Leaks from the "secret" meeting at Keele University (9 April hyperlink below) which led to predictions of 400,000+ human  nvCJD deaths forced the UK Government to go public (28 April) to refute the predictions to maintain public calm.

However, most scientists and researchers have declined to follow the "official" line and have expressed reservations about drawing any conclusions from the test programme results thus far. It is not entirely clear why the leaks from the Keele University meeting indicated that several positive results had been found but the the Government news release states none. One possible solution is that it has been reported that "the most sensitive tests could not be used on archive samples", so positive results from these tests could have been discarded. This would explain the reservations of scientists and researchers.

The Ministry of Agriculture, Food and Fisheries and the Ministry of Health had planned to release initial conservative fatality estimates concurrently with the publication of Phillips BSE Enquiry report. A higher than expected workload at the Enquiry and the requirement for Civil Servants to "vet" the report before its release have delayed the publication of the report from Spring 2000 to Autumn 2000, at the beginning of the General Election campaign.

Document Directory

29 Apr 00 - CJD - 'I watched my vibrant daughter just waste away' for nine years
29 Apr 00 - CJD - Britain may harbour CJD timebomb, says professor
29 Apr 00 - CJD - CJD study shows no sign of epidemic
29 Apr 00 - CJD - Experts refuse to rule out epidemic
29 Apr 00 - CJD - Nightmare is not over, warns victim's mother
29 Apr 00 - CJD - Tonsils research finds no evidence of CJD epidemic
28 Apr 00 - CJD - CJD study fails to provide answers
28 Apr 00 - CJD - Keeping the lid on CJD
28 Apr 00 - CJD - Sheep to be tested for 'hidden' BSE
28 Apr 00 - CJD - Research allays fears of CJD epidemic
28 Apr 00 - CJD - Research to reveal size of CJD threat



29 Apr 00 - CJD - 'I watched my vibrant daughter just waste away' for nine years

By Terri Judd

Independent ... Saturday 29 April 2000


John Williams remembers his daughter Alison as the smiling girl who could "wrap me round her little finger.

"She was marvellous. She always had a smile on her face and we used to tease each other," he said. Yet for nine years he watched her slowly deteriorate , lose the will to live and eventually fall into a fatal coma because of variant Creutzfeldt-Jakob disease.

The first signs of the illness began in 1987 when Alison was 22. She started retreating into herself and losing her confidence. The young woman, who had always been bright and athletic, suddenly gave up her business studies course.

"We put it down to the fact that she was upset about her mother having heart surgery," the 71-year-old retired chief engineer, from Caernarvon, explained. "She lost all confidence. She walked, skied, played badminton and loved sailing but she would not go out and meet people."

Gradually, she retreated to her bedroom, staring out of the window for long periods.

In October of that year, her mother Irene, who has since died, took her to the doctors. They feared thyroid problems and diabetes but tests proved negative. Alison tried attending various training courses but she was even fearful of getting on a bus. By 1992, she was treated for a suspected nervous breakdown.

In August 1994, four months after her mother died, Alison collapsed at work and was treated for acute depression. Mr Williams was forced to watch as his daughter began to waste away, her walking became unsteady and she started to lose her memory. Noise became intolerable to her.

After endless tests, Alison's father was told there was a strong possibility his daughter had CJD but it could not be confirmed until death. "I was shattered. It was absolutely terrible. My son David [aged 34] still can't come to terms with it," Mr Williams said.

By November 1995, she became incontinent, had to be fed and thrashed about in bed. Two months later she went blind and lost control of the muscles in her tongue. In February 1996, she went into a coma and died five days later. She was 30.

The pathologist's report confirmed variant CJD, and Alison became the 14th person to be so diagnosed.

Mr Williams is a founder ofthe CJD support network. Set up in 1995, it aims to offer support to families, promote research, campaign on issues and offer accurate information about the illness.

"My daughter died at the age of 30 - not because of an accident, smoking, taking drugs, or alcohol... but because she ate contaminated beef , a food we always thought was safe."


29 Apr 00 - CJD - Britain may harbour CJD timebomb, says professor

By Charles Arthur, Technology Editor

Independent ... Saturday 29 April 2000


Britain could still be heading for a hidden iceberg of the variant Creutzfeldt-Jakob disease (v-CJD) disease, caused by eating BSE-infected beef in the 1980s. The trouble is, nobody knows for sure - and nobody knows how many years it will be before the all-clear can be sounded, if it ever is.

The Government will announce by summer if it will replace steel surgical instruments with disposable ones to minimise the risk of transmission.

Although early results from a study of 3,000 appendixes and tonsils stored at hospitals in Scotland and south-west England have been encouraging, with no "incubating" cases of v-CJD uncovered, health chiefs are still worried because the scale of infection is unknown .

"The estimates of the number who will eventually develop v-CJD ranges from hundreds to several hundred thousand ," said Professor Liam Donaldson, the chief medical officer, yesterday. "There is no change in that from this study."

Professor John Collinge, who is carrying out a similar study on tonsils, added: "This study could only give us a warning of something imminent brewing - but it is such a small number of samples that a negative doesn't really give us any reassurance at all ." He thinks many more people could die decades from now, because the v-CJD can incubate for up to 30 years. Since 1995, 53 people have died from the incurable disease.

Meanwhile the Department of Health is considering what action to take over surgery. It has already minimised the risk of transmitting v-CJD by blood donation, by removing white blood cells from donated blood and sourcing blood products from outside the UK.


29 Apr 00 - CJD - CJD study shows no sign of epidemic

By David Derbyshire Science Correspondent

Telegraph ... Saturday 29 April 2000


The first major study into the threat posed by the human form of mad cow disease has found no evidence of an impending CJD epidemic.

Tests on tissue from more than 3,000 people revealed no trace of the infectious agent linked to the fatal brain disorder, the Government's chief medical officer said yesterday. But Prof Liam Donaldson said an epidemic was still a possibility . The results came from a study of the first 3,170 of 18,000 tonsils and appendices removed in operations in the mid to late Nineties.

Prof Donaldson said: "The fact that no positives have been found is welcome news, but these early results should not be taken as indicators of an all-clear ." The eventual number of deaths from variant CJD (vCJD) could be anywhere between the hundreds and the hundreds of thousands , he said.

"This is a complex and mysterious disease and the results of today's study throw a little bit more light on it, but it's very much an evolving conundrum ."


29 Apr 00 - CJD - Experts refuse to rule out epidemic

By David Derbyshire, Science Correspondent

Telegraph ... Saturday 29 April 2000


While many CJD experts may privately suspect that Britain has been spared a major epidemic, preliminary results from a government study released yesterday offer little in the way of comfort .

Tests of 3,170 tonsils and appendices stored in hospitals found no evidence of variant CJD, the fatal brain disorder linked to eating BSE-infected beef. But because the sample was relatively small, and because so little is known about the disease, a major epidemic in years to come still cannot be ruled out.

The results also highlight the difficulty in trying to predict the spread of disease. Research published earlier this year showed that even if a survey of 20,000 tonsils came out negative, the result could still be consistent with an epidemic affecting between 50 and 45,000 people.

Variant CJD (vCJD) - the prefix "new" was dropped last year - is thought to be caused by a rogue prion protein which concentrates in lymphoid tissue. As the disease takes hold, holes appear in the brain triggering mental and physical degeneration. Until recently, vCJD could be diagnosed with 100 per cent certainty only during a post-mortem examination.

But in 1998 researchers found the rogue prion in an appendix removed from a patient at Derriford Hospital in Plymouth nine months before he died. The following year, Prof John Collinge, of St Mary's Hospital in London, showed that prions could also be detected in tonsils . The discovery paved the way for a government investigation into the scale of the infection in the population.

Prof Les Borysiewicz, from the University of Wales College of Medicine, led a scientific committee that is examining 18,000 tonsils and appendices after routine operations. As the results from the first 3,000 tests were announced yesterday, Prof Borysiewicz stressed the limitations of the study. He said: "The sample size is small and may not reflect the population as a whole. And the methods used to study the tissue material have major limitations."

The patients may have been incubating the disease, even though there were no signs in their tonsils or appendices, he added. Dot Churchill, whose son Stephen, 19, was the first confirmed vCJD victim in 1995, said she was not surprised by the findings: "They are half way through the research and this is quite a small sample. I think it's just too early to make any predictions."

But Mrs Churchill, of Devizes, Wiltshire, whose husband David chairs the Human BSE Foundation, added: "Any research that gives us any answers at all has got to be welcomed." A second study has been commissioned from Prof Collinge which will look at fresh samples of tonsil and appendix. One of the drawbacks of the current study is that the samples have been stored for a long time .


29 Apr 00 - CJD - Nightmare is not over, warns victim's mother

By Michael Smith

Telegraph ... Saturday 29 April 2000


Frances Hall, whose son Peter died of the human form of CJD four years ago, believes the government-backed study needs to broaden its scope before it has any real significance .

Mrs Hall said: "It's such a small sample. It was rather like looking for a needle in a haystack. There's still another 15,000 samples to look at. But even if nothing came up in the 18,000 it doesn't mean that there weren't very many people out there who are going to develop it because it doesn't show up until very late ."

There was also the problem that some of the samples tested went back to the early Eighties. Mrs Hall said: "I think they'll have a clearer picture if they start taking more recent random samples." Peter was 20 and a first-year student at Sunderland University when the CJD variant began to show itself.

His mother said: "He was a lovely young man, gregarious, very bright and always with his mates." Mrs Hall, of Chester-le-Street, Co Durham, first spotted something was wrong shortly before Peter's 20th birthday. She said: "I asked him what present he wanted. He didn't seem very interested, which was very unlike Peter."

Initially, the family doctor suggested he was simply depressed and put him on Prozac. But soon he had to give up his studies and his mother was nursing him 24 hours a day at home. Mrs Hall said: "We went through a nightmare - something so dreadful happening to your family and the realisation that they weren't going to get well and that you're losing. It's very difficult to live with."

Eventually, Peter was moved to the County Hospital in Durham. Mrs Hall said she virtually lived there until he died on Feb 8, 1996. A post-mortem examination confirmed Peter had the human version of CJD. Mrs Hall, who runs the Human BSE Foundation to help victims, wants more done to help doctors treating patients suffering from the human form of CJD.

She said: "We need a centralised care package for CJD cases, with centralised funding . In some areas the treatment is great and in some areas it's not as good as it should be. They need somewhere they can refer to where there's expert help." The foundation was concerned that there was a risk of complacency among the authorities which might be reinforced by the study's negative results.

Mrs Hall said: "The majority of the population think the problem has gone. I don't think they realise we still have victims of this disease. I want people to know there is a problem out there. We still have to be careful."">

29 Apr 00 - CJD - Mass tissue tests spark hope over BSE links

By Nigel Hawkes, Science Editor

Times ... Saturday 29 April 2000


A Study of tissue samples from 3,000 people has supported the view that there will not be an epidemic of the human form of "mad cow" disease.

No sign of the protein believed to be responsible for the human form of BSE was found in 3,170 tonsil and appendix specimens tested. Professor Liam Donaldson, the Chief Medical Officer, said the result was "welcome news", but added that it could not be taken as an indication of an all-clear .

However a member of a team at City University, London, which has long claimed that the final death toll would be no more than 100, said the findings made the group even more sure that they are right. "Our claims have had lots of criticisms, but we are being proved right," Philip Thomas said.

"The outbreak is now on the decline and will be all over by 2006. Today's additional evidence from tonsils is further, welcome confirmation," Professor Thomas said.

The tests of tonsil and appendix samples held in hospital pathology departments began after it was found that the prion - a microscopic protein particle implicated in variant CJD, the human form of BSE - can be detected in these tissues before symptoms become apparent.

A total of 18,000 samples are to be tested, at two centres, the CJD Surveillance Unit in Edingburgh, and Derriford Hospital in Plymouth. Yesterday's results are the first to emerge from the programme.

Les Borysiewicz, of the University of Wales College of Medicine, who chairs the steering group responsible for the tests, said that although no sample had tested positive, there were some important caveats . Professor Borysiewicz said the sample was small and the most sensitive tests could not be used on the archive samples. There was also uncertainty about the incubation period for vCJD, and how soon the prion would be seen.

The samples were collected during operations that took place between 1996 and 1998. Had any positives been seen, the implications would have been alarming: even one positive in 3,000 samples would imply that as many as 20,000 people in the whole population were carrying the vCJD agent.

More sensitive tests are to be used in another study, using fresh material from operations, which may give a clearer picture.

Professor Donaldson said yesterday that it would be "several years " before the final numbers could be more precisely predicted.

But the more extreme numbers bandied about a few years ago, of half a million victims or more , are now considered implausible. There have so far been 53 confirmed deaths from vCJD, plus two others for whom post-mortem examinations are not complete. In addition, there are another 12 people still alive but with the symptoms of vCJD.

Professor Donaldson stuck yesterday to estimates of the final toll ranging from "hundreds to hundreds of thousands ", saying that it was simply too early to say .

But Professor Thomas said: "We think this is quite wrong, and it is becoming strongly misleading. For a long time the information has been pointing in the same direction: a total number of 100 cases, though it could be as low as 75 or as high as 150."


29 Apr 00 - CJD - Tonsils research finds no evidence of CJD epidemic

Sarah Boseley, health correspondent

Guardian ... Saturday 29 April 2000


Tests on more than 3,000 tonsils and appendixes stored after routine operations have left scientists no wiser as to the number of Britons who are infected with new variant CJD, the human form of mad cow disease.

The government's chief medical officer, Liam Donaldson, admitted yesterday that although there was no trace of the prion protein - which some think is the infective agent - in any of the preserved tissue examined at hospitals in Edinburgh and Plymouth, no news was not necessarily good news .

"This doesn't take us any further forward on the size of the epidemic," he said in a briefing yesterday. "We have still to rely on the very wide estimates scientists have made."

These have ranged between a few hundred and several hundred thousand infections. So far, 53 people are confirmed to have died from the disease, which has no cure; two are suspected to have died from it but await postmortem examinations; and 12 are ill.

Yesterday's announcement of a negative result in the early stages of the retrospective population study was partly an attempt to dampen speculation about a CJD epidemic. The scientists will eventually examine 18,000 samples removed in routine operations from the late 1980s.

The exercise is something of a stab in the dark . The prion protein was found in late 1998 in the appendix of a man who had developed vCJD. In February last year, Professor John Collinge of St Mary's hospital, Paddington, found the abnormal protein in the tonsils of patients who had died of vCJD.

But scientists have no idea at what stage in the incubation of the disease the abnormal prion protein might show up in lymphatic tissue such as tonsils and appendixes.

Prof Donaldson said: "We do not even know whether any individuals who were found positive would necessarily go on to develop the disease. These research findings are another contribution to increasing our understanding of this complex and mysterious disease."

Les Borysiewicz, professor of medicine at the University of Wales college of medicine and chair of the scientific committee that is reviewing the tissue analysis, pointed out that the sample is relatively small , and of 4,166 specimens only 3,170 were appropriate for analysis.


Leaks from the "secret" meeting at Keele University (9 April hyperlink above) which led to predictions of 400,000+ human  nvCJD deaths have forced the UK Government to go public today (28 April) to refute the predictions to maintain public calm.

It appears that some positive nvCJD have been belatedly discarded as inconclusive. However, scientists and researchers are not confirming the official line.

The Ministry of Agriculture, Food and Fisheries and the Ministry of Health had planned to release initial (conservative) fatality estimates concurrently with the publication of Phillips BSE Enquiry report. A higher than expected workload at the Enquiry and the requirement for Civil Servants to "vet" the report before its release have delayed the publication of the report from Spring 2000 to Autumn 2000, at the beginning of the General Election campaign.


28 Apr 00 - CJD - CJD study fails to provide answers

By Andrea Babbington

Independent ... Friday 28 April 2000


Scientists are no closer to knowing how many people will die from human mad cow disease, despite the release today of a study aimed at predicting its effects.

No signs of variant CJD were found by Government-funded experts in 3,000 tonsil and appendix specimens removed in operations since the 1980s. However they warned the results should not be seen as an all-clear.

The scientists, based at the CJD surveillance unit in Edinburgh and at Derriford hospital in Plymouth, said these were the first findings from a survey that will eventually examine tissue from around 18,000 samples.

At a news conference at the Department of Health Professor Liam Donaldson, the government's chief medical officer, said: "The fact that no positives have been found is welcome news, but these early results should not be taken as an indication of an 'all clear'.

"The methods of analysis used on the small sample of specimens have some limitations . In addition we do not know at what point in the incubation period tissues such as tonsils or appendices would turn positive, how long the incubation period would be, or even whether any individuals who were found positive would necessarily go on to develop the disease."

Variant CJD emerged in 1995 as a previously unrecognised form of the human brain disease, and to date has claimed 53 confirmed victims in the UK.

Most experts now accept that the variant CJD is a human version of the cattle disease BSE and acquired from eating contaminated beef.

The majority of infections are thought to have occurred in the late 1980s before the introduction of controls to prevent contaminated meat entering the human food chain.


28 Apr 00 - CJD - Keeping the lid on CJD

James Meikle

Guardian ... Friday 28 April 2000


The lack of evidence of nascent traces of the human form of BSE in a survey of tonsil and appendix samples dating back to the 1980s is at best only partly reassuring .

Trawling through a few thousand results of operations undergone by a population of 50m to try and establish the likely threat from what is technically called variant CJD was always going to be a long shot , given that even now only 55 people in Britain are thought to have died from the fatal condition since March 1995, with another 12 likely victims still living.

Specialists in both the clinical disease and statistical prediction of the size of the epidemic disagree wildly about the future. Some believe we still may get away with fewer than 100 cases, although that estimate is looking less likely as the death toll mounts, while others think it still may run into hundreds of thousands of even millions . The problem is that there are still so many unknowns .

There is no conclusive proof yet that the disease came from cattle, although the analysis of the "signature'' of the disease in the brain reveals many similarities with BSE. The likelihood is that it came from eating infected beef, although other sources including vaccines and cosmetics with bovine material are possible.

The scientific establishment believes the behaviour of an abnormal prion protein is a key factor in the disease but just when does it first show itself? The incubation period of the disease may be anything from just over 4 to nearer 40 years.

It was the chance discovery of an abnormal prion in the appendix of a variant CJD victim , removed eight months before he showed the first obvious outward signs of the disease and three years before he died, that provoked the screening programme whose first results were published today. It has also accelerated changes in the use of medical instruments, with more disposable equipment being used in several types of operation and medical treatment.

There have been some important developments recently in being able to diagnose pretty conclusively the existence of the disease before death , through tonsil tests on those already showing outward signs. There is now hope, too, of being able to develop ways of "modulating'' the disease, as happened with HIV and Aids.

However, we are still a long way from developing tests for the condition before clinical signs become evident, or drugs to block it. Even before those arrive, there is a huge debate to be had on the ethics of telling victims of a disease that is incurable.

But the longer deaths from the appalling symptoms - including dementia , aggression and loss of bodily control - remain on a relatively small scale, the more ministers and their advisers are having to balance the cost of prevention. This includes expensive precautions on spreading nvCJD through blood donations and potentially infected food, against the as-yet-unquantified risk of a doomsday-sized epidemic . The government once rubbished anyone who suggested that people might die from eating BSE-infected cattle. Now it is ready to err on the side of caution .


28 Apr 00 - CJD - Sheep to be tested for 'hidden' BSE

James Meikle

Guardian ... Friday 28 April 2000


The government is to increase significantly research into whether sheep are harbouring "hidden " BSE and posing a new food risk to humans.

A programme was announced yesterday to find new tests for live animals, comparisons of sheep BSE with cattle BSE, and ways of determining whether scrapie, a similar disease long endemic in British flocks, is helping to mask a potentially more devastating threat to people's health.

It follows concern among scientific advisers that there are still gaps in knowledge about how BSE and similar conditions may be affecting livestock.

Researchers around the world are being invited to compete for a share of an extra 5m worth of work next year. Since 1986 90m has already been spent on research into the issue.

As few as one in eight sheep farmers might be obeying the law by reporting suspect cases of scrapie, government vets warned earlier this month, and ministers have announced plans to tag or tattoo 40m sheep in an effort to improve the tracking-down of diseases and the recording of animals' provenance.

Much of the work will involve sheep purchased from New Zealand farms, where there is no scrapie , to act as controls for the experiments. There is no evidence that scrapie is a danger to humans, and BSE has only been transmitted from infected cattle to sheep via the laboratory.

One theory on the cause of BSE is that the remains of scrapie-infected sheep were eaten in the meat and bone meal which used to be commonly fed to cattle and sheep, and the infective agent then began working in a totally different way in cows. Even if that is not the case, it is possible that BSE was spread from the remains of infected cattle to sheep before the 1988 ban on the use of such feed.

Although all sheep fed contaminated materials would probably now have died naturally, scientists see no reason why BSE , if it exists in sheep, should not have become endemic like scrapie. Animals may simply have become carriers of the disease without displaying outward signs. In the laboratory, sheep BSE already appears to behave differently from BSE in cattle.

There is no test for identifying scrapie in live sheep, BSE in live cattle, or, until it is clinically apparent, variant CJD (the human form of BSE) in people while still alive. There are promising signs for a scrapie test using blood that is being developed by the US department of agriculture.

The government believes successive measures introduced to protect humans by banning parts of cattle and sheep from food are sufficient at present, but needs to know more about how both BSE and scrapie work and their minimum infective "doses" .

Baroness Hayman, the agriculture minister, said: "Although our scientific knowledge in this field has increased and evolved significantly, there are still identifiable areas where more work needs to be done."


28 Apr 00 - CJD - Research allays fears of CJD epidemic

Staff and agencies

Guardian ... Friday 28 April 2000


A study aimed at predicting the threat to humans from BSE has found no evidence of the human form of the disease in 3,000 specimens of human tissue, the Department of Health said today. The government's chief medical officer, Professor Liam Donaldson welcomed the news, but warned that the early results should not be taken as an indication of an "all clear". Government-funded experts analysed about 3,000 tonsil and appendix samples routinely taken from people since the 1980s. Preliminary results from the survey, which will eventually examine about 18,000 samples, found no traces of the abnormal prion protein associated with new variant CJD, the human form of mad cow disease, which has killed 52 people in the UK.

The brain disease is believed to be acquired from eating BSE-contaminated beef . Sufferers are thought to have become infected in the 1980s before the introduction of controls to prevent contaminated meat from entering the human food chain. BSE was first diagnosed in November 1986 and nv-CJD in 1995.

Some scientists have warned of a CJD epidemic affecting up to a half a million people during the next decade, but because the incubation period of the disease is unknown, it may still be too early to predict how many will succumb to nv-CJD.

Professor Donaldson said: "The fact that no positives have been found is welcome news, but these early results should not be taken as an indication of an 'all clear'. The methods of analysis used on the small sample of specimens have some limitations .

"In addition we do not know at what point in the incubation period tissues such as tonsils or appendices would turn positive, how long the incubation period would be, or even whether any individuals who were found positive would necessarily go on to develop the disease."

Nonetheless, today's results were being seen by experts as the most reliable indication so far of how many people are infected with nv-CJD.

The rogue prion proteins which cause nv-CJD are thought to accumulate in the tonsils and appendix, making them potentially useful for keeping track of the disease.

Experts from the CJD National Surveillance Unit in Edinburgh and Derriford Hospital in Plymouth carried out the study.

The research followed the death from new variant CJD in 1998 of Devon coastguard Tony Barrett. His appendix had been removed in 1995. When scientists re-examined it after his death they found the abnormal prion protein, showing that his condition could have been diagnosed before he fell ill .

Since then the two research centres have been collecting tonsil and appendix samples removed between the mid-1980s and late 1990s.

"The critical gap in our knowledge remains not having a reliable diagnostic test to detect the presence of the abnormal prion protein at the pre-clinical stage of the disease." said Professor Donaldson.

"The government is committed to spending some 26 million over the next year into research into CJD and BSE issues to address these important outstanding issues."

Commenting on today's results, a spokeswoman for the CJD Support Network said: "Obviously this is very welcome news as it may indicate that there are not going to be huge numbers of people with the disease. But we have to remember that this was a small study of only 3,000 samples and we will want to wait and see the outcome of the rest of the study.

"Meanwhile, we will have to go on supporting people and their relatives who are struck down by this devastating disease."

Like the bovine form of the disease (BSE), nv-CJD is a poorly understood condition that attacks the brain, leaving it full of holes like a bath sponge.

Victims display symptoms of dementia , becoming unsteady and confused and losing their memory . As the brain degenerates, they decline to a state where they cannot walk , talk or look after themselves . There is no known cure.


28 Apr 00 - CJD - Research to reveal size of CJD threat

By Andrea Babbington

Independent ... Friday 28 April 2000


The size of the threat posed by human mad cow disease will be revealed today as scientists release research that will give the best indication yet.

About 2,000 tonsil and appendix samples routinely taken from people since the 1980s have been analysed by government-funded experts.

The results are likely to provide the first reliable estimates of how many people are infected with new variant CJD.

The results of the study, which has been carried out by experts from the CJD National Surveillance Unit in Edinburgh and Derriford Hospital in Plymouth, have been shrouded in secrecy.

The research followed the death from new variant CJD in 1998 of Devon coastguard Tony Barrett.

His appendix had been removed three years earlier. When scientists re-examined it after his death they found the abnormal prion protein, showing that his condition could have been diagnosed before he fell ill.

Since then the two research centres have been collecting tonsil and appendix samples removed between the mid-1980s and late 1990s.

The brain disease first emerged in 1995 but it is believed that many victims probably become infected in the 1980s before the introduction of controls to prevent contaminated beef from entering the human food chain.

To date nvCJD is known to have claimed 52 victims in the UK, but whether or not many more people will succumb depends on the length of its incubation period.

The longer the incubation period, the larger the number of people likely to be infected with nvCJD but not yet showing any symptoms.

The rogue prion proteins which cause nvCJD are thought to accumulate in the tonsils and appendix, making them potentially useful for keeping track of the disease.


09 Apr 00 - CJD - Scientists fear thousands (400,000+) may have CJD

Jonathan Leake Science Editor

Sunday Times ... Sunday 9 April 2000


Scientists have moved one step closer to confirming that thousands of Britons could be infected with variant CJD, the human form of "mad cow" disease (BSE).

A government-funded study of appendix and tonsil tissues taken from 2,000 people since the 1980s is understood to have found a small number of samples which tested positive for the infective prion particles that cause the disease. If confirmed, the results suggest the number of people who might get the disease could reach thousands.

The researchers are, however, thought to be worried about the accuracy of the tests and are to carry out checks be-fore announcing them to a government committee next month.

Details of the work were discussed last week when researchers into CJD, BSE and other prion diseases held a private meeting at Keele University .

The event, organised by the agriculture and health ministries and by the government's Biotechnology and Biological Sciences Research Council, was conducted amid unprecedented security . It was not announced to the press and every scientist attending had to pledge not to reveal anything.

The study was ordered to try to find out how many people were at risk of contracting variant CJD. Half of it is being carried out by Professor James Ironside at the CJD national surveillance unit in Edinburgh, and the other half is being done at Derriford hospital in Plymouth by Dr David Hilton.

Both men attended last week's meeting and are understood to have held discussions with other scientists on the nature of their results.

The research followed the death of Tony Barrett, a Devon coastguard, who died of variant CJD in 1998. His appendix had been removed in 1995. When it was re-examined after his death scientists found abnormal prion protein - showing that his illness could have been diagnosed before he became ill.

Since then the two research centres have been collecting tonsil and appendix samples re-moved between the mid-1980s and late 1990s - the period when exposure to BSE-contaminated meat was at its peak.

If the tests do find any positive cases of variant CJD then the implications would be serious. Extrapolating the result to the population as a whole suggests that for each positive result picked up by the study, 30,000 people in the population would get the disease .

So far there have been more than 50 deaths from variant CJD with at least a dozen more people currently dying from the disease. Among them is a woman who gave birth just before she was diagnosed and whose child is now suspected of being the first case in which the disease has passed from mother to child .

UK Correspondents Note : It seems there have been approximately 8 positive results in 2000 tests. Kenneth Calman, the previous Chief Medical Officer is on record as saying 1 positive result per thousand tests equated to 50,000 cases countrywide. On this basis it would seem the lower limit of the forthcoming nvCJD epidemic is in the region of 400,000 deaths .

This number of potential fatalities explains why the US is anticipating a ban on US visitors to the UK for a considerable period (take the FDA link from the left side bar to the proceedings of the FDA meeting that initiated the ban on US blood donors who spent 6 months cumulatively in the UK between 1980 & 1996, scroll down and click on "Det Norsk Veritas Risk Assessment" by Philip Comer, then search - CTRL + F - for "travel").