Document Directory

29 Mar 00 - CJD - Pressure for earlier detection method grows over fear of explosion in CJD cases
29 Mar 00 - CJD - Half CJD victims in twenties
26 Mar 00 - CJD - Sheep flocks to be tagged and tested for BSE
22 Mar 00 - CJD - EU blood donors face travel check
18 Mar 00 - CJD - Tests can diagnose CJD before death
17 Mar 00 - CJD - 40m sheep to be tagged to monitor transmission of BSE from
10 Mar 00 - CJD - Friends' tributes to CJD victim
09 Mar 00 - CJD - Girl, 15, is youngest to die from CJD
08 Mar 00 - CJD - Teenager dies from CJD
06 Mar 00 - CJD - Seven mothers 'in CJD risk'
06 Mar 00 - CJD - GPs put on CJD alert after victim gives birth
05 Mar 00 - CJD - Tragedy of the baby feared born with CJD
05 Mar 00 - CJD - Can CJD be passed from mother to child?
05 Mar 00 - CJD - Anatomy of a tragedy - How vCJD kills
25 Feb 00 - CJD - Bad blood
23 Feb 00 - CJD - France backs down on CJD blood ban
16 Feb 00 - CJD - Action begins on German beef ban
14 Feb 00 - CJD - mad cow Found in French Abattoir
12 Feb 00 - CJD - EC warning to Germany over ban on British beef
12 Feb 00 - CJD - Germany to face BSE legal action
12 Feb 00 - CJD - EU poised to start action against Germany over beef
07 Feb 00 - CJD - French attack EU's critical BSE report
06 Feb 00 - CJD - EU condemns France for lax curbs on BSE
29 Jan 00 - CJD - Young mother is suspected CJD victim
29 Jan 00 - CJD - Young Mother Is Suspected CJD Victim
25 Jan 00 - CJD - Coroners 'concealed' BSE deaths
24 Jan 00 - CJD - French to test herds for mjx disease
14 Jan 00 - CJD - Injunction against ban on UK beef ruled out
12 Jan 00 - CJD - Britain has 24 times more CJD than in France
08 Jan 00 - CJD - Millions at risk from CJD, say EU scientists



29 Mar 00 - CJD - Pressure for earlier detection method grows over fear of explosion in CJD cases

David Montgomery

The Scotsman ... Wednesday 29 March 2000


The human death toll from the BSE crisis is a matter of heated debate among medical experts, with estimates ranging from a few thousand to several million across Britain. The findings of the two-year BSE inquiry, chaired by Lord Philips and which will be published later this year, are expected to shed more light on the extent of infection. Predictions that many more will die from the disease are based on an assumption that it has a long incubation period and has proved impossible to detect in healthy individuals.

Until this month, there was no means even of confirming whether someone suspected of having new variant Creutzfeldt-Jacob Disease (nvCJD), the human form of mad cow disease, was infected until after they died and a brain biopsy could be carried out. Doctors now claim to have sufficient techniques to be able to say a person is "probably" suffering from nvCJD, which should allow a more accurate prediction of future rates. The new criterion means that patients should be able to be identified as probable nvCJD sufferers within six months of the first signs. However, there is still no test available to detect those who may be infected with nvCJD in the pre-clinical stage, before they become ill.

Professor James Ironside, of the National CJD Surveillance Unit at the Western General Hospital in Edinburgh, said they were involved in the development of a blood test to screen for the disease. He said: "We have a number of means of telling if someone is likely to have the disease when they become clinically ill, but there is no reliable way of doing that at present on an apparently healthy individual to see if they are carrying the disease. The development of a blood test would be the best way to do that."

Such a test remains one of the Department of Health's research priorities and is also being pursued by pharmaceutical companies. Other studies are also being conducted by the government, including a retrospective programme of screening tonsils and appendix tissue from 3,000 patients in Scotland and 15,000 in the south-west of England. If successful, it is hoped this will help to identify how widespread the disease is in the general population.

Traces of a protein implicated in nvCJD were found in appendix tissue removed three years ago from Tony Barrett, 45, a coastguard, as part of a routine operation at Torbay Hospital in Devon. The operation took place eight months before he showed any symptoms of nvCJD and almost three years before his death .

If it is possible to detect infection on a wide scale, a screening programme could be set up, but this would pose the dilemma of whether it was best to tell victims they were incubating the disease. The figures released by the government indicate that some people, notably those in their twenties, appear to be more at risk than others.

The slaughtering of millions of cattle at risk of being infected with BSE, and only allowing young cattle into the food chain, have ensured meat should now be free of the disease. Other measures, such as the ban on beef-on-the-bone and the issuing of ID cards or passports to cattle, were intended to reduce the risk of the disease being transferred to humans.

But scientists believe that if people are going to get the disease they will, in nearly all cases, already be incubating it. The issue is fraught because there is no treatment for nvCJD and there is no guarantee that a person carrying the infective agent will develop the disease. Government figures have revealed that nearly half of the victims of new variant Creutzfeldt-Jakob disease, the human equivalent of mad-cow disease, were in their twenties. Of the 52 people who have died from the illness in Britain since 1985, 23 were aged between 20 and 29, raising fears that young people could be most at risk from an nvCJD epidemic predicted by scientists.

Experts at the National CJD Surveillance Unit at Edinburgh's Western General Hospital believe it may be possible that young people were exposed to more of the BSE agent than older age groups. A study of past and present cases of nvCJD is now exploring the likelihood that variations in diet and the body's efficiency in absorbing the protein-corrupting agents believed to cause the disease could increase the risk of becoming infected. Previous work by the Edinburgh unit has shown that diet is the most likely factor in becoming infected with nvCJD.

Beef itself, which is mostly skeletal muscle, was unlikely to have high levels of infection, but meat products containing infected nerve tissue, such as sausages, burgers and patties, were more likely to be contaminated. However, researchers at the hospital have also warned that the figures must be treated with caution because of the relatively low number of deaths and difficulties in pinpointing the cause of the disease.

Professor James Ironside, of the CJD unit, said yesterday that it was unclear why more young people were being infected with the disease, but he said one possibility was that younger people could be more at risk because they absorbed protein more efficiently through the gut than older generations. The efficiency of the lymphoid tissue in the tonsils and the spleen may allow the disease to be transferred more easily. "That's a possibility being looked at in terms of dietary differences," he said.

"There may be distinct dietary, physical and physiological differences in how the gut works in different age groups. All of these could be important factors, or just one, or even a combination of all three." However, he said no clear-cut findings had yet emerged for these or other possible factors, such as the likelihood that lower-income groups may be exposed to more processed meat products containing BSE-infected matter.

He said: "That does not seem to be emerging. The reason why young people are being affected more by this illness is not clear. We are still dealing with relatively small numbers, and these make it difficult to get any statistical power from them."

The figures on nvCJD deaths were disclosed on Monday night in a written reply from the junior health minister, Lord Hunt of Kings Heath. They showed that 23 people had died aged 20 to 29, 14 had been in their thirties, eight aged ten to 19, four were in their fifties, and three in their forties. There were no recorded cases of nvCJD deaths of people aged over 59 or under ten, the minister added. Lord Hunt also published a list showing, in each case, the month the symptoms began, the month of death and the month nvCJD was confirmed. The list shows that three deaths occurred in 1995, ten in 1996, ten in 1997, 17 in 1998, 11 last year, and one so far this year.

Prof Ironside said the true extent of the spread of nvCJD would be unclear for some time, since its incubation period could be upwards of 15 years . "That means we don't know whether these cases are just the beginning of a larger number, and the disease will increase in frequency as the years go on, or they will just remain at a low rate," he said.

Part of the research being carried out at the Edinburgh unit to establish the cause of nvCJD in humans, and whether there are any particular risk factors for developing it, involves visiting each patient suspected of having nvCJD and comparing their circumstances with someone of the same background, age and sex.

Professor Robert Will, the scientist in charge of the project, said the reliability of information gathered was variable, because the patients' health meant they were often unable to provide details themselves, and the long incubation period of the disease meant memories were sometimes sketchy.

"We have great difficulty in knowing what exposure [to infection matter] was in the food chain, and we don't know whether there was more than one relevant exposure," said Prof Will.


29 Mar 00 - CJD - Half CJD victims in twenties

By Nigel Hawkes Science Editor

Times ... Wednesday 29 March 2000


NEARLY half of the victims of new variant CJD, the human equivalent of BSE, were in their twenties, according to official figures.

Lord Hunt of Kings Heath, a junior health minister, said in a written reply to Parliament that of the 52 people who had died, 23 were between 20 and 29, 14 in their 30s, eight between 10 and 19, three in their 40s, and four in their 50s.

There were no recorded cases of nvCJD deaths of people aged over 59 or under 10.

The most plausible explanation is simply that the developing brain is more susceptible to infection than the mature one.

A less likely explanation is that some form of exposure unique to children - vaccination, for example - could be the route of infection. Sir Richard Southwood, who chaired an inquiry in 1989 to assess the risks to humans of BSE, judged that vaccines containing infected material were a greater risk than eating beef.

But the warnings were not made public because the Committee on Safety of Medicines judged that it had a duty not to cause a panic .


26 Mar 00 - CJD - Sheep flocks to be tagged and tested for BSE

Jonathan Leake, Science Editor

Sunday Times ... Sunday 26 March 2000


Under suspicion: scientists plan to tag 40m sheep as fears grow over the spread of BSE

Government scientists have become so concerned about the threat of BSE appearing in sheep that they are setting up a national programme to test thousands of flocks. It coincides with a decision by ministers to order civil servants to draw up a scheme to tag all the nation's 40m sheep.

The moves follow new laboratory evidence suggesting that sheep diagnosed with scrapie, a related disease, could actually have been suffering from BSE .

There is no hard evidence of any sheep having contracted BSE, but scientists have warned that the risk of such a diagnosis is getting higher. They have also asked civil servants to draw up provisional plans for a cull. This would, however, be enacted only if BSE were confirmed in sheep.

Professor Peter Smith, acting head of the Spongiform Encepalopathy Committee (SEAC), the independent scientific group that advises the government on BSE, CJD, scrapie and other so-called prion diseases, said the consequences of finding BSE in sheep would be "very profound". He added: "We could be very close to getting the bad news. If we do get BSE in sheep, then the implications will perhaps be more serious than the discovery of BSE in cattle."

In cattle, BSE infects only certain parts of the body. In sheep, it affects the whole animal, meaning that more infected material may enter the human food chain.

Scientists have known for some years that sheep can catch BSE from eating infected material. The difficulty in spotting the disease has been that it has identical symptoms to scrapie, which is believed to be harmless to humans.

New research, carried out at the Institute for Animal Health (IAH), has now suggested some cases diagnosed as scrapie may, in fact, be BSE . The findings have been backed by further research showing that animals immune or resistant to scrapie are indeed vulnerable to BSE.

Professor Chris Bostock, director of the IAH, who is also a member of SEAC, said: "We have found sheep that should be resistant to scrapie that have been diagnosed with it. This raises a suspicion they could have had BSE. Samples taken from 200 infected animals are being tested to determine the strain of prion. We have had 30 results so far, none of which showed BSE. The results for the remainder are still awaited."

Despite the fact that infective feed is now banned, if BSE had once entered the sheep population, it could still persist. In cattle BSE passes from cows to calves and in sheep scrapie is renowned for its ability to transmit between generations and across flocks.

It was confirmed last week that the agriculture, fisheries and food ministry (Maff) is putting on trial a test developed by the American government that can detect prions - the agents believed responsible for both BSE and scrapie - in sheep from two weeks old.

It has also established a dedicated laboratory in Weybridge, which is preparing the protocol for a national programme to screen every flock in which scrapie or any other prion disease is diagnosed. Britain has 78,000 sheep farmers.

Maff officials say the scheme for tagging sheep will be announced within weeks and be operating by October . A spokesman for the National Farmers Union said the impact on sheep farmers of BSE would be "very serious" , but stressed that Britain already had regulations that made its lamb among the safest in the world.

The SEAC has advised Maff it needs to make contingency plans for a possible cull . Ian McConnell, professor of veterinary sciences at Cambridge University and a member of SEAC, said: "We have told Maff it would be wise to have an operational plan in case BSE is found. Having been caught short over culling cattle, they do not want to be caught again."

The ministry would not discuss plans for a cull, although it confirmed it wanted to eradicate all prion disease in sheep. "For practical and commercial reasons, it is not possible to discuss the government's response if BSE is found in sheep, but clearly it would take account of the risk to public health," a spokesman said.


18 Mar 00 - CJD - Tests can diagnose CJD before death

By Ian Murray, Medical Correspondent

Times ... Saturday 18 March 2000


New tests have been developed that make it possible for the first time to diagnose Creutzfeldt-Jakob disease in patients before they die .

The breakthrough, which involves taking an image of the brain and a biopsy from the tonsils of a suspected victim, enables doctors to determine with reasonable confidence whether the person is suffering from the human form of "mad cow" disease, or BSE.

Until now a diagnosis has been possible only by taking a brain biopsy after the death of a patient. With the new method it will be possible to reach a quicker and more accurate picture of the spread of the illness . Living "probable" CJD victims are therefore to be included for the first time in the monthly figures showing the number of cases of the disease.

Interim figures show that at present there are 12 living patients with the incurable disease, and brain biopsy results are awaited for three more suspected victims who have died.

So far there have been 52 deaths from new variant CJD - the type believed to be associated directly with BSE.

Pat Troop, the Deputy Chief Medical Officer, said the new tests would allow "more accurate predictions about the future of this disease".


10 Mar 00 - CJD - Friends' tributes to CJD victim

By Simon De Bruxelles

Times ... Friday 10 March 2000


Friends yesterday paid tribute to Claire McVey after it was disclosed that the 15-year-old Devon schoolgirl had become the youngest victim of the human form of "mad cow" disease.

They said, however, that Claire probably did not know she was suffering from the incurable disease which she may have contracted from eating infected meat.

Last December, the local fire brigade in Ilfracombe, where she went to school, raised £6,000 from a sponsored hike to send her on a holiday to Disney World in Florida with her mother and a nurse. At the time it was thought Claire was suffering from a brain tumour.

Sub officer Barry Webb, 35, said yesterday: "She seemed to have an amazing, calm acceptance about what was going to happen. She realised that she had to make the most of her time. Our memory of her is as a very brave, resolute young woman."

By the time of her death in January she was, according to one friend "like a very young child". She showed symptoms of dementia and was confined to a wheelchair. One classmate at Ilfracombe School and Community College, said: "She was a bright, blonde, really likeable girl."

Claire's mother Annie gave up her job as a nurse to provide full-time care for her daughter. They lived in a neat, detached cottage in the North Devon village of Kentisbury before Claire had to be admitted to hospital.

Yesterday Mrs McVey declined to talk about her daughter. Friends said that she has still not come to terms with the diagnosis that it was Creutzfeldt-Jakob disease (CJD), that was responsible for her death at the Derriford Hospital in Plymouth. The hospital, however, said that the results of a post-mortem examination "confirmed a diagnosis of new variant CJD".

Scientists are still unable to predict if an epidemic of CJDwill hit Britain. The incubation period for the disease could be ten to 14 years and it may be another decade before the true picture is known.


09 Mar 00 - CJD - Girl, 15, is youngest to die from CJD

By Sean O'Neill

Telegraph ... Thursday 9 March 2000


A 15-year-old girl who has died after a long illness is believed to be the youngest victim of the human form of mad cow disease.

Claire McVey died in January from suspected variant Creutzfeldt-Jakob disease, which is linked to consumption of BSE-infected beef . If vCJD is confirmed as the cause of her death, she may have contracted it in infancy. Since vCJD was identified in 1992 there have been 52 deaths from the disease in Britain. The average age of those who have died is 29 .

Claire was a pupil at Ilfracombe Community College in Devon. An inquest into her death has been opened and adjourned pending scientific investigation of tissue samples. But the CJD surveillance unit in Edinburgh has been notified of her death and she has been included in the latest statistics recording deaths from the illness.

She died at Derriford Hospital in Plymouth, where a post mortem examination was carried out. A spokesman said: "We can confirm that we conducted an autopsy on a teenager which confirmed a diagnosis of variant CJD."

Dr Mark Kealy, consultant in communicable diseases at North Devon health authority, said there was no danger of the disease having spread to her family or classmates. He said: "One assumes that she must have had links with bovine offal and consumed it in the past before the controls were put in place and properly enforced.

"What we are seeing at present are people who were presumably infected in the mid to late Eighties . It is possible this girl may have contracted the illness when she was a toddler ."


08 Mar 00 - CJD - Teenager dies from CJD

by PA News

Evening Standard ... Wednesday 8 March 2000


A teenage girl has died from new variant CJD, it was confirmed today.

The case of the human form of so-called mad cow disease was confirmed by an autopsy , said a spokeswoman at Derriford Hospital in Plymouth.

No details of the case, which occurred in Devon, were being given.

The spokeswoman said: "We can confirm we carried out an autopsy on a teenager which confirmed a diagnosis of NV CJD".

Doctor Mark Kealy, consultant in communicable disease control at the North and East Devon Health Authority, said the girl probably contracted it through eating infected beef between five and 10 years ago.


06 Mar 00 - CJD - Seven mothers 'in CJD risk'

by Jo Revill

Evening Standard ... Monday 6 March 2000


Seven mothers run a very small risk of having contracted CJD following the birth of Britain's first baby suspected of suffering from the fatal disease, it emerged today.

Officials said there was a "vanishingly small" chance that instruments used in the delivery of babies at the NHS hospital in the West Midlands were contaminated, even though they were sterilised after each Caesarean operation.

No case has yet been recorded of the human form of mad cow disease being passed from mother to child, nor has there been a case of CJD being transmitted via medical equipment.


06 Mar 00 - CJD - GPs put on CJD alert after victim gives birth

By Christopher Leggett

Telegraph ... Monday 6 March 2000


Family doctors are being alerted after surgical instruments used when a CJD sufferer gave birth were reused on several other women.

Doctors are to advise patients concerned about what health officials describe as a "theoretical" risk of infection. Equipment used in a caesarean section was removed from use when the woman was diagnosed with CJD, the human form of BSE.

Doctors at a West Midlands hospital discovered the woman's illness last autumn, two months after she had given birth at a nearby hospital. Neither the mother, who is said to be severely ill, nor the hospitals can be identified because of an injunction.

Prof Rod Griffiths, the director of public health at the West Midlands NHS Executive, said yesterday that the women who underwent subsequent operations would not be contacted unless the CJD sufferer's baby girl was diagnosed with the disease.

He said: "Anyone who is concerned should contact their GP, who will be in a position to tell them where and when it happened so they can allay anxieties. "There is a risk of unnecessarily worrying everyone who has had a caesarean; they might be perfectly normal for the rest of their lives but worrying about it."

CJD sufferers display symptoms of dementia, becoming unsteady, confused and losing their memory. Death normally occurs about six months after the onset of the disease.


05 Mar 00 - CJD - Tragedy of the baby feared born with CJD

Jonathan Leake, Science Editor

Sunday Times ... Sunday 5 March 2000


Doctors believe this may be the first baby to be infected with variant CJD (vCJD), the human form of mad cow disease, by her mother. The woman had a caesarean section four months ago at a west Midlands hospital and has since been found to have the disease.

The hospital has confirmed that other expectant mothers and their babies were also accidentally exposed to vCJD after contaminated instruments used on the sick woman continued in use on the maternity ward for two months afterwards. The Department of Health has launched an inquiry, but the hospital cannot be named for legal reasons.

The infected mother was showing many of the early symptoms of vCJD when her child was delivered, but was not properly diagnosed until later.

Doctors now believe that the woman's daughter has contracted the deadly disease. Brain scans indicate that she has characteristic tissue damage. If this is confirmed, it will establish that the disease can be passed from mother to child. The instruments used in the section were sterilised, but the infective agent, known as a prion, has been shown to be invulnerable to conventional sterilisation methods.

CJD experts told the hospital two months ago that up to 10 women were at risk . The director of public health said they had not contacted them. "There are no tests for vCJD and no cure. If we told them then they would have 10 or more years of anxiety." He said the authority was considering informing their GPs.

Professor John Collinge, head of the Medical Research Council's prion research unit at Imperial College in London, said: "There is no means of sterilising surgical instruments against prions."

The infected woman, who is in her twenties, was formally diagnosed only two months ago. The illness has killed 51 people in Britain.

This weekend the woman was severely ill at home. One family member said: "Nobody knew what was wrong with her at the time. We are desperately sorry for all the women and babies who will have to spend years wondering if they are infected and then possibly go through the same tragedy as us."


05 Mar 00 - CJD - Can CJD be passed from mother to child?

Jonathan Leake

Sunday Times ... Sunday 5 March 2000


Can CJD, the fatal brain disease linked to BSE, be passed from mother to child ? Doctors suspect this baby is the first case...

They took her baby away before dawn. It was less than 24 hours after the mother - let us call her Janet - had given birth, but already the medical staff could see her baby was failing to feed and in urgent need of help.

The nurses were not too surprised. Janet, who cannot be identified for legal reasons, had spent two weeks of her pregnancy in the hospital, diagnosed with severe depression. She had also undergone the trauma of a caesarean section. For a few days, they thought, they would have to help her and the baby get used to each other, and assist with feeding.

But that night turned out to be the last Janet would spend with her little girl, Amanda. The depression grew worse; most worryingly, not all was well with the baby, which reacted strangely to tests.

It was another two months before the pieces of the jigsaw finally fell into place. By then doctors, realising Janet's condition was unlike any usual psychiatric illness, had put her through an intense set of brain scans: to their dismay, they found degenerative changes indicative of new-variant Creutzfeldt-Jakob disease (vCJD). Further tests confirmed the presence of abnormal "prions" - the agent found in cattle suffering from BSE, known colloquially as mad cow disease.

Variant CJD has claimed the lives of 51 people in Britain. The only thing they had in common was youth - most were between 18 and 40 years of age - and a genetic make-up that is shared by about 40% of the population.

Though the disease can only be diagnosed with complete certainty after death, more than a dozen other people are believed to have the symptoms . But up to now there has been no suggestion that the devastating condition might pass from mother to child. Baby Amanda may be about to change all that.

Even after several weeks, Amanda was failing to swallow and had to be fed through a tube. She also seemed unable to gain significant weight. Doctors, filled with foreboding, tried a brain scan. They saw what the experts had prayed would never happen: the lesions and plaques indicative of vCJD in a child that had never eaten meat. Nor does the damage seem static. It is getting worse.

The diagnosis, which is likely to be confirmed by other tests within the next few weeks, means the threat already hanging over everyone who ate infected beef products in the 1980s and early 1990s, when BSE was at its height, could extend to future generations. The seemingly long in-cubation period of vCJD means that Amanda may be the first, but not the last, child born with the disease.

It is four months after Amanda's birth and Janet is getting ready to see her baby. Her mother, Sara, and a nurse wash and dress Janet, and support her as she staggers downstairs, head rolling, arms waving. Home is a suburban, three-bedroom semi in a quiet cul-de-sac; a peaceful setting at odds with the drama within.

Amanda arrives in the morning, brought from the nearby hospital where she is cared for at night.

Janet can still speak, but the mental deterioration she has suffered makes her speech and mannerisms childlike. Her balance has become so unsteady she must drink from a toddler's beaker.

Repeatedly, she says she is waiting for her baby to arrive. "She's gorgeous, she's so lovely," she says, over and over again. After a few minutes the baby does arrive, in the arms of the nurse who accompanies her wherever she goes, helping to feed her through a tube and dealing with emergencies.

Today, however, Amanda is fine. She and Janet sit together on the sofa, the proud mother trying to control her own drooling and involuntary shaking while also trying to wipe away the saliva that trickles from her daughter's mouth.

Amanda, however, hardly responds. At four months old she weighs less than 10lb, is unable to see and makes little more than yelping noises.

Sara builds the day around the time her daughter and granddaughter spend together - six hours. She knows that, probably in just a few months, Janet is unlikely even to remember she has a baby - let alone recognise Amanda.

The doctors have warned her that the abnormal prions that have rampaged through the part of Janet's brain that control short-term memory and motor function are now attacking the rest of her mind. She has been told there is no hope of a cure.

"I was so proud of my daughter and pleased to be having a grandchild. I could never have imagined that I would be nursing both of them to their deaths," says Sara.

"We are just an ordinary family, but we're being destroyed by a man-made disease that should never have happened."

Just two years ago Janet was preparing to be married. She was a happy, talkative and affectionate young woman, full of life. "She was always laughing, telling jokes and making friends wherever she went," says her mother. "She was out most weekends, dancing or meeting people."

Born in the south, Janet was raised in the Midlands by her mother after she separated from Janet's father. The women were not just mother and daughter, but friends. When she was 18, Janet joined Sara in a catering business they ran with success for some years. Janet's bubbly character hid a sharp business edge.

"It was hard, but Janet made it work," recalls Sara. "She was so cheerful and friendly that the lads all loved her. I hired the staff and organised the cooking and she looked after the customers. Thanks to her, we did good business."

The same cheerful nature and good looks attracted would-be boyfriends. In her early twenties, Janet was engaged to a young man called Andrew with a wedding planned for mid-1998.

Looking back, Sara realises that the fraught months before the wedding were the first sign something was seriously wrong . Just as the ceremony and reception had been booked, Janet called it off. She never gave a reason and Andrew was devastated. "He was distraught," said Sara. "He begged her to come back, but she wouldn't have any of it."

The next few months saw Janet plunged into frequent depressions and moodiness , which Sara had never seen before. They were just the start. Later she also began suffering from pains in her back and legs, which made her mental state even worse.

It was during this time that Janet struck up a new relationship with another young man. It was a troubled match and the couple parted in late 1998, only to reunite shortly afterwards. When Janet became pregnant she was delighted at first - but the disease was stealthily taking its toll.

The pregnancy that followed was, says Sara, a nightmare . Janet's personality was deteriorating as the disease got more of a grip and, as with many vCJD victims, she went through a phase of being nasty to everyone around her.

"One of the midwives told me they had never met anyone as evil as Janet," says Sara. "We all still thought she was depressed at her relationship breaking up. Nobody spotted how ill she really was."

Twice during her pregnancy Janet's mood became so black, the pains so severe and her ability to carry out simple tasks so poor that she was admitted to hospital. Doctors could find nothing wrong and she was not allowed to stay more than a few days.

Despite her extreme suffering, the doctors had not progressed beyond their diagnosis of acute depression by the time Janet came to give birth.

Only some weeks later, in November last year, did she undergo the brain scan that indicated she was suffering from something worse.

Many people with vCJD have been diagnosed so late in the course of the disease that they are no longer capable of understanding what is happening. Not so with Janet.

"She has twice told me she knows she has got mad cow disease and that she is going to die," said her mother. "I hated to think what must have gone through her mind when she lay in bed at night." Janet now uses sedatives to help her sleep.

All known cases of vCJD have proved fatal, which is why the implication of her daughter's preliminary diagnosis - that transmission of the disease from mother to child is possible - is so worrying. Experts, however, have expected it.

While emphasising that he cannot comment on individual cases, John Collinge, the Medical Research Council's professor in charge of prion research, said: "It was something that was always on the cards. In sheep scrapie, a similar prion disease, the disease passes from ewes to their lambs. There is good evidence that in cattle about one in 10 infected animals transmits the disease to a calf.

"The prion that causes BSE is identical to the one found in humans with vCJD, so it is logical that there would be a risk of vCJD jumping from mothers to children."

If Amanda's diagnosis is confirmed, scientists will want to find out just how she got it. One possibility is that the vCJD prions can pass across the placenta - something that has already been observed in sheep.

Fred Cohen, professor of pharmacology at the University of California and co-author of the Nobel prize-winning research that identified prions as the agent behind BSE and vCJD, said the diagnosis on Amanda was "very scary" .

"For her to be showing the symptoms of vCJD so early she would probably have had to contract it in the womb, perhaps very early on in her foetal development," he said. "It means any woman unknowingly incubating the disease could pass it to their children, too. If that potential is proven it makes this whole situation much more serious ."

What happens to Amanda will be of concern to one small group - the children of other women who have died of vCJD. There are believed to be at least three such cases , all currently healthy.

Anthony Bowen, whose son was born three weeks before his wife Michelle died of the disease, has told interviewers how he lives with the prospect of his son also becoming ill.

Such concerns are, however, already way beyond Janet's understanding. All she knows is that, for now, she has a beautiful baby to try to look after. As Sara said: "They haven't got much time left and all I want now is to make sure they spend as much of it together while they can still enjoy each other."

Names have been changed to protect identities


05 Mar 00 - CJD - Anatomy of a tragedy - How vCJD kills

Staff Reporter

Sunday Times ... Sunday 5 March 2000


Scientists believe BSE and its human form, variant CJD (vCJD), are caused by abnormal versions of agents called "prions".Formed from small proteins, normal prions have been found in many mammal and bird brains, although their role is unknown. They are easily destroyed by the body's enzymes.

Abnormal prions, however, are resistant to most of the body's defences. They are so strong they can survive an hour at near boiling point . Inside the body, they clump together into small lumps which attack and destroy the nervous tissue around them.It is unknown how abnormal prions are created.

But some scientists suggest that introducing some to the body, by eating infected beef for example, starts a chain reaction changing other normal prions to abnormal. Eventually they destroy the nervous system. Prion diseases or similar conditions are found in sheep, cats and other animals as well as cattle. Not all are fatal. But the four known prion diseases that affect humans are all deadly.

Too little, too late

1986 Ministers told that animals are dying of a new brain disease. No slaughter ordered until the following year. Officials reassure consumers that the disease cannot be passed to humans

1989 The government bans sale of meat and dairy products from BSE-infected cattle; it bans use of cattle brains and spinal cord in food products

1990 Mass cattle cull underway. Many European and other countries ban British beef. Ministry of Agriculture and House of Commons agriculture committee say there is no threat to human health

1994 Number of cattle diagnosed with BSE reaches 120,000

1995 Two teenage boys die of what is later diagnosed as vCJD. 600 BSE-infected cattle a week still entering the food chain . John Major says there is no link between BSE and vCJD and that British beef is safe

1996 Stephen Dorrell, health secretary, admits there is a link between the two diseases, but claims it is minimal. The government orders the slaughter of 4.6m cattle. Worldwide companies including McDonalds and British Airways ban British beef

1998 Number of slaughtered cattle reaches 4.4m at a cost of £4.4bn

1999 EU allows Britain to export beef, but France and Germany refused to import it

2000 BSE inquiry report is delayed for six months


23 Feb 00 - CJD - France backs down on CJD blood ban

by Zoe Morris

Evening Standard ... Wednesday 23 February 2000


The French government decided today not to ban blood donated from people who had visited the UK , despite fears about the transmission of "mad cow disease".

Experts warned any ban would leave the French with a 10 per cent shortfall in its blood supplies.

Plans to outlaw donations from anyone who visited Britain during the Eighties and early Nineties had been on the cards because French ministers are concerned at the incidence of CJD - the human form of the disease. But health minister Dominique Gillot announced today they would be stepping up checks on British blood products rather than opting for a ban.

She said: "It would appear that the exclusion of donors who have stayed in Britain is not feasible in the campaign to control the risk of transmission of Creutzfeld Jacob Disease."

Medical experts are still unclear about whether CJD can be transmitted though blood, but the Government announced in July 1998 that white cells would be removed from all blood donated in England and Wales as a precautionary measure because scientists suspect this is where traces could be found.

The Department of Health insists any "theoretical risk" is outweighed by the dangers of depleting supplies. A spokesman said today: "There is no evidence that CJD is transmitted by blood transfusions or blood products." (UK editor's note : Absence of evidence is not evidence of absence - it seems that MAFF have learnt nothing from the BSE crisis).


22 Mar 00 - CJD - EU blood donors face travel check

James Meikle

Guardian ... Wednesday 22 March 2000


Blood donors throughout the European Union are to be asked if they have visited Britain or other countries with BSE so that scientists can assess the risk of them spreading the human form of the disease, CJD.

Medical advisers to the European commission say checks should be made on all British meat exports for 16 years up to 1996 and on the dangers of transmission of other diseases through blood transfusions before deciding whether to ban donations from people who have stayed in BSE-affected countries.

EU-wide blood controls may be introduced, including the filtering of white blood cells from donations, since these are thought most likely to carry the infective agent of CJD.

EU scientists accept there is no proof this will help cut the risk of transmission of CJD, despite the fact Britain already does it as a precaution.

The Department of Health last night expressed scepticism over whether EU-wide bans would make blood any safer.

The latest recommendations from the commission's medical products and medical devices committee reflect the difficulty in balancing the potential risks of transmitting the incurable CJD with the consequences bans on potential donors could have on blood supplies. There currently is a world shortage of blood.

There is no proven or even probable case of CJD having been passed on through blood transfusions.

There is as yet no test for identifying CJD in people's blood and no ethical code on whether people who might be identified as having it before symptoms occur should be told of the diagnosis.


17 Mar 00 - CJD - 40m sheep to be tagged to monitor transmission of BSE from cattle

James Meikle

Guardian ... Friday 17 March 2000


The government is to tag all Britain's 40m sheep as scientists demand more screening to check that BSE has not leapt from cattle.

The measures are expected to be in place by October to stave off threats of legal action by the European commission to ban all live sheep exports for the country's alleged failure to enable livestock to be traced back to the farms of their birth.

The government wants to prove that there could be no repeat of the BSE in cattle fiasco when for years there was no watertight tracking system to find out the provenance of cows that fell ill.

A commission official has told animal welfare activists led by TV scriptwriter Carla Lane that the government had told him that the "permanent marking" of all sheep and goats involving tagging would be in force within months.

A letter signed on behalf of Robert Coleman, director general of the commission's health and consumer protection directorate said his officials would "monitor the compliance" by Britain and would not proceed with legal action "providing the proposed timetable for regularisation is adhered to."

Ms Lane, director of the Protesters Animal Information Network, which opposes all live sheep exports now topping 1m animals a year, said: "The government is breaking the law . Farmers are not supposed to get subsidies unless they have proper traceability on their farms.

"There is a growing worry about BSE in sheep and if it is in sheep, God help us all ."

Leaders of the industry which provides about 385,000 tonnes of sheep meat to consumers and receives £300m each year in so-called headage payments on sheep through the common agricultural policy, last night disputed whether BSE could appear in sheep.

They also warned the cost of implementing the scheme could prove another body-blow to hard pressed farmers but some conceded measures to ensure traceability of food could help farmers survive any further collapses in public confidence.

Scientists in Britain and Europe have been concerned about the possibility of BSE in sheep for at least four years . Although there is no evidence this has happened outside the laboratory, the government's spongiform encephalopathy advisory committee which advises on both measures against both BSE and its human form, variant CJD, have called for more checks on sheep brains for tell-tale signs of the disease.

Minutes of a meeting held last month and published yesterday state that the committee "agreed further work needed to be done on screening of sheep for evidence of infection by the BSE agent but concluded that at present there was no need for further public health controls".

John Thorley, chairman of the National Sheep Association, said yesterday there was little likelihood of BSE in sheep.

In addition, most people ate sheep as lambs between six and 10 months old, when the evidence suggested that BSE took years to develop. But he warned the industry could not bear excessive costs.

Henry Lewis, an official at the meat and livestock commission, said: "We have to be whiter than white and endorse a more rigid regime of indentification and traceability."


25 Feb 00 - CJD - Bad blood

Paul Brown

Guardian ... Friday 25 February 2000


First they spurn our beef. Now the French have banned blood donations from people who have visited Britain

How does it feel to be an international pariah? Offer British blood to save the life of a Canadian, American or New Zealander and you will be politely turned down. To make matters worse, any of the citizens of these three countries who visited Britain for a six-month period between 1980 and the end of 1996 are also banned from giving blood.

(UK Correspondents note: other reports state that the French have not banned blood donations from non-UK citizens who have spent 6 months in the UK betwee, 1980 and 1996 as this would have reduced the blood supply by an unacceptable 10.5%)

The reason is that Britain's national dish is tainted. Visitors from the former colonies are certain to have wanted to sample the delights of roast beef and yorkshire pudding. As a result, so the blood-donor agencies of these countries have decided, their citizens might be infected with the "British disease" of new variant Creutzfeldt-Jakob disease, the human equivalent of mad cow disease.

These visitors may already have unwittingly sentenced themselves to an early death from this terrible disease by visiting Britain and they do not want them to pass it on to fellow citizens back home in their pints of blood. Now the French, who claim their would not eat our beef anyway, are anxious to rub salt in the wounds by banning any of their citizens, who have so much as set foot in Britain in the same period, from giving blood.

A visitor from outer space might be forgiven for avoiding Britain as a place of pestilence.

Let's examine the evidence for our quarantine as unclean inhabitants of an offshore island. So far there have been 52 victims of the human variant of this disease (known as nvCJD). It is a crippling and always fatal disease that rots the brain and reduces people to cabbages. The tragedy is that it strikes mostly at teenagers and young adults; middle-aged parents face watching their children die slowly and painfully .

The problem is that the scientists do not know where it will all end. They believe that the incubation period is so long that eventually 400,000 of us may die of it - although this is an absolute upper estimate it is a terrifying possibility. Who will live and who will die no one knows, the only certainty is that anyone who ate beef in the 15 years or so after the early 80s might get the disease.

It has taken a long time for the awfulness of this all this to sink in. From the first it was clear that the scandal had wrecked an export industry. It has also made Britain a byword for disgraceful farming practices, cutting corners to make a fast buck by turning cows into cannibals .

It was some time before we realised that this in turn made our blood contaminated - or at least potentially so. In 1998 the UK expert advisory committee said there was a theoretical chance that transmission could come from infected blood. This would be through the white blood cells. As a result all white blood cells were removed from all donated blood in the UK. As a further precaution all plasma used for the manufacture of blood products, like the factor VIII, used as a clotting agent by haemophiliacs, is imported from abroad.

The shame of all this was compounded last year when our former colonies began to single out their own citizens who had visited the mother country as potentially unclean too - and now the old enemy, the French, have joined in.

The department of health in London is taking a balanced view. There was no evidence that nvCJD had ever been transmitted in human blood. The two cases of nvCJD in France have almost certainly come from French people eating cattle exported from Britain which were already carrying the disease.

The action of the four countries in banning British blood is understandable. After all there is the terrible memory of the tainted blood scandal of the 80s when 1,200 haemophiliacs were infected with HIV from imported human plasma products and 600 died. Three thousand got hepatitis C. In France this is currently an emotive issue, because a former prime minister and two other ministers were tried last year for failing to prevent the use of Aids-infected blood by the French health service in the 80s. The current crop of politicians does not want to risk the same with CJD-contaminated blood.

The problem for the French is that up to 22% of their blood donors have visited Britain in the last 15 years and they are already critically short of supplies in the hospital service. The risk of losing lives because of shortage of blood in hospitals is greater than the chance of getting nvCJD. But the stigma of Britons being classed as unclean remains.

The long running CJD inquiry is still to report and the evidence of the appalling behaviour of the ministry of agriculture in trying to cover it all up still haunts us. Richard Packer, permanent secretary at MAFF, has just taken early retirement. It may be coincidence but at the same time new, unproven, suggestions have emerged that cattle brain may have been used as a substitute for egg white in mayonnaise , and Mr Packer was accused of turning a blind eye to the scandal at late as 1995 - ignoring the fact that six years after high-risk bovine offal was banned from sale some of it was still reaching the food chain.

How long will it be before we can again be proud of having British blood coursing through our veins?


16 Feb 00 - CJD - Action begins on German beef ban

By Martin Fletcher European Correspondent

Times ... Wednesday 16 February 2000


The European Commission started legal action against Germany yesterday for failing to lift its ban on British beef.

David Byrne, the Consumer Protection Commissioner, who is already taking France to court for the same offence, said he must ensure that EU law is respected .

The move was welcomed by Conservative MEPs, who said it was obvious that neither France nor Germany had any intention of lifting their illegal bans. "There can be no more stalling or prevaricating," Robert Sturdy, their agriculture spokesman, said. "We have long argued that it is best to begin legal proceedings to pre-empt any further attempts at wriggling on this issue."

The German Government has said that it wants to lift the ban but must first obtain approval of its 16 regional governments. Their representatives are to meet on March 17 and several remain opposed.

The Commission has given Germany until late March to state its position. If it fails to lift the ban, it will be sent a formal indictment or "reasoned opinion". If it still fails to comply, it will be taken to the European Court of Justice in Luxembourg, where the average case takes about 20 months.

Mr Byrne said he hoped the matter could still be resolved out of court. "We can clearly avoid a legal confrontation if Germany complies quickly with the law," he said.

The EU ban on British beef was imposed at the height of the "mad cow" scare in 1996 and lifted last summer after Britain introduced some of the world's toughest safeguards against BSE.


14 Feb 00 - CJD - mad cow Found in French Abattoir

from Ian Sparks in Paris

Evening Standard ... Monday 14 February 2000


A cow suspected of being infected with BSE came within hours of entering the food chain in France last week, the country's veterinary officials have admitted.

The animal was said to have been displaying "clear signs of mad cow disease" and was spotted minutes before it was due to be slaughtered at an abattoir in Brittany.

The cow was destined for consumption in france, where a ban on British beef is still in place despite the European Union having lifted the trade bar.

The animal did not have a compulsory medical certificate and was reported by a worker as it was being transported to the Gallais slaughterhouse in Sourn. Abattoir manager Louis Rossignol said the cow had "appeared to be in good condition" when he bought it along with 27 others in Finistere last week.

Mr Rossignol said: "It was while it was being transported that the person in charge of receiving the animals noted that a cow wasn't in a normal condition . It had appeared well when leaving the market. I don't know what could have happened."

The entire herd has been slaughtered as a "precautionary measure" and tests for BSE are being carried out on the animal's carcass by the veterinary agent DSV.

A veterinary spokesman said: "If we find this animal was infected with BSE, we will consider prosecuting the abattoir."

If the case is confirmed, it will be the sixth case of BSE in France this year and the 87th since the disease was first identified.

DSV said it was also investigating why the animal did not have a medical certificate . Later this month France will begin a national programme of BSE testing to determine how widespread the disease has become.


12 Feb 00 - CJD - EC warning to Germany over ban on British beef

By Ambrose Evans-Pritchard in Brussels

Ielegraph ... Saturday 12 February 2000


The European Commission is preparing to take legal action against Germany for failing to lift its ban on British beef.

David Byrne, the Consumer Affairs Commissioner, has drafted a warning letter to Berlin, the first step in legal procedures that could ultimately lead to the European Court of Justice. The decision must be endorsed by the 20 commissioners at a meeting next Tuesday in Strasbourg, but commission sources have said the outcome is a foregone conclusion.

Unlike France, which is openly defiant and already faces court action , Germany has effectively stalled since the EU voted to lift the ban last August, pleading the complexities of its constitutional system. Each of Germany's 16 federal Länder has the power to make its own decisions on food safety issues. Several have taken a vehement stand, especially in northern regions, where beef consumption collapsed by 30 per cent when the BSE crisis erupted.

The Commission can only target the German government, which, in turn, says it is powerless to act. The Bundesrat, the voice of the Länder, is due to vote on the issue on March 17. However, the lengthy legal process means that it will take at least two years before the case ever goes to court.

The BSE inquiry was given a six-month extension, until September, yesterday after Lord Phillips, the Appeals Court judge in charge, asked for more "thinking time".


12 Feb 00 - CJD - Germany to face BSE legal action

By Martin Fletcher, European Correspondent

Times ... Saturday 12 February 2000


The European Commission threatened yesterday to take legal action against Germany as well as France for refusing to lift its ban on British beef.

The 20 commissioners are expected to agree to begin the legal process next Tuesday after months of apparent stalling by the German authorities while the Commission tried unsuccessfully to resolve the Anglo-French "beef war" through diplomatic means.

Such a move would raise the prospect of the European Commission being simultaneously engaged in high-profile legal battles over British beef with the EU's two largest member states.

It took France to the European Court of Justice in January.

The German Government has said that it wants to lift the ban but argues that there are procedural problems to be overcome, as half a dozen of Germany's 17 regional governments remain opposed .

"The Germans have told us there will be a vote of the regional ministers in the Bundesrat on March 17, but we are not prepared to risk any further delays," a spokesman for David Byrne, the Consumer Protection Commissioner, said.

"The commissioners will be asked by Mr Byrne on Tuesday to agree to start legal proceedings in order to prevent any further delays."

The first step would be to send Germany a "letter of formal notice" saying that it appears to be violating EU law. If Germany's response was unsatisfactory, it would be sent a "reasoned opinion" or indictment and, if it still refused to lift the ban, it would be taken to the European Court in Luxembourg.

The process would be stopped immediately if the Bundesrat voted to lift the ban on March 17.

The EU's ban on British beef was imposed in 1996 at the height of the BSE scare, but it was lifted last summer after Britain introduced some of the world's tightest safeguards against the cattle disease.


12 Feb 00 - CJD - EU poised to start action against Germany over beef

By Stephen Castle in Brussels

Independent ... Saturday 12 February 2000


Germany faces the start of legal action next week over its failure to lift the ban on British beef imports, after the European Commission finally lost patience yesterday.

David Byrne, the European Commissioner for health and consumer protection, will ask his 19 fellow Commissioners to take the first legal step against the German government - a written warning - at a meeting in Strasbourg on Tuesday.

Brussels is embroiled in a case against France, which has refused to remove the embargo, and that has been referred to the European Court of Justice in Luxembourg. Legal proceedings there are expected to take about 18 months.

Germany is in a different position because it has promised to lift the beef ban, but argues that it still needs time to get the approval of its upper chamber, the Bundesrat, which is set to debate the issue on 17 March.

Taking the first legal steps next week would be designed to ensure that the German timetable does not slip, and to put the European Commission in a position to act quickly if the vote on 17 March goes against lifting the ban.

Several German state governments represented in the Bundesrat are on record as saying they want the ban to stay.

"We are still confident that the German government will lift the ban on British beef", said Beate Gminder, spokeswoman for the European Commission.

A ban was introduced at the height of the mad-cow scare in 1996.

Berlin should have begun permitting imports of British beef from 1 August last year, when every other EU nation bar France did so, following a European decision.

If the Commission goes ahead, as expected, with its warning letter on Tuesday, Berlin will have several weeks to respond, and the deadline is expected to be set for a date after 17 March.

If the reply is unsatisfactory or the ban stays in place, Brussels would then send a "reasoned opinion", the final stage of warning before the matter is referred to the court in Luxembourg.


07 Feb 00 - CJD - French attack EU's critical BSE report

By Patrick Bishop in Paris

Ielegraph ... Monday 07 February 2000


The French agriculture minister has hit back at a highly critical European Commission report on France's handling of the bovine spongiform encephalopathy epidemic.

Jean Glavany claimed that France had one of the most rigorous systems in Europe and was "top of the class" when it came to tackling mad cow disease. The minister's defence followed the publication of a report by commission veterinary surgeons expressing concern over lax procedures in identifying and reporting BSE. It disclosed that French farmers were still using animal products in cattle feed, despite a European Union-wide ban.

The report offered the first official backing to years of allegations that France has covered up cases of BSE. M Glavany hinted that the report may have been intended to punish his government for its refusal to conform with European law and lift its ban on British beef. The claim seems difficult to sustain given that the four-member commission team carried out its research in May and June, several months before France decided to maintain the embargo.

French determination to defend the ban seems likely to be reinforced by a leaked customs report in Le Monde yesterday which showed that French imports of British beef products doubled between 1988 and 1995, making France the second most exposed European country to the risk of BSE.

The commission inquiry found that suspect animals were examined by technicians rather than vets, as is the case in Britain, before slaughter. In some cases animals showing BSE-type symptoms were quickly diagnosed as suffering from other neurological diseases, without any subsequent tests being done.

The inspectors were most concerned at the discovery that traces of meat and bonemeal were found in six per cent of cattle feed, despite a 1996 ban on using animal protein to feed ruminants. The report repeated the widespread suspicion that French farmers are reluctant to report suspected cases of BSE. When cases are proved, the entire herd is destroyed.

French officials say that the government pays generous rates of compensation in order to encourage reporting and insist that the highest standards of discovery are in place. They point out that only 82 cases of BSE have been officially reported and only three people have been diagnosed with Creutzfeld-Jacob disease, suspected as being the human variant.

"I imagine this mission doesn't just examine France but all European countries," said M Glavany. "I would very much like to know the results from those other countries." Officials in London said inspectors had visited Britain twice in 1999 without finding any grounds for fresh concerns.


06 Feb 00 - CJD - EU condemns France for lax curbs on BSE

By Ambrose Evans-Pritchard in Brussels

Telegraph ... Sunday 06 February 2000


The European Commission has published a highly critical report on the failure of the French authorities to control a growing epidemic of bovine spongiform encephalopathy (BSE), casting serious doubts on France's claim to have the disease under control.

The report, which describes a series of lax procedures that would be unthinkable in Britain, comes a week after the decision by the European Commission to take France to the European Court of Justice for its refusal to lift its illegal ban on British beef. France claims that its hands are tied by the recommendations of its independent food safety agency.

The country has reported 82 cases of BSE so far and recently announced its third case of new-variant Creutzfeld-Jakob disease in humans. There have long been allegations of under-reporting of BSE by the French authorities, but this is the first time that the European Commission has taken a stand on the matter.

The report found that inspections of suspect animals before slaughter were performed by technicians rather than vets and emergency slaughters were "not carried out at all during weekends and at night". This contrasts with a rigorous enforcement regime in Britain, where a vet must be present in all cases of emergency slaughter. A number of farmers has been prosecuted for failing to comply, facing fines of up to £20,000.

The report, carried out by four EU vets from May 31 to June 4 last year, found that "a number of potential BSE cases have entered emergency slaughter without suspicion and consequent sampling". There was lax compliance with rules demanding special certificates for possible BSE cases and basic information on clinical signs was not collected. "In one case, there were no records at all ."

Animals suffering from neurological disorders were instantly diagnosed as victims of non-BSE diseases, even though it was impossible for an untrained observer to reach such a conclusion without doing follow-up checks over a period of at least two weeks. "In some cases, listeriosis was assumed for the final diagnosis, due to the sudden onset of the signs. The animals were sent for emergency slaughter without additional investigations."

One animal with clear neurological signs of BSE was treated as having a brain tumour and was dispatched "without further observation, treatment or traditional examinations". The report said that the meat in such cases was not incinerated, but was sold for human consumption. In violation of French regulations, "veterinarians do not always follow the course of the disease, and often rely on data given by the owner". There was no systematic compliance with requirements to take blood, urine, and cerebro-spinal fluid from each "legitimate suspect".

The 200,000 French head of cattle that die before slaughter each year, known as "fallen stock", were not considered as suspect and were admitted into the food chain without further notice to the authorities. This contrasts sharply with British practice, where cases of fallen stock are treated as a potential risk and must be inspected by a vet.

The report said that French farmers were not provided with systematic information to help detect BSE, relying only on videotapes. In the rare instances when BSE was diagnosed, farmers displayed "a certain hesitation" in informing the authorities and there was no evidence that the French government was taking action to ensure proper reporting.

Samplings of French cattle feed showed that 4.2 per cent contained traces of meat and bone meal, despite the EU-wide ban on the use of animal protein in cattle feed, thought to be the original cause of the BSE epidemic. The French government says this is the result of cross-contamination of meat and bone feed given to pigs and poultry.

But the British Ministry of Agriculture has blamed similar, or lower, trace levels for the unexpected continuation of BSE in Britain long after the 1989 feed ban. To ensure that there is no such cross-contamination in Britain, the Government maintains a ban on meat and bone meal for pigs and poultry, putting British farmers at a disadvantage.


29 Jan 00 - CJD - Young mother is suspected CJD victim

By Samantha Savage

Independent ... Saturday 29 January 2000


An unnamed 24-year-old woman is in a serious condition with a suspected case of new variant Creutzfeldt Jakob Disease, the human form of mad cow disease.

She is being cared for at Walsgrave Hospital, Coventry, where she is being examined by specialists in the the brain disease.

The Hospital's chief executive David Loughton said that doctors were still awaiting the result of tests to establish the diagnosis.

Mr Loughton said the woman, who has not been named, was admitted to the hospital earlier this month. She was being cared for on a neurology ward, he added.

The woman's three-month-old daughter is also seriously ill in hospital, but the child is not thought to have the same illness.

The woman will be taken to St Mary's Hospital in Paddington, west London, for tests next week, but her baby will not undergo further examination for the brain disease, which is believed to have an incubation period of at least four years.

New variant CJD (nvCJD) is thought to be caused by eating beef infected with the cattle disease bovine spongiform encephalopathy (BSE).


29 Jan 00 - CJD - Young Mother Is Suspected CJD Victim

From the Press Association

Guardian ... Saturday 29 January 2000


A 24-year-old mother is in a serious condition in hospital with a suspected case of new variant Creutzfeldt Jakob Disease, the human form of mad cow disease.

She is being cared for at Coventry's Walsgrave Hospital, where she has been seen by specialists from the CJD surveillance unit based in Edinburgh who fear she may have the brain disease.

The Hospital's chief executive David Loughton said that doctors were still awaiting the result of tests to establish the diagnosis.

Mr Loughton said the woman, who has not been named, was admitted to the hospital earlier this month.

The woman's three-month-old daughter is also seriously ill in hospital, but the child is not thought to have the same illness. The woman will be taken to St Mary's Hospital in Paddington, west London, for tests next week.

Her baby will not undergo further examination for the brain disease, which is believed to have an incubation period of at least four years.

New variant CJD (nvCJD) is thought to be caused by eating beef infected with the cattle disease bovine spongiform encephalopathy (BSE).


25 Jan 00 - CJD - Coroners 'concealed' BSE deaths

By David Brown, Agriculture Editor

Telegraph ... Tuesday 25 January 2000


The number of victims of the human form of bovine spongiform encephalopathy could be higher than official figures suggest because coroners refused full inquests on some of them, it was claimed yesterday.

A campaign group, Inquest, said that a number of families had been denied inquests on victims of the new variant Creutzfeldt-Jakob disease (vCJD) which has been linked to BSE in cattle, after coroners decided that the deaths were due to "natural causes".

So far 49 people in Britain have died from vCJD according to Government figures but the group, which is demanding tougher ministerial guidelines for coroners, said it was possible that some cases had "slipped through the net". Figures which are expected to show an increase in the number of victims will be issued next month.


24 Jan 00 - CJD - French to test herds for mjx disease

By Patrick Bishop in Paris

Telegraph ... Monday 24 January 2000


France is to test tens of thousands of cattle to try to evaluate the prevalence of mad cow disease.

The decision, announced yesterday by the health minister, Dominique Gillot, followed calls for a similar screening process to be established in Britain before the French government would agree to lift the embargo on British beef. France is following a system pioneered in Switzerland to identify cases of BSE.

New cases are identified every 10 days or so in France. The herds are then destroyed and farmers compensated. France said it wanted to set an example to the rest of Europe and to satisfy public concerns about food safety.


14 Jan 00 - CJD - Injunction against ban on UK beef ruled out

By Stephen Castle in Brussels

Independent ... Friday 14 January 2000


The European Commission decided yesterday not to seek an immediate court injunction lifting France's embargo on British beef .

The Commission said it took the decision on the advice of its legal service, which believes an application would be highly unlikely to succeed . The case before the European Court of Justice, complicated by a counter-case by France against the Commission, is expected to take at least 18 months.

The Commission said it had not ruled out taking legal action soon against Germany, which has also failed to lift the ban.


12 Jan 00 - CJD - Britain has 24 times more CJD than in France

By Stephen Castle in Brussels

Independent ... Wednesday 12 January 2000


News of Britain's first big beef export order to Europe was marred yesterday by French predictions that the UK faces a rash of new cases of CJD , the human equivalent of mad cow disease.

Members of a French parliamentary delegation conducting an inquiry into food safety supported Paris's continuing beef ban, criticised British safety standards and pointed out that 48 cases of CJD have been detected in the UK as against two in France .

On a visit to Brussels, André Angot, a vice president of the committee, criticised arrangements in the UK, arguing that traceability, slaughtering and testing methods are unreliable . Pointing out the disparity in the number of cases of CJD in the UK and France, he said: "There are probably many people who are contaminated in Britain and who will show symptoms in seven or eight years or, at least, over the next 15 years ."

Félix Leyzour, the president of the committee, which was set up by the French national assembly, backed the Paris government's decision to block UK beef imports. "I think the government has taken a good political decision," he said.

Indications of the depth of French resistance to a lifting of the ban contrasted with the British Government's coup in achieving a big beef export order to the Netherlands.

The contract with Ven International, worth £2.5m a year, is to supply hotels and restaurants with premium beef. Officials estimate that the order will be for around 350tons, equivalent to 700,000 steaks. The meat will be processed by St Merryn Meat near Truro, Cornwall, one of two companies licensed under the Date-Based Export Scheme backed by the EU.

Despite yesterday's success, exporters are sanguine about the prospects of recovering their former markets, particularly since France - once the biggest importer of UK beef - remains a closed market.

According to the European Commission, Britain exported 105,803tons of beef to France in 1985. Trade for that year to the EU was the equivalent of 202,500tons or £457m and a further 71,500tons worth £63m went to the rest of the world. When the mad cow crisis broke in 1996 exports plummeted .


08 Jan 00 - CJD - Millions at risk from CJD, say EU scientists

James Meikle

Guardian ... Saturday 8 January 2000


Millions of European consumers may be at risk of catching Creutzfeldt-Jacob disease (CJD), the fatal human version of BSE - despite their governments' assertions that their countries are free of the cattle disease, the European Union's most senior scientists warned in a report yesterday.

Up to 400,000 people in some member states could be exposed to infected material from a single cow if it were allowed to enter the food chain because it had displayed no clinical signs of bovine spongiform encephalopathy (BSE).

The EU's scientific steering committee believes that Austria, Denmark, Finland, Germany, Greece and Sweden should introduce bans on the most infective parts of cattle, including brain, spinal material and intestines, while Italy and Spain should extend their measures to cover beef from all countries, not only from those known to have BSE.

Only seven countries, including Britain and France, at present operate such anti-BSE measures. In Britain all meat from cattle more than 30 months old is banned from being used in food. Even so, a handful of infected animals not showing "mad cow" symptoms are still thought to slip into food production .

The European commission has failed to introduce precautionary measures throughout the EU because some countries claim they have no BSE or that it has been evident only in imported cattle.

The new advice is likely to undermine European confidence in beef as the legal wrangle between the commission and Paris over the safety of British beef continues. But the committee, which cleared British beef for export after a 40-month ban, is worried by cross-border trade in live animals, organs, offal and processed foods. It says the risk of exposure to BSE "is not necessarily linked" to geographic incidence of the disease.

"Recent evidence suggests that in countries with a reported low incidence, the actual rate of BSE-infected animals entering the food chain is not nil," the report says. It says many people within the EU are eating potentially dangerous material contained in common meat products such as ptés and sausages.

Tests to identify BSE in cattle carcasses in its early stages do not offer reliable screening. But the removal of risky parts of animals significantly reduces the potential for infecting humans. The scientists conclude: "Failure to do this is likely to expose a large number of consumers to an unnecessary risk ."

Their bleak warnings give added significance to the reluctance of British scientists to predict the eventual size of the CJD outbreak , which has killed 48 people in Britain, two in France and one in Ireland so far. Those victims are believed to have become infected before most controls were introduced in 1989, though the first death did not occur until 1995.