others were also teenagers. Stacey Robinson, 19 , and an unnamed 19 -year-old man are among those who have died in the cluster centred on the Leicestershire village of Queniborough.
Conventional CJD rarely strikes people under 50, and most victims are in their 60s.
Dr Will said one explanation of new variant CJD in the young could lie in the amount of mechanically extracted meat in the food of babies and children in the 1980s.
Dr Will said that it "could have contained remnants of the spinal cord ", one of the parts of a cow which becomes most highly infected when the animal develops BSE.
"Foodstuffs which contained that material could be particularly dangerous," he said. "One possible explanation for the age distribution is that young people tend to eat these products more than the adult population."
Mechanically recovered meat was widely used in baby foods and was likely to have been common in school meals . "It was used in the cheapest products ," he added. Sausages , pies , burgers , patés and some ready-cooked meals - as well as infant foods - were particularly likely to contain it.
Dr Will stressed that he had no proof that this explained the toll among young people. Other explanations could be that children absorbed the prion that carries the disease more than adults, or that it affected their cells more.
Dr Erik Millstone, senior lecturer at Sussex University's science policy research unit and an expert on food policy, said "one very plausible explanation" of the higher rates of the disease among young people lay in the food given to babies and young children. Another cause might be that young people were more vulnerable to infection. He thought that both factors were probably involved.
The investigation into the Leicestershire cluster - by Dr Will's unit, the Public Health Laboratory Service, London School of Tropical Hygiene, Leicestershire Health Authority, the Department of Health and the Ministry of Agriculture, Fisheries and Food - is focusing on local abattoirs and their slaughtering practices .
Last week Dr Will's unit disclosed that 67 Britons are now thought to have caught the disease from infected beef, an increase of 25 on a year ago.
16 Jul 00 - CJD - Expert: School Meals May Have Spread CJD
From the Press Association
Guardian ... Sunday 16 July 2000
Infected baby food and school meals may have helped cause the human form of mad cow disease, an expert has warned.
Dr Robert Will, director of the Government's CJD surveillance unit, believes those foods may be responsible for the high proportion of young people among those dying from the illness.
His comments came as scientists investigating the deaths from variant CJD of three people who had links with Queniborough, Leicestershire, are working to pinpoint the source of infected meat the victims may have eaten 20 years ago.
Dr Will believes one of the greatest mysteries of the disease is why "new variant CJD", the human BSE, disproportionately hits young people, The Independent on Sunday said.
He said one explanation could lie in the amount of mechanically-extracted meat in the food of babies and children in the 1980s .
It "could have contained remnants of the spinal cord", one of the parts of a cow which becomes most highly infected when the animal develops BSE, he added.
He said: "One possible explanation for the age distribution is that young people tend to eat these products more than the adult population."
Mechanically-recovered meat was widely used in baby foods and was likely to have been common in school meals. Sausages, pies, burgers, pates and some ready cooked meals - as well as infant foods - were particularly likely to contain it.
Investigations into the Queniborough cases have so far shown that mother-of-one Stacey Robinson, 19, of Thurmaston, formerly lived in the village; Pamela Beyless, of Glenfield, was a frequent visitor before she died aged 24; and Glen Day, who lost his battle against the disease aged 35, lived there too. They all died in 1998.
A fourth Leicestershire man died of variant CJD in May and a fifth probable case from the county is currently "poorly" in hospital.
15 Jul 00 - CJD - Village shaken to find itself CJD blackspot
By Andrew Norfolk
Times ... Saturday 15 July 2000
It is not a comfortable experience to wake up and discover that your village has suddenly become the the focus of one of the most horrifying illnesses.
There was an air of bewilderment yesterday in Queniborough, identified by the Government as the likely centre of Britain's first cluster of cases of new-variant CJD, the fatal degenerative brain condition linked to BSE in cattle.
Four certain and one probable nvCJD cases have been recorded in Leicestershire since 1998, four of them linked to the village, six miles north of Leicester, and its 2,000 population.
Two of the victims lived in Queniborough. One - still alive - worked on a farm there and another regularly ate meat bought in the village butcher's shop, run by David Clarke's family since 1981.
Mr Clarke's award-winning shop, halfway along the narrow main street which links the village post office at one end with a beautiful 13th century church at the other, was under siege yesterday from a small army of cameras, microphones and notebooks.
"What are they all doing here?" asked an unimpressed local in the pub. "Are they waiting for people to fall over on the ground and start twitching?"
If the medical and scientific experts still have so much to learn about nvCJD, it should not be surprising that the villagers were confused yesterday, greeting questions with defiance and uncertainty.
Many knew Stacey Robinson, described by Rosemary Smith, a village historian, as a "lively and mischievous youngster" who lived in Queniborough until a few years ago but died in 1998 at the age of 19, leaving behind a young child.
Mrs Smith had also spent time in the pub chatting to Glen Day, whom she describes as a "kind and loving chap" who also died in 1998, aged 34. She also knew, but would not name because he is still living, the 24-year-old man who worked on a farm in the village before falling ill with what is thought to be nvCJD. The fourth victim with Queniborough links, Pamela Beyless, 24, did not live or work in the village but had family ties there and her father, Arthur Beyless, has said the family often ate beef bought at the local shop.
"It is all very strange how the three deaths seem to be linked to the village," said Mr Beyless yesterday. "We have been told very little but I trust the experts are looking into it."
Mr Beyless, 53, and his wife June, 41, said it came as little surprise that experts had now linked the Leicestershire cases. Mr Beyless said: "It is something we have talked about in the past - about how strange it was that three of the people who died should have come from Queniborough, or had links with it."
The couple have kept in regular contact with the CJD Surveillance Unit in Edinburgh.
Raymond Williams, 79, who regularly buys his beef in the village, said: "This is a wonderful village and I wouldn't want to live anywhere else, but it's just shaken everybody."
In the village shop, outside which the local newspaper bill proclaimed the headline: "National CJD Focus Falls on Village", Jane Johnson insisted that no one was showing any sign of panic.
"People are saying it's all a coincidence. Most of them have been very laid back about it," she said.
Mr Clarke said: "We've traded here with trust and confidence for 19 years. I can appreciate the scientific excitement, but let's hope they can get to the bottom of this quickly."
He added: "One woman who had not been in for a couple of months came in this morning after hearing the news and said that she just wanted to be there for me."
Philip Monk, Leicestershire Health Authority's consultant in Communicable Disease Control said that the investigation would focus on a period between 1980 and 1985.
"This is a very important scientific event. We have a group of patients, a cluster related in time and space and it's unlikely that this has occurred by chance. Something has happened very locally and we need to find out what that is."
The inquiry is likely to take up to two years, during which life under the thatched roofs of Queniborough may well turn to normal for all except those still coming to terms with the loss of their loved ones.
15 Jul 00 - CJD - Tonsil and appendix checks could show spread of CJD
By Valerie Elliott, Countryside Editor
Times ... Saturday 15 July 2000
The Department of Health is considering urgent tests on more than 10,000 tonsils and appendices removed in Leicestershire to assess the scale of possible infection in the county of the human form of "mad cow" disease.
The checks would be anonymous but would include every operation since 1985 and any new patients. The proposal was conceived after experts said the pattern of five cases of new variant Creutzfeldt-Jakob disease (nvCJD), the fatal brain illness, centred on the village of Queniborough, north of Leicester, could not have happened by chance . Whitehall as asked Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, to prepare a testing plan.
Dr Monk believes the cases are linked to eating beef infected by bovine spongiform encephalopathy (BSE). He has already spoken to parents and relatives in four of the five cases and has details of the victims' eating habits and where they bought their food.
He said that he did not believe the cases were linked to an abattoir but did not wish to develop any theories until he had all the relevant information. His initial investigation is to be discussed shortly by experts from the CJD Surveillance Unit in Edinburgh and the Communicable Diseases Surveillance Centre of the Public Health Laboratory Service.
Once a possible cause has been established a new control area involving more people in the community is likely to be set up for further testing.
Tests would be made on tonsil and appendix operations performed at the Leicester Royal Infirmary, its sister site, the Glenfield General Hospital, and at Leicester General Hospital. A target of 18,000 tonsils and appendices are to be analysed. So far a test of 3,000 has failed to find a positive case (UK editor's not: positive cases were found on stored body parts by the Collinge test, but the results were discarded)
15 Jul 00 - CJD - Eating habits may explain the link between victims
By Nigel Hawkes, Science Editor
Times ... Saturday 15 July 2000
Clusters of disease offer epidemiologists a priceless opportunity to identify their causes - but they are no guarantee of success.
Until now, no clear vCJD cluster has been identified. Media reports of a cluster in Kent in 1997 were misconceived, as several of the cases cited were not vCJD at all, but the classic, sporadic version of the disease. And the victims of vCJD in Kent were not very close geographically.
The Leicestershire vCJD cluster appears to be different. It could be coincidence but experts believe this is unlikely. There are two possible factors which could link the cases: genetics or the environment.
Dr Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, yesterday implied that "genetic susceptibility" was the probable link, but this seems unlikely . It would imply that the local population possessed a genetic variant not found in other parts of the country that made them more likely to develop vCJD. If they had all come from the same family, that could be plausible.
The chances are that the victims do possess a particular genetic variant - as do all vCJD victims so far - but it is a variant carried by 40 per cent of the population, and distributed evenly. There is no reason to suppose that any other variant, if it exists, would be restricted to Queniborough. The British population is too mobile and homogenous for that.
Much more plausible is a common environmental link. Dr Robert Will, head of the CJD Surveillance Unit based at the Western General Hospital in Edinburgh, said yesterday: "We have examined their occupation, where they lived and their medical history.There has been no link found with those and so we feel the disease has come from the ingestion of affected meat ."
So the investigation is likely to focus on whether there was a common pattern in the eating habits of the victims: whether they ate beef and whether it came from the same source .
By detailed questioning of the victims' families the team from the health authority will seek to discover any other common factors which might explain the cluster.
Other diseases have historically presented researchers with an opportunity for a breakthrough. For at least 20 years scientists have sought common features lying behind clusters of childhood leukaemia. Many explanations have been put forward, with radiation from nuclear plants originally the favourite.
Today epidemiologists say that the best explanation for clusters is infection. Clusters of disease are caused by people with no immunity moving into areas where the infective agent is present. There is still no proof.
At the other extreme, is the most famous story in all epidemiology, the discovery by Dr John Snow in London in the 1850s that cholera was carried by water contaminated by sewage. A cluster of cases around a particular pump in Soho led Snow to this deduction.
15 Jul 00 - CJD - The five deaths that link a village in middle England to a CJD epidemic
Jeevan Vasagar and James Meikle
Guardian ... Saturday 15 July 2000
The village of Queniborough in Leicestershire was yesterday at the heart of a national investigation into an apparent "cluster" of five victims of the human form of BSE.
For the second time in less than two years, doctors and scientists began asking whether this was just an extraordinary coincidence or something that might help explain the spread of the harrowing disease.
A teenage mother, Stacey Robinson , 19, was first to die in August 1998. Stacey, who left a young son Josh, only moved to Thurmaston, three miles from Queniborough, shortly before her death.
Pamela Beyless , a finance administrator for a mercantile bank, died aged 24 in October the same year. Until she was 18, her family lived in Rushey Mead, a suburb of Leicester close to Thurmaston. Ms Beyless moved to Hampshire in 1991 but returned to Glenfield, seven miles from Queniborough, when she fell ill in March 1996, the same month the then Conservative government announced a probable link between eating beef during the 1980s and the arrival of a horrible new disease.
Glen Day , a 35-year-old van driver from Queniborough, died in the same month as Ms Beyless.
It was then that Ms Beyless's father Arthur, a 53-year-old milkman, began asking questions: "When Glen, Stacey and Pam all died within months of each other, I spoke to Glen's dad and we discussed how strange it was that it was all Queniborough. It's such a rare disease. Pam ate well. We always ate meat. It used to be ever so cheap in those days."
The connection with Queniborough (parish population 2,297) had not gone unnoticed by the CJD surveillance unit in Edinburgh. They tried to find a link between the three cases other than the connection to the village. They could not. But last month they were back. A 19-year-old man had died in Leicester royal infirmary. His identity remains confidential but he is thought to have come from the cluster area. A 25-year-old is slowly slipping towards death nursed by his mother at home and is considered another probable victim. He once lived in Rearsby, just one-and-a-half miles from Queniborough.
Within the past week there has been a double-dose of disturbing news about the terrifying illness that is popularly known as the human form of BSE. First, the national death toll has risen sharply to 67 . Eight other probable victims remain alive but their condition, if it is vCJD, is incurable. The news of the Queniborough cluster followed.
Investigators face major difficulties. It is not life now that is under the micro scope. It is life as it was in the mid-1980s to early 1990s. Was BSE more prevalent in cattle than in other parts of the country? What abattoirs operated locally? Who were the local butchers? Where were the local restaurants and burger bars? Was there a problem with the water courses? And most difficult of all, what were the families of victims eating? Was it the same as other families who have so far been free from the disease?
The government may have been advised by statistical experts that the fact that such a high number of people appear to have caught the slow-burning disease locally is unlikely to have occurred by chance. But chance it still might be.
Robert Will, head of the CJD unit at Ed inburgh, said: "Statistical evidence of a cluster might be of importance in identifying further information on the causes of vCJD. But I am a bit cautious about it. We have previously identified clusters which in the end turned out not to be significant. That is the problem with rare phenomena _ I am still not sure we will find anything definite from this statistical anomaly."
Apparent links between some victims and Ashford in Kent are among those that have been previously examined by the unit. Members have used 1991 censuses to try to gauge the structure of local populations but sometimes their inquiries have had to go much further back.
Roy Anderson, the Oxford epidemiologist and government adviser on the disease, said the number of vCJD cases was "trickling upwards" . In 1995 there were three cases, 10 in 1996, the same in 1997, and 18 in 1998. Last year the number fell to 14, but this year there had been 12 cases already and the epidemic could be starting .
EPIDEMIC
"This year's figure is likely to be bigger than previous years. It is still too early though to say whether the epidemic will be small, medium or large."
It is understood that Leicestershire hospitals may soon be included in a national survey of tonsils and appendixes removed from patients since the late 1980s. These are areas parts of the body in which the disease is known to incubate before emerging into obvious signs. Checks carried out on tonsils and appendixes elsewhere in the country have so far failed to uncover any trace of the disease. Meanwhile Philip Monk, consultant in communicable diseases in Leicestershire, said: "We have received a lot of assistance from national experts in their field. It is early days. We may however soon be at the stage where we are close to being able to develop a hypothesis."
There did not seem to have been panic locally. "We have had people phoning in with very helpful information but we have not had worried people."
The Beyless family's faith in authority was shaken to the foundations by the death of their bubbly and sociable daughter. They no longer eat beef. But other villagers in this quintessential slice of middle England feel differently. Queniborough, with its smart thatched roofed and Tudor cottages, lies in the middle of farmland hunted by the Quorn and the Cottesmore.
There is sympathy for the vCJD victims, but strong feelings for the farmers too.
A 56-year-old farmer, who refused to give her name, said: "This is terrible. It's not going to do any good at all."
Sue Allen, 39, a villager, said: "My grandad was a butcher so I've eaten meat all my life. I buy beef now it's back on the bone. I think you'll find a lot of people who will feel very sorry for our butcher."
David Clarke, the local butcher, was near to losing his cool by mid afternoon yesterday.
He insisted: "We are talking about something from the 1980s - not something that is necessarily relevant to the present time.
"It's unfortunate that the local butcher has to take all the flak when 90% of the food bought in the village is from local supermarkets.
"We always get our meat from reputable suppliers and it's always good quality.
"The whole community is stunned by having their home village linked to this disease."
Mr Clarke said he was confident customers would rally round.
Retired hosiery worker Lucy Bigley, 76, stuffing a silverside cut of beef into her handbag said: "I've been coming here for 40 odd years. I think the disease must have been brought into the village from outside."
15 Jul 00 - CJD - How human BSE emerged
James Meikle
Guardian ... Saturday 15 July 2000
The families of victims call it human BSE, rather than its scientific name of variant CJD. Those who watched the first young people die from the horrific new disease were always convinced their loved ones had effectively been poisoned by eating infected beef .
Ten were dead - Stephen Churchill the first in May 1995 - before doctors and other scientists told the Conservative government in March 1996 this probably was the case. Now the death toll is 67 and eight others are thought to have the incurable condition, which often manifests itself in mood swings and depression, before involving the slightly shambling, swaying gait associated with BSE-infected cows. Gradual loss of bodily functions follow before a harrowing death .
Important knowledge has been gained since the momentous announcement of the probable link with beef, but the theory remains unproven if stronger than ever. The disease's signature of bright blotches of abnormal proteins in the brain is similar to that identified in BSE cattle.
All the victims have been of the same genetic make-up, one shared by 40% of the population, although whether this means they are more likely to contract the condition or simply develop clinical signs more quickly is still not clear.
The most favoured theory is that these rogue proteins are responsible for de stroying healthy ones next door and creating "holes" in the brain. The appendix and tonsils are also thought to be hotbeds for signs of the disease before it becomes outwardly apparent.
Treatment to modulate the disease , let alone cure it, is a distant prospect. Incubation periods are unknown although the suspicion is that it could be between a few years and more than 30. The guess is that most people who have died so far were infected before measures designed to protect food from the most infected parts of cows were introduced in 1989. This means some victims were actually infected as babies.
15 Jul 00 - CJD - Village CJD investigation centres on abattoirs
By Andrea Babbington
Independent ... Saturday 15 July 2000
An investigation into a cluster of CJD cases centred on a country village is now focusing on local abattoirs and their slaughtering practices, an expert said today.
Scientists investigating three deaths from variant CJD of people who had links with Queniborough, Leicestershire, are working to pinpoint the source of infected meat the victims may have eaten 20 years ago .
Dr Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, said they were looking at patterns of slaughtering in this area where meat from these slaughterhouses entered the food chain.
He said the area has many small and independent abattoirs.
But Professor Peter Smith, acting head of the Government's Spongiform Encephalopathy Advisory Committee, said that it may never be known whether the Queniborough cases were linked.
Investigators from the CJD Surveillance Unit, Leicestershire Health Authority, the Communicable Diseases Surveillance Centre and the Department of Health are also examining the victims' genetic susceptibility to variant CJD.
CJDSU director Professor Robert Will said other possible links, such as where the people lived and worked, and their medical histories, had already been ruled out by investigators.
Village butcher David Clarke, who took over his shop 19 years ago, said he was "obviously worried" by the deaths.
Leicestershire Health Authority gave Queniborough Primary School's 250 pupils a questionnaire to take home to their parents on Tuesday, asking detailed questions about where they live and what they eat, headteacher Chris Davis said.
15 Jul 00 - CJD - Britain is facing 'long, slow epidemic' of CJD
By Charles Arthur, Technology Editor
Independent ... Saturday 15 July 2000
Discovery of cluster of vCJD cases in Leicestershire may suggest a prolonged outbreak of fatal brain disease over 25 years, say scientists
A leading scientist warned yesterday that Britain faces an epidemic of CJD cases caused by BSE-infected beef, after the discovery of an apparent cluster of cases around a Leicestershire town.
Professor Roy Anderson, who made a detailed analysis of the spread of "mad cow disease" in 1966, believes the country faces a "long, slow" epidemic of variant Creutzfeldt-Jakob disease (vCJD), reckoned to be caused by eating BSE-infected food.
Senior scientists agreed the number of cases has probably not peaked, but it was impossible to say what significance the series of cases around the village of Queniborough - or even whether they are more than an accident of location.
Previous clusters, such as one in Ashford in Kent identified in 1987 where four people fell ill, had no clear cause. And the only obvious connection between the 75 people so far dead or diagnosed with vCJD is that they predominantly come from northern England or Scotland, and all have a common variation in their genetic make-up, though it is one shared by 41 per cent of the population. Experts said the danger of becoming infected with vCJD is now vanishingly small, and hundreds or thousands of times lower than it was in Britain in the 1980s.
But huge numbers of people could have eaten infected food then, and still be incubating the disease, which may take up to 25 years to show symptoms and makes the search for any causal link more urgent.
Professor Robert Will, head of the CJD Surveillance Unit (CJDSU) in Edinburgh, which is leading that search, said: "The one question I would most like to answer is how many people are going to get this terrible disease... this latest cluster only appears if you analyse where people were living when they fell ill. That might or might not be connected to the reason why they develop the disease."
Because vCJD probably takes at least 10 years to produce symptoms, an entire history of the victims would have to be examined. But that is often impossible to do. Previous analysis by the CJDSU has failed to find links such as occupation, diet or medical treatment when compared with a random group of the population.
Professor Anderson, of Oxford University's zoology department, reassessed the BSE epidemic and the latest CJD cases and said he now thinks the number of cases will probably be "in the lower bounds" of earlier estimates. Those suggested that anywhere between many hundreds and millions of people might eventually die from the disease, which causes the brain in effect to rot, leaving the victim helpless and incapacitated over a period of one to two years before death.
The first recorded death was in 1995, and 75 people have been confirmed with the disease, five from Leicestershire.
"The worst scenario of millions of deaths is unlikely," said Professor Anderson. "It is more likely the number of deaths will be in the bottom of the range."
But he said he was concerned by the increase in the number of vCJD cases this year. Thr first recorded death was in 1995. Since then the largest annual total came in 1998, when 18 people died. Last year there were just 14 cases.
"I am worried by this year's figures," said Professor Anderson. "We're now seeing the consequences of exposure [to BSE] in the early 1980s, followed by long incubation period, then cases appearing in a trickle. That's what you expect in an epidemic ."
15 Jul 00 - CJD - Queniborough, where adverts for beef look like local indignation
By Ian Herbert, Northern Correspondent
Independent ... Saturday 15 July 2000
They were flaunting their beef as they always do in the earthy Leicestershire village of Queniborough yesterday. Aboard outside the butcher's shop encouraged villagers to "Enjoy a Bar-B-Q with English rib of beef, £1.50 lb" and the Britannia pub countered strongly, advertising 8oz rump steaks on a board of its own.
Two weeks ago, these would have been the signposts of healthy rural life but now they read like local indignation as the village absorbed news that four people may have died of Creutzfeldt-Jakob disease because of their associations with the place. Statistical experts have advised that this number of cases in a single county, against 74 nationally, is no coincidence .
Arthur Beyliss, a milkman aged 53, watched his daughter Pamela - a bright, confident 24-year-old bank worker who frequently visited this village - lose co-ordination for the most elementary tasks, before the disease deprived her of all movement and speech and finally killed her in October 1998. She was Leicestershire's first victim .
Then he heard about two more victims - 35-year-old villager Glen Day , who died in the same month, and 19-year-old Stacey Robinson , a former villager, who died in August 1998. "I just thought 'cluster'," he said, yesterday. "We have been told very little but I trust the experts are looking into it."
Scientists are also examining the death of a 19-year-old man at Leicester Royal Infirmary in May and a 24-year-old man who probably also has the disease.
Even Bob Pendleton, the 74-year-old Queniborough parish councillor and churchgoer who "tends to know who's died", could only speculate anxiously yesterday. The young people "have their burgers as a quick fix", he says. "This could come from anywhere." Two of the victims, locals observed darkly, came from the same housing estate.
Few know their beefburgers better here than Rosemary Smith, 65, another parish councillor, who was born in the back bedroom in the village's solitary butcher's store, which her father ran until 1947.
"Every bit of the beast was used to make things pay back then," she said. "When my husband died I went back there to work. I made up the burgers. I knew exactly what went into them. We must get to the bottom of this."
On Mondays, in days gone by, the blood of pigs slaughtered at the butcher's would run out through the open drains of Main Street, which is still lined with picturebox thatched cottages.
"We even used to eat sheep's head when my father died. It was a good, cheap meal and we came to no harm," said Mrs Smith. But yesterday, by contrast, local schoolchildren were issued with questionnaires for their parents about family eating habits over the past 20 years.
The village's besieged current butcher, David Smith, who has been in business in the village for 20 years, was braving all this with alacrity. "Luckily, our clientele have got a little bit of grey matter between their ears and they know it has been blown out of all proportion," he said. "They buy 90 per cent of their food from supermarkets."
Similar fears surfaced in Kent in 1997 but were discounted by Edinburgh's CJD Surveillance Centre last year, it should be said, and Dr Philip Monk, consultant in communicable diseases at Leicestershire Health Authority, yesterday said it was unlikely that his county's cases were "linked geographically" either. Rather, "it was the victims' genetic susceptibility", he said.
But Professor Roy Anderson, epidemiologist in infectious disease at Imperial College, London, said the cluster threatened to push the number of vCJD cases to a new annual high , despite an encouraging fall to 14 last year after a gradual rise from three to 18 cases between 1995 and 1999.
"This epidemic is just starting ," he said. "It could be a small one but this disease has such a long incubation period [after] exposure... in the 1980s. There's an analogy with HIV and Aids [with its] 10-year incubation. This is even more difficult because incubation is even longer - 10, 20, 30 years."
As the Community Health Council called for a public inquiry, Steve Pownall, a 17-year-old villager cycling home at lunchtime with a 12oz steak he had bought from the butcher's for his supper, was phlegmatic.
He had vaguely known Stacey Robinson from schooldays and worked as a "cleaning lad" in the butcher's. There was "no problem at all", he said. "Life has to go on."