Document Directory

28 Apr 01 - CJD - How BSE Went Mad
28 Apr 01 - CJD - Family Silenced As Vicky Lay Dying
28 Apr 01 - CJD - CJD turned 'perfect girl into a wreck'
28 Apr 01 - CJD - Inquest uncertainty over CJD death
28 Apr 01 - CJD - CJD victim, 20, had brain of 90-year-old
28 Apr 01 - CJD - Cattle develop BSE after injection with scrapie-infected material
27 Apr 01 - CJD - Victim of human variant of Mad Cow dies
27 Apr 01 - CJD - Third French CJD victim dies
27 Apr 01 - CJD - Third French victim of human BSE dies
27 Apr 01 - CJD - Human Mad Cow Disease Claims Oldest Victim
27 Apr 01 - CJD - Inquest probes girl's alleged CJD death
27 Apr 01 - CJD - Inquest into death of suspected CJD victim
27 Apr 01 - CJD - Morocco Denies Mad Cow Infection
27 Apr 01 - CJD - Tests show BSE caused by infected sheep
27 Apr 01 - CJD - New evidence for BSE scrapie link
27 Apr 01 - CJD - Scrapie gives cattle BSE-like disease
27 Apr 01 - CJD - Mad Cow disease puts a hole in sole business
25 Apr 01 - CJD - BSE-Style Threat To Pork
25 Apr 01 - CJD - Nobel laureate connects Mad Cow disease and Alzheimer's
25 Apr 01 - CJD - EU: Beef consumption still hit by BSE fears
25 Apr 01 - CJD - BSE, FMD confusion may erode consumer confidence
25 Apr 01 - CJD - Asia livestock output seen to rise with FMD and Mad Cow diseases
25 Apr 01 - CJD - Mad Cow disease theory challenged
25 Apr 01 - CJD - Conference confronts Mad Cow
25 Apr 01 - CJD - State of European research on BSE and related diseases
25 Apr 01 - CJD - Emu farmers gain from European meat scares
25 Apr 01 - CJD - EU ministers prolong ban on Meat and Bone Meal-based animal feed
25 Apr 01 - CJD - Scientists 'develop quick BSE test'
25 Apr 01 - CJD - Leather Furniture Makers Feel Pinch



28 Apr 01 - CJD - How BSE Went Mad

by LeRoy D. Olson, DVM, PhD

IndustryClick.com--Saturday 28 April 2001


A first-person chronicle of the development and mishandling of the BSE crisis.

The first event in Britain that led to the development of Bovine Spongiform Encephalopathy (BSE) in British cattle began around 1980. That's when the British government forced the heat-digestion processors of offal to discontinue the use of trichloroethylene as a fat solvent in the rendering process. The source of this offal was slaughtering plants and animals that had died of various causes - including sheep with scrapie.

The reason for the discontinuance was that trichlorethylene irritated the eyes and upper respiratory tract of the workers in the plants. Little effort was made to find another solvent, however, because the value of fat had dropped drastically due to a drop in demand by the soap industry.

Trichloroethylene had been used for many years in Britain to extract the fat from animal residues, which includes meat meal, meat scraps, tankage and bone meal. During this time, there hadn't been any problems with cattle developing the central nervous system changes characteristic of BSE.

20/20 Hindsight

In retrospect, it appears it was trichloroethylene that was destroying the prions, the acronym for the proteinaceious infectious particles of sheep scrapie. These prions were in the offal of slaughtered sheep and sheep that had died of scrapie.

(Incidentally, trichloroethylene was used to extract the oil from soybeans in the U.S. in the early 1950s. It was discontinued, however, because the residue that adhered to the meal destroyed the blood forming cells of the bone marrow in ruminants.

If sufficient heat were used to vaporize the remaining trichloroethylene, the result was the destruction of the soybean protein. With animal residues, sufficient heat was used in the processing to vaporize the remaining trichloroethylene.)

When trichloroethylene was removed, uneasy British regulatory officials suspected the compound could be destroying the scrapie prions since Britain had a relatively high number of sheep flocks with scrapie. They knew scrapie had been transmitted to mink being fed sheep offal infected with scrapie and that scrapie had been transmitted to other sheep by injections of infected brains.

They also knew that infected brains and spinal cords subjected to autoclaving, which is 250F for 15 minutes at 15 lbs. of pressure , remained infectious when injected into sheep and had produced the clinical signs and lesions of scrapie.

To compensate for the possible prion destruction by trichloroethylene and the heat resistance of the prions, the British regulatory officials decided to increase the temperature to which the animal residues were subjected in the processing plants. What happened was that the additional heat made the animal residues containing the prions of sheep scrapie more infectious. It probably did this by freeing the prions from the sheep tissues in the heat-digesting process and providing more available prions to the cattle.

After the first few cattle developed the clinical signs and lesions of scrapie in Britain in the late 1980s, but before the British or the continental European public was told of the seriousness, word was leaked to the processors of animal residue that the use of their product for cattle and sheep would be discontinued. To minimize economic loss, the processors immediately shipped and sold most the product they had on hand to continental Europe, namely France, Belgium, Netherlands and Germany, British veterinarians told me.

I was told by British veterinarians after this occurred that within 15 years these countries also would have BSE. It's a prophecy that has come true.

The British Problem Erupts

When the first cattle in Britain developed BSE, the regulatory officials knew they had a gigantic problem. Their policy was to keep it quiet , hoping it would go away.

Several veterinarians told me the ban on the sale of meat and bone meal derived from ruminant offal imposed in 1988 was only minimally enforced until 1996. This, they said, was due to the prevailing anti regulatory philosophy of Margaret Thatcher, the Prime Minister at that time.

In 1990, British veterinarians were in the U.S. hoping to find the disease. They had no luck. The only other country where they found the disease was in Saudi Arabia in several cows purchased previously from Britain.

The toll on the British livestock industry has been disastrous. Britain has some of the best beef cattle in the world, and many countries depended upon it as a source of breeding stock.

With the development of the human variant disease of Creutzfeldt-Jakob disease (CJD) in the mid-1990s, supposedly from the eating of meat from BSE-infected cattle, a new wall of secrecy has been established , various news organizations report.

The effects of BSE now extend to other countries. BSE has increased the concerns of Europeans and Asians toward genetically modified crops, and toward U.S. beef from cattle given hormones of plant origin to increase their rate of gain. It's also undermined the trust among EU member nations.

Scrapie Is Endemic

Scrapie has been around for centuries. It's endemic in the sheep of many countries, including Britain and the U.S.

The first case of scrapie on Earth probably was the result of a mutation. It appeared in high numbers in the U.S. after World War II when numerous sheep of the Suffolk breed were imported from Britain.

I remember in the 1950s while in veterinary school at the University of Minnesota when all of the pens on one side of the veterinary clinic were full of sheep with this disorder. They spent all of their time rubbing against the sides of the pen and bunting each other.

The name, scrapie comes from scraping their skin because it itches. At the time, most of the faculty thought that the cause was a genetic disorder. Then in the early 1960s, I saw at Purdue University that it could be transmitted to other sheep with infective brains. Also, that it could be transmitted with injections of infective brain subjected to autoclaving.

It was not until the mid-1990s, almost 10 years after the first case of BSE appeared in Britain, that a scrapie eradication program was begun in the U.S. One cannot get a state veterinarian to talk about the program because the policy is no publicity, but eradication is occurring. This is evidenced by the lawsuits filed by sheep breeders over the condemnation of their herds and what they consider insufficient indemnity payments.

The U.S. has never had a case of BSE and probably never will unless there is a spontaneous mutation in a single bovine with the formation of prions or a quirk with the transmission of sheep scrapie to cattle.

One favorable factor is that the U.S. has not had to rely on animal-derived supplements due to inexpensive sources of plant proteins. In Britain and Northern Europe, however, it's too cool to grow cotton and soybeans, and flax seed oil cannot complete with other plant oils in today's market. Thus, they have to import expensive soybean meal.

How is scrapie related to CJD, a spongiform encephalopathy in humans? The most significant statement I've heard about CJD was from C.J. Gibbs, a retired physician at the National Institute of Health in Washington, D.C., who has studied this disease extensively. He said on National Public Radio a few years ago that he has never seen a case in the U.S. that did not involve a person who raised roses .

Why? Because all rose growers use a lot of bone meal , which is high in phosphorus and calcium. Roses require phosphorus as a nutrient and calcium for neutralizing the acid in the soil. In addition, the prions of scrapie in sheep are not only located in the brains and spinal cord, but also in the viscera, lymph nodes and bone marrow.

I postulate that the rose growers he is talking about got CJD by inhaling the scrappie prions in the dust of bone meal when it was applied to the soil because:

- The bones and bone marrow of U.S. sheep with scrapie are mixed with the bones of other animals in the processing,

- Bone meal is subjected to high temperatures in the heat-digestion process, thus making the prions in the marrow highly available, and

- Bone meal is very dusty because it is finely ground.

Scrapie Transmission

Scrapie-infected ewes have been shown to transmit the disease to their lambs. Whether it is prenatally, through the ewe's milk or via licking is unknown. The same avenues of transmission are also suspected with BSE.

Trying to detect and identify scrapie or BSE prions in the laboratory is far more complicated than isolating or identifying infective viruses or bacteria. As a result, most studies have been in animals.

Iceland is the only country to eradicate scrapie. Yet, it was reported at a 1998 symposium held in Reykjavik, Iceland, that scrapie-free sheep introduced on pastures previously grazed by scrapie-affected sheep, but idle for several years, developed scrapie .

With the suspicion that the prions of scrapie are discharged by the mouth and the fact that they are extremely hardy, it's possible that elk or deer, which initially developed chronic wasting disease - another spongiform encephalopathy in the U.S., could have become infected with scrapie prions by grazing pastures simultaneously grazed by scrapie-infected sheep or by eating out of feed bunks or licking salt blocks simultaneously available to scrapie-infected sheep. This proposed transmission route is also possible for our cattle.

Stanley Prusiner, discoverer of the prion, once said that the incubation period for Kuru, a spongiform encephalopathy of humans in New Guinea and similar to scrapie, was up to 40 years. With the remote possibility of sheep scrapie being transmissible to our cattle, and with its long incubation period, why do we in the U.S. allow the many flocks of Suffolk and Suffolk-Southdown sheep with endemic scrapie to survive in a society where the longevity of life is ever increasing? Having prions in the environment, let alone in the food chain, poses a serious health problem .

Prions are an abnormally modified structural form of a normal protein in the neurons of the brain. They're located as fibrils or fine fibers in and around the neurons that result in the development of large vacuoles in the neuron and is diagnostic of scrapie and the other spongiform encephalopathies. When the neurons die, the result is the microscope holes in the brain.

Spongiform encephalopathy is a microscopic description of the many holes that develop in the brain as a result of the death of nerve cells; there are no gross or visible lesions.

Even though prions don't contain DNA or RNA, they apparently propagate themselves by contacting their normal metabolic counterparts in the neuron and reshape the conformation of the normal protein into what is a prion. This cycle continues, ever-increasing the number of prions, until the neuron dies.

In summary, the events in Britain which led to the development of BSE in cattle and its spread to continental Europe were:

- The discontinuance of the use of trichloroethylene as a fat solvent in the rendering process,

- Increasing the heat in the digestion of the animal residues after the withdrawal of trichloroethylene, and

- The clandestine shipment and sale of BSE-infected animal residues to other countries of Europe.

LeRoy D. Olson is a professor emeritus in the University of Missouri's Department of Veterinary Pathobiology in Columbia. His interest in the various spongiform encephalopathies dates to his veterinary education in the mid-1950s. Since that time, he has closely followed the BSE situation and over the past 12 years has discussed the BSE problem extensively with British veterinarians and researchers.


28 Apr 01 - CJD - Family Silenced As Vicky Lay Dying

Darren Devine

Total Wales--Saturday 28 April 2001


SENIOR medics urged the grandmother of a CJD victim not to go to the media about her illness and pleaded with her to "think of the economy and the EC".

Doctors feared that 20-year-old Vicky Rimmer, from Connah's Quay, had fallen victim to new variant Creutzfeldt-Jakob disease, which is linked to BSE in cattle.

They told her grandmother, Beryl Rimmer, who had brought Vicky up from birth, "We know you are too sensible to go to the media", and that only a handful of youngsters had died of the disease.

Ironically, Vicky Rimmer probably did not die of the human form of Mad Cow disease, an inquest at Flint was told yesterday.

Vicky, who was just 15 years old when she fell into a coma, probably died from the naturally-occurring sporadic CJD, which is not linked to Mad Cow disease.

(Mad Cow correspondent's note: Sporadic CJD is unheard of in someone this young. This would appear to be an attempt to "massage" the vCJD fatality figures)

The coroner for North Wales central, John Hughes, said officials at the time were mindful of the effects that stories about CJD could have on the economy but the comments made to Mrs Rimmer urging her to keep quiet about her granddaughter's illness were inappropriate and added to her suffering.

Mrs Rimmer told the doctor involved, "This is a child's life, for God's sake."

She told the inquest there appeared to be an organised attempt to keep the public in the dark over the disease .

Her own GP was warned by medics at the Walton Neurological Centre in Liverpool not to speak to her about Vicky's illness , she told the inquest.

But she was undeterred and her fight for the truth was instrumental in persuading the Government to set up a full public inquiry into links between CJD and BSE.

Vicky died in 1997 after four and a half years in a coma.

When she died at the age of 20 her brain resembled that of a 90-year-old who had undergone severe neurological damage.

Scientists who investigated her death described her case as the most unusual and difficult they had come across.

Sporadic CJD has not been linked to BSE-infected cattle and normally affects people in their sixties.

Vicky was 15 when she first showed symptoms of CJD in March

1993.

Her family claimed she died as a result of variant CJD, which has been linked to eating meat from BSE-infected cattle.

The inquest heard that she was transformed from the "perfect teenage daughter" into a moody and depressed wreck.

She was admitted to Wrexham Maelor Hospital in August 1993 and later had tests at the Walton Neurological Centre in Liverpool.

She fell into a coma weeks later and never regained consciousness, dying on November 21, 1997.

Professor James Ironside, from the National CJD Surveillance Unit, said Miss Rimmer's case was unique in that her clinical and pathological tests showed signs of both sporadic CJD and new variant CJD.

"Our understanding of the case is not complete," he said.

"It is one of the most unusual and difficult cases I have ever come across.

"The characteristics of the disease suffered by Miss Rimmer do not fall neatly into any category.

"The investigations that we have performed so far would indicate that this case, unique as it is, has more similarities to sporadic CJD than to new variant."

Professor Ironside also said that after Miss Rimmer's death her brain was seen to be so damaged as to look like that of a 90-year-old who had undergone severe neurological damage.

Mr Hughes concluded that Miss Rimmer died of bronchial pneumonia caused by CJD and recorded a verdict of death by natural causes..

He said, "I ask you to recognise that I take this step simply on the weight of evidence before the court.

"New knowledge can confound us all."

He made a personal pledge that if new evidence came to light he would lobby the Home Secretary to have the inquest reopened.


28 Apr 01 - CJD - CJD turned 'perfect girl into a wreck'

Staff Reporter

Telegraph--Saturday 28 April 2001


The brain of a 20-year-old CJD victim resembled that of a "90-year-old who had undergone severe neurological damage", an inquest heard yesterday. Scientists who investigated Vicky Rimmer's death described her case as the most unusual and difficult they had come across. Miss Rimmer, who loved sports, died in November 1997 after spending four and a half years in a coma.

Miss Rimmer, from Buckley, North Wales, was 15 when she first showed symptoms of CJD in March 1993. Her family claimed the "bubbly, sports-mad, animal lover" died as a result of variant CJD, which has been linked to eating meat from BSE-infected cattle.

But John Hughes, the coroner, recorded a verdict of natural causes, saying the evidence was not clear cut but tended to suggest she died from sporadic CJD. Sporadic CJD has not been linked to BSE-infected cattle and normally affects people in their sixties. The inquest at the North East Wales coroner's court in Flint was told Miss Rimmer was transformed from the "perfect teenage daughter" into a moody and depressed wreck.

Her grandmother and legal guardian, Beryl Rimmer, 61, of Heswall, Merseyside, said: "She did every sport available - ice skating, horse riding, karate, swimming, ballet dancing. She excelled in all of them and was full of health. She was a bubbly girl and always happy. She was the perfect daughter.

"Her health was excellent until March 1993. She was always tall and slim but ate everything in sight, but she started losing weight and started to look anorexic. She started falling, like you see cows with BSE staggering on television."

Prof James Ironside, from the National CJD Surveillance Unit, said Miss Rimmer's case was unique in that tests showed signs of both vCJD and new variant CJD, but had "more similarities to sporadic CJD". He told the inquest that after her death, Miss Rimmer's brain was so damaged it looked like that "of a 90-year-old who had undergone severe neurological damage".


28 Apr 01 - CJD - Inquest uncertainty over CJD death

Staff Reporter

BBC--Saturday 28 April 2001


Relatives have expressed disappointment after a coroner ruled that a girl was likely to have died of a form of CJD unrelated to BSE-infected cattle.

Following an inquest into the death of 20-year-old Vicky Rimmer from Deeside - who had lain in a coma for fOur Worldand-a-half years - Coroner John Hughes said the evidence was unclear but suggested she died from sporadic CJD.

Beryl Rimmer said her treatment was 'unbelievable'

Sporadic CJD has not been linked to BSE-infected cattle and normally affects people in their sixties.

Grandmother Beryl Rimmer, 61, who had looked Vicky since she was a small child, had been pressing for a verdict of new variant CJD which is related to cattle.

She told North East Wales Coroner's Court in Flint how she first noticed symptoms in her granddaughter - believed to be the youngest person in Wales to have died of the disease - when she was 16.

From that point on, she said turned from the "perfect teenage daughter" into a moody and depressed wreck.

"Vicky was always full of life, she was sports mad and animal mad," she said.

'Staggering'

"Her health was excellent until March 1993. She was always tall and slim but ate everything in sight, but she started losing weight and started to look anorexic.

"She started falling, like you see cows with BSE staggering on television...

"She couldn't understand and said "What's happening to me mum?'

According to Professor James Ironside of the National CJD Surveillance Unit, Miss Rimmer's case was "unique" and tests showed signs of both vCJD and new variant CJD.

"Our understanding of the case is not complete. It is one of the most unusual and difficult cases I have ever come across," he explained.

"The characteristics of the disease suffered by Miss Rimmer do not fall neatly into any category.

"The investigations that we have performed so far would indicate that this case, unique as it is, has more similarities to sporadic CJD than to new variant."

Neurological damage

Miss Rimmer was initially admitted to Wrexham Maelor Hospital in August 1993.

Shortly after being moved to the Walton Neurological Centre in Liverpool, she fell into a coma. Never regaining consciousness, she died in November 1997.

At the time of her death, the inquest was told that her brain was so damaged as to look like that "of a 90-year-old who had undergone severe neurological damage".

After tests had been carried out, Mrs Rimmer told the coroner, a doctor had told her Vicky had spongiform encephalopathies.

Not sure what it meant, she told Vicky's GP who said: "That's Mad Cow disease."

When she pressed doctors at the hospital, she said she was told not to go to the press as it "could damage the economy".

"I think the way I was treated was unbelievable," Mrs Rimmer said.

Coroner John Hughes explained the reason for the four year delay to the inquest was because Beryl Rimmer wanted both the results of an inquiry into BSE and medical tests into CJD to be made public before any hearing.

Mr Hughes returned a verdict of death by natural causes and concluded that Miss Rimmer died of bronchial pneumonia caused by CJD.

"I ask you to recognise that I take this step simply on the weight of evidence before the court," he said.

He went on to say that "new knowledge can confound us all" and said if new evidence came to light he would petition the Home Secretary to have the inquest reopened.


28 Apr 01 - CJD - CJD victim, 20, had brain of 90-year-old



Scotsman--Saturday 28 April 2001


The brain of a 20-year-old CJD victim resembled that of a "90-year-old who had undergone severe neurological damage", an inquest heard yesterday.

Scientists who investigated Vicky Rimmer's death described her case as the most unusual and difficult they had come across. Sports-mad Miss Rimmer died in November 1997 after spending fOur Worldand-a-half years in a coma.

Coroner John Hughes yesterday returned a verdict of natural causes, saying the evidence was not clear-cut but tended to suggest she died from sporadic CJD.

Miss Rimmer, from Buckley, North Wales, was 15 when she first showed symptoms in March 1993.

Her family claimed she died as a result of variant CJD, which has been linked to eating meat from BSE-infected cattle.

The inquest at the North East Wales Coroner's Court in Flint heard she was transformed from the "perfect teenage daughter" into a depressed wreck.


28 Apr 01 - CJD - Cattle develop BSE after injection with scrapie-infected material

Editorial Team

Just Food--Saturday 28 April 2001


Government scientists have shown that it is possible to infect cattle with a BSE-like disease through injecting scrapie-infected material from sheep. This may bring scientists closer to identifying the cause of the BSE epidemic in Britain.

Scientists from the veterinary laboratories agency injected the scrapie-infected material into two cows eighteen months ago, and they have now developed symptoms regularly associated with BSE. The cows have been put down, and what is important now is for the scientists to determine whether the disease they contracted behaves similarly to BSE or scrapie, and whether there may have been consequences for human health if the cattle had entered the food chain.

Scrapie is an animal disease that has long been thought harmless in humans. The farming industry is now concerned that if scientists prove the cattle disease is caused by scrapie and harmful to humans, then the blow would prove disastrous for sheep farmers. It is extremely difficult to remove all parts of a sheep that might be infected with scrapie.

Scientists have cautioned that the results and significance of these experiments may not become official for another five years, a fact that worried officials from the Food Standards Agency, whose job it is to review food safety controls. In the meantime however, scientists have insisted on the possibility that the disease exhibited by the cattle may be the result of neither BSE nor scrapie.


27 Apr 01 - CJD - Victim of human variant of Mad Cow dies

Associated Press

Star--Friday 27 April 2001


PARIS (AP) - A French teenager believed to have been suffering from the human variant of mad-cow disease has died after slowly losing the ability to walk, speak and breathe.

Arnaud Eboli, 19, died Wednesday after fighting the brain wasting ailment for more than two years, according to the Association of Victims of Creutzfeldt-Jakob disease.

His death marks France's third fatality from variant Creutzfeldt-Jakob disease, which is linked to the consumption of tainted beef. In Britain, where mad-cow disease was identified in 1995, 90 people have died of the disease.

Eboli, once an athlete who excelled at skiing and martial arts, lost the ability to bathe or feed himself. Before he died, he was paralysed and kept alive through a feeding tube.

Doctors diagnosed Eboli in December 1999 after a biopsy of his tonsils detected traces of an infectious protein, prion, often found in people suffering from the disease. The disease can only be confirmed by a brain biopsy, usually after death.

Eboli's family was one of two French families that filed a lawsuit in November charging that French, British and European Union authorities did not act quickly enough to wipe out mad-cow disease or Bovine Spongiform Encephalopathy (BSE).

The suit alleges that Eboli and Laurence Duhamel, who died in 1999 at age 36, were victims of poisoning and manslaughter.

France has taken drastic measures - such as outlawing certain at-risk cuts of beef, like the T-bone steak - to try to safeguard public health.

New cases of the animal ailment are expected to break out in France until 2002 - five years after agriculture authorities took rigorous measures to prevent more outbreaks. About 150 cows were discovered with the disease in France last year, compared to 31 the year before.


27 Apr 01 - CJD - Third French CJD victim dies

Staff Reporter

BBC--Friday 27 April 2001


A 19-year-old man has become the third victim in France to die of the human form of Mad Cow disease.

Arnaud Eboli died on Wednesday from variant Creutzfeldt-Jakob disease (vCJD), the human type of BSE, his family have announced.

Last year, a lawyer acting for Mr Eboli and Laurence Duahamel - another victim of the disease - sued the French, British and EU authorities alleging they had failed to take all the necessary steps to contain the epidemic.

The law suits - filed in a Paris civil court - accuse Britain of knowingly exporting possibly contaminated material, and France and the European Commission of not taking the threat of disease seriously enough.

Britain, where the outbreak of BSE has been most severe, has so far confirmed 97 deaths from vCJD and the Republic of Ireland one.

Feed investigation

The news of Mr Eboli's death comes a day after French magistrates placed under official investigation a firm allegedly distributing contaminated feed.

Youssef Chataoui, head of the French company Euro Feed, is accused of illegal involvement in trafficking meat-based animal feed.

Feed products were allegedly brought from France, Ireland and the Netherlands to Belgium, where they were relabelled.

France banned bone meal from animal feed in 1990 and further tightened its controls in 1996.

"This discovery, if confirmed, could explain the scientific mystery of how certain cows born after the ban on animal derivatives in their feed could develop Mad Cow disease," the Le Parisien newspaper commented.


27 Apr 01 - CJD - Third French victim of human BSE dies

Reuters

Environmental News Network--Friday 27 April 2001


France's third suspected victim of the human form of Mad Cow disease has died at age 20, the man's family said Thursday.

"He died in appalling conditions. He looked like an old man," the mother of Arnaud Eboli told Reuters.

Eboli, who died late Tuesday, had been ill for two years and was identified last autumn as France's latest suspected victim of the fatal brain wasting new variant Creutzfeldt-Jakob disease (vCJD).

Scientists believe the disease can be passed from cattle to humans who eat beef infected with Mad Cow disease, or Bovine Spongiform Encephalopathy (BSE).

Nearly 100 people have died in Europe from the disease - about 90 in Britain, three in France and one in Ireland - and there are several more suspected or confirmed sufferers.

Eboli's body was transferred to the Pitie-Salpetriere hospital in Paris for post-mortem tests to confirm if he was suffering from vCJD.

Families of French victims of the disease, including the Ebolis, filed suit last November "against persons unknown" on poisoning and manslaughter charges.

The Eboli family has also campaigned for health authorities to provide guidance and assistance to families struggling to come to grips with the little-understood illness.

"We insisted on keeping him at home, because this disease is too atrocious. We held his hand until the end," said Eboli's mother, Dominique. "We knew he was going to die, but today it's very hard. It's emptiness."


27 Apr 01 - CJD - Human Mad Cow Disease Claims Oldest Victim

Reuters

YAHOO--Friday 27 April 2001


LONDON (Reuters) - More elderly people will fall victim to the human form of Mad Cow disease, medical experts said on Friday after a 74-year-old man became the oldest victim of the fatal brain affliction.

Variant Creutzfeldt-Jakob disease (vCJD) was diagnosed after an autopsy was requested for the 74-year-old man on the basis that some of his symptoms were not associated with dementia and he died just seven months after they began.

Professor James Ironside of the CJD Surveillance Unit at Western General Hospital in Edinburgh said the death of the unidentified man is unlikely to be an isolated event, saying more cases of the disease could occur in people in their 50s, 60s and 70s.

Most of the 95 cases of the brain wasting illness reported in Britain have been in people decades younger than the oldest victim.

``This case has important implications for the surveillance of vCJD, and raises the possibility that cases of vCJD in the elderly might be missed ,'' they said in a letter to The Lancet medical journal.

Symptoms of the illness, which include loss of co-ordination, confusion and personality changes, can be mistaken for dementia in older people. Cases of vCJD are usually confirmed by an autopsy, which is not commonly performed on the elderly.

Doctors ``should be aware that vCJD can arise in elderly patients so that appropriate investigations are done,'' the experts added.

Doctors should request scans and autopsies in suspected cases of vCJD in the elderly.

The elderly man, a retired electrician, had no family history of brain disease and was healthy until he complained of pains in his hands and then became forgetful and started having hallucinations and paranoid delusions.

But the scientists said he ate meat pies and sausages at least once every week and pate every month. Researchers suspect humans get vCJD, which was first identified in 1996, by eating meat contaminated with Mad Cow disease or Bovine Spongiform Encephalopathy (BSE).

Last month an inquiry into a cluster of vCJD deaths in central England said local butchering practices were the most likely cause of the vCJD cases.

Because of its long incubation period, which can be up to 30 years, scientists say it is impossible to predict how many people will be struck down by the disease. Estimates range from thousands to tens of thousands over the coming years.


27 Apr 01 - CJD - Inquest probes girl's alleged CJD death

Staff Reporter

ITN--Friday 27 April 2001


"Vicky was always full of life, she was sports mad and animal mad" - Beryl Rimmer, Vicky's grandmother

An inquest heard today of the death of a suspected CJD victim who was described as becoming a moody and depressed wreck.

Vicky Rimmer died at the age of 20, in November 1997, after spending four and a half years in a coma.

Her physical and mental health had deteriorated in the months before she slipped into the coma, the hearing, at the North East Wales Coroner's Court, Flint, heard.

Miss Rimmer's grandmother, Beryl Rimmer, who was her effective legal guardian, said: "Vicky was always full of life, she was sports mad and animal mad. "She did every sport available - ice skating, horse riding, karate, swimming, ballet dancing. She excelled in all of them and was full of health.

"She was a bubbly girl and always happy. She was the perfect daughter.

"Her health was excellent until March 1993. She was always tall and slim but ate everything in sight, but she started losing weight and started to look anorexic.

"She started falling, like you see cows with BSE staggering on television. She would go to feed herself and as she put the food to her mouth it went all over her clothes.

"She couldn't understand and said `What's happening to me mum?'

"She used to come home from school and go straight to bed. That wasn't like her because she had always been full of life," Mrs Rimmer, of Heswall, Merseyside, told Coroner John Hughes.

The teenager would also go off in a daze and complain to her friends that her mind had gone blank.

"Vicky was like someone I didn't know, it wasn't her. She was either really high and singing or really down and crying," Mrs Rimmer said.

"She never told me this, but her friends said sometimes she would be lying down listening to music when she would put her hands to her head and say `Oh, my God, there goes my head again, all black'.

"I thought she might be on drugs and I would yell at her to ask what drugs she was taking."

Miss Rimmer was admitted to Wrexham Maelor Hospital in August 1993, and later to the Walton Neurological Centre in Liverpool.

She fell into a coma weeks later, and never regained consciousness, dying on November 21, 1997. The inquest continues.


27 Apr 01 - CJD - Inquest into death of suspected CJD victim

Ananova

PA News--Friday 27 April 2001


An inquest is being held into the death of a suspected victim of Creutzfeldt-Jakob's disease who died more than three years ago after falling into a coma.

Vicky Rimmer, of Connah's Quay, north Wales, was thought to be the first teenager diagnosed as suffering from suspected CJD.

The former part-time kennel worker died at the age of 20 in Deeside Community Hospital some four years after falling into a deep coma.

Ms Rimmer, said to be a "keen meat eater", originally fell ill when she was 15-year-old and suffered from static pneumonia for the six months prior to her death.

She was a bright, happy-go-lucky teenager until she fell ill with what experts believed to be the human form of Mad Cow disease. Her relatives always blamed hamburgers for her debilitating illness.

Speaking at the time of her death, Vicky's grandmother Beryl Rimmer, of Heswall, Wirral, Merseyside, said she hoped others would benefit from the family's tragedy.

"I am determined to find out what was wrong with her and how she got CJD, she had never been ill in her life, never even had a cold," she said.

The inquest is being heard at the magistrates court in Flint before North East Wales coroner John Hughes.


27 Apr 01 - CJD - Morocco Denies Mad Cow Infection

Panafrican News Agency (Dakar)

All Africa--Friday 27 April 2001


Rabat, Morocco: Moroccan Agriculture minister Ismail Alaoui has affirmed in Parliament that no Mad Cow disease has been detected in Morocco to date.

The minister said the absence of Bovine Spongiform Encephalopathy (BSE or Mad Cow Disease) in Morocco is due to the non-use of meat and bone meal in cattle feed.

He said other preventive measures have been taken by the government since the early 1990s to avoid BSE contamination of cattle.

Alaoui said that about 60 per cent of Morocco's bovines have been vaccinated against BSE.

He expressed regrets that 20 million sheep and goats were not immunised due to financial reasons.

Last March, a high ranking official at the Agriculture ministry said the immunisation campaign was completed and that regular surveys carried out indicated the absence of the BSE virus from all sensitive species including bovines and sheep.

On 15 March, Rabat banned the importation of livestock and meat from countries affected by the foot-an-mouth disease, and requested importers of cereals to obtain health certification before doing so.

However, the importation of milk and dairy products is not subject to prior authorisation.


27 Apr 01 - CJD - Tests show BSE caused by infected sheep

By Steve Connor, Science Editor

Independent--Friday 27 April 2001


Government scientists have found direct evidence to support the theory that the BSE epidemic was originally caused by feeding cattle the rendered carcasses of sheep infected with scrapie.

The theory was dismissed last year as "fallacious " by the BSE inquiry, but preliminary results of an experiment to test it have revealed that cows injected with sheep scrapie fall ill with what appears to be Bovine Spongiform Encephalopathy. Results of the experiment were passed this week to the Government's spongiform enceph-alopathy advisory committee (Seac), but further work is needed before the evidence can be considered conclusive.

Knowing the precise origin of BSE is important because it would reassure scientists that the measures introduced to prevent a recurrence are watertight. The experiment involved either feeding or injecting scrapie into calves to see whether they were susceptible to the disease. Two calves have so far succumbed to BSE-type symptoms. Their brains are the subject of post-mortem tests.

Scientists from the Veterinary Laboratories Agency near Weybridge in Surrey began their investigation after criticism by Seac in 1996 that research into the origins of BSE was sorely lacking. Ten calves have been injected intracerebrally with material from the brains of sheep that died of scrapie before 1975, when it was thought there would be no risk of the sheep being cross-contaminated with BSE.

Another 10 calves were similarly injected with scrapie material from sheep that died after 1990. Since the experiment was started in July 1999, one calf in each group has succumbed to BSE-like symptoms.

Another experiment, started last September, has involved feeding scrapie material to separate groups of calves to see if the sheep disease can be transmitted orally to cattle, as the "scrapie hypothesis" predicts. Danny Matthews, the head of molecular biology at the Veterinary Laboratories Agency, said that even if the cattle fed on scrapie developed similar symptoms to cattle with BSE, it would take many years of further tests on mice before that could be confirmed.

The BSE inquiry dismissed the scrapie hypothesis largely because many other countries had also rendered sheep carcasses into cattle feed and had not suffered a BSE epidemic.

The inquiry suggested a spontaneous mutation in a gene for the prion protein implicated in BSE and scrapie. The inquiry concluded: "BSE probably originated from a novel source early in the 1970s, possibly a cow or other animal that developed the disease as a consequence of a gene mutation."

However, Dr Matthews criticised the inquiry's dismissal of the scrapie hypothesis. He said: "It's crazy that they should have come up with that conclusion... I think the evidence for spontaneity is pretty thin."

Other research into the origins of BSE is being done by Professor Gabriel Horn, of Cambridge University, who has been asked by the Department of Health and Ministry of Agriculture to investigate the idea that the epidemic was caused by the rendering of a zoo animal carrying an endemic form of the brain disease.

Professor Horn said: "It is important to know the origins of BSE because if we get some real insight that turns out to be surprising it might alert us to a way we might act in the future to prevent such a calamity."


27 Apr 01 - CJD - New evidence for BSE scrapie link

Reuters

YAHOO--Friday 27 April 2001


New research into the cause of BSE shows that its origin may lie in cattle feeds derived form the carcasses of sheep that had suffered from scrapie.

Although the theory has been previously dismissed, preliminary experiments have shown that cattle injected with scrapie show symptoms of BSE. The research results have been passed on to the governments Spongiform Encephalopathy Advisory Committee, SEAC, with more work required to confirm the link.


27 Apr 01 - CJD - Scrapie gives cattle BSE-like disease

James Meikle, health correspondent

Guardian--Friday 27 April 2001


Government scientists have infected cattle with another BSE-like disease using scrapie from sheep in an experiment that raises new questions about the origins of BSE and whether food safety measures are satisfactory.

Two cows developed the symptoms 18 months after injections into their brains of material from sheep brains infected with scrapie, a disease long considered harmless to humans. But it might be five years before the experiment's significance can be assessed.

If scrapie was considered a danger after all, there would shock among the political, scientific and medical establishments. British farming could be dealt another blow, since it would be far harder to remove all potentially infective parts of sheep from food.

There may, however, be no significance for human health.

The two animals put down after showing clinical signs of illness will be tested to see whether the disease behaves similarly or differently from BSE or scrapie.

The experiment is being carried out by the veterinary laboratories agency. Officials were insisting last night that the new disease might be neither scrapie nor BSE.

One official, Danny Matthews, said it was likely that the results "of this one study will not be interpretable until we have the results of other studies that are under way".

The experiment, the first in Britain, started in July 1999, 13 years after BSE was identified in cattle and three years after the admission that people were probably dying because they had eaten infected parts of cows.

Any "scrapie in cattle" would not be as worrying as BSE in sheep, which has been caused in laboratories. There is no evidence that this has happened naturally, but the government has contingency plans, including widespread sheep culls.

The warning of delays caused consternation at the food standards agency, which reviews all controls against BSE-like diseases when there is emerging scientific evidence. A spokesman said scientists "have said to our people that this result is uninterpretable in scientific and policy terms. We simply cannot scrutinise things that are uninterpretable."

Until recently the idea that scrapie from sheep remains in cattle feed had turned into the more deadly BSE was the frontrunner among explanations for BSE.


27 Apr 01 - CJD - Mad Cow disease puts a hole in sole business

By Asante Green

The Advocate--Friday 27 April 2001


Don't try to tell Izet Music that Mad Cow disease hasn't hit the United States.

With the disease forcing the slaughter of thousands of head of cattle, supplies of leather are dwindling and becoming more expensive. It is a cost that Music, owner of Izet's Leather and Shoe Repair, has no choice but to pass on to his customers.

"I am not passing the cost of labor on to my customers, but the cost of the leather will go up," Music said. "Most of my prices will remain comparable to others. The only difference is, I use a higher quality of leather than they do."

Bovine Spongiform Encephalopathy, also known as Mad Cow disease, is a lethal central nervous system disease affecting cattle. It is characterized by the appearance in neurons in the brain of affected cattle of clear holes that give the brain the appearance of a sponge.

"Because of Mad Cow disease, manufacturers are paying more for the hide leather," said Lewis Turco, manager of National Leather, which sells leather to Izet's. "In Europe, they are burning all of the cows and not saving the hides, creating a shortage for us. The decline in leather has lasted two months, prices have gone up 15 percent, and in some places 30 percent."

Music said a shipment of leather has been on back order for several weeks. When it comes in, he expects to pay up to 40 percent more than usual.

Music, grew up in Yugoslavia watching his uncle make leather shoes and repair other leather products such as belts, pocketbooks and jackets. Over time, his uncle allowed him be his apprentice.

"I fell in love with it. Watching him I thought it was an art," Music said. "I saw the challenge and wanted to face it."

But he wouldn't accept the challenge until years later.

The soles of his own leather shoes wore thin while working in a Manhattan restaurant. After searching for a cobbler to repair his shoes, he decided he could do the repairs himself with the skills he'd learned from his uncle.

In 1986, Music, his wife and their son visited Stamford to get a passport. Music said he fell in love with the city because of its growing economy - a perfect place for his new venture.

That year, he opened a leather and shoe repair shop on Atlantic Street, next to Atlantic Pizza but later moved to 60 Atlantic St. where he has remained.

"When you do work with your hands, you can't get rich," Music said. "You can make a living, and I am happy with that."

Music repairs leather hats, books, jackets, handbags and more. He also makes handbags and leather belts on special order.

"I love the finished product of leather, it is natural and it has it's own beauty. People love natural things and I think that is the reason they come in to have it restored," Music said.


25 Apr 01 - CJD - BSE-Style Threat To Pork

Staff Reporter

Sky--Wednesday 25 April 2001


People who eat pork could be at risk of brain disease because the Government ignored warnings from its own safety experts about a possible BSE-style infection.

Downplayed

Sky News has discovered that a major BSE-type epidemic could have been unleashed by feeding pigs swill mixed with pork.

The Government was warned three years ago by its BSE advisory committee that the disease "would be difficult to detect" and recommended that the risk be removed "at the earliest opportunity". But it downplayed the findings and did not act on the advice.

The advisory committee was also unaware of the extent of the practice. Up to 100,000 pigs would have eaten 500,000 tonnes of the swill a year. Pig swill has been linked to the outbreak of foot and mouth and will be banned in the next few days.

'Outrageous'

Cannibalistic feeding practices - feeding animals the remains of their own species - was behind the BSE outbreak. Boiling the food would not be enough to get rid of any BSE-type organism.

Now shoppers will be wanting to know why the Government did not act more quickly to inform people of possible dangers.

Mona Patel, of the Consumers' Association, said: "It is outrageous that the Government hasn't taken action. Steps must be taken immediately to make sure it isn't ignoring other advice that may have an impact on public health."


25 Apr 01 - CJD - Nobel laureate connects Mad Cow disease and Alzheimer's

C.P. Kaiser

Diagnosic Imaging.com---Wednesday 25 April 2001


Rogue proteins -- or prions -- responsible for Mad Cow disease are also implicated in other more common neurodegenerative diseases such as Alzheimer's and Parkinson's, according to Nobel laureate Dr. Stanley B. Prusiner.

Degenerative diseases of the central nervous system are disorders of faulty protein processes, which include misfolding, abnormal modification, and aberrant cleavage, Prusiner said in his keynote address Sunday at the American Society of Neuroradiology meeting in Boston.

He has demonstrated that aberrant proteins are deposited in the central nervous system in diseases such as Mad Cow (Bovine Spongiform Encephalopathy), Alzheimer's, and Parkinson's.

"It is the accumulation of these misprocessed proteins that causes the nervous system to malfunction, resulting in problems such as dementia," said Prusiner, a professor of neurology at the University of California, San Francisco.

Prion is an entirely new class of infectious pathogen, inherited disorder, and sporadic disease. The known prion diseases, all fatal, are sometimes referred to as spongiform encephalopathies, so named because they frequently cause the brain to become riddled with holes. They include BSE and Creutzfeldt-Jakob disease.

The conditions can brew for years, or even decades in humans, and are widespread in animals. The most common form is scrapie, a loss of coordination and development of intense itch found in sheep and goats.

Prusiner believes that Alzheimer's and Parkinson's may have an infectious strain, as do known prion diseases. All these disorders have some marked similarities:

- They occur sporadically but sometimes run in families.

- They are marked by similar pathology: neurons degenerate, protein deposits can accumulate as plaques, and glial cells (which support and nourish nerve cells) grow larger in reaction to damage to neurons.

- "If we knew how to find it, there would be an infectious form of Alzheimer's and Parkinson's that we could demonstrate," he said.

Although still controversial, evidence Prusiner presented from transgenic mouse studies suggests that people dying of a new variant of Creutzfeldt-Jakob disease (nvCJD) contracted the disease from cows. He uses the term industrial cannibalism to describe the rise of BSE in cattle.

Prusiner's theory is that in the 1970s, the meat and bonemeal produced from rendered offal of sheep, cattle, and pigs underwent a dramatic change in the heating process, which effectively destroyed the sheep scrapie prions (not infectious to humans) and allowed the BSE prions to remain. BSE prions then multiplied and were fed into other cattle until they became pathogenic for humans.

"It is unreasonable to knowingly expose any person to meat or meat products from prion-infected livestock, and it tends to occur more often than we like to think," he said.

Although more research needs to be done to identify other prion-related neurodegenerative diseases and to determine how the mechanisms work in the different strains, Prusiner believes imaging will have a huge role in these diseases, not only for diagnosis, but in treatment as well.

For example, researchers have added antibodies to cultured cells infected with prions, which not only stopped their production, but also cleared out the remaining prions.

"I can imagine in humans we'll come to a point that a therapy stops the production of prions. We'll see the brain clearing the prions and many of the cells returning to normal function. We should see very dramatic differences on imaging," he said.


25 Apr 01 - CJD - EU: Beef consumption still hit by BSE fears

Staff Reporter

Just Food--Wednesday 25 April 2001


Fears over Mad Cow disease continue to hold back sales of beef despite a slight recovery in consumption figures for the meat in the last month.

The European Commission said today (Tuesday) that beef consumption was now down by an average of 18% across the bloc compared with a decrease of 23% in March. The rise in consumption might be temporary however because of the Easter holidays.

Germany is suffering the most from the Mad Cows crisis with beef sales still down by some 40% compared with levels before the latest crisis. Consumption was 30% lower in Italy and 20% down in Spain, France and Portugal.

The EU remains unable to export most of its beef as its trading partners have closed their borders to EU meat as a result of the BSE and foot and mouth crisis. The Commission said the EU had lost some 94% of its beef exports, 73% of its pork exports and 31% of its poultry exports.

The Commission was hopeful that the Russian and Japanese governments would soon lift the embargoes put in place after the recent food crises. Veterinary experts from those countries had been inspecting individual member states to assess the possibility of relaxing their bans.


25 Apr 01 - CJD - BSE, FMD confusion may erode consumer confidence

By Lisa Foust Prater, Commerce Editor

Agriculture News--Wednesday 25 April 2001


American consumer misconceptions about Bovine Spongiform Encephalopathy (BSE), or Mad Cow disease, and foot-and-mouth disease (FMD) may be beginning to impact consumer confidence and affect purchasing decisions, according to a survey just released by public relations firm Porter Novelli.

Dan Snyder, director of Porter Novelli Washington's Food, Beverage and Nutrition Practice, told @griculture Online there weren't any big surprises in the survey. "The results came out pretty much as we had expected," he says.

The survey was conducted among primary food shoppers throughout the country, and 14% of participants said they have already changed their food purchase or eating habits based on reports of BSE and FMD.

Difficulty distinguishing between BSE, FMD

Despite ongoing media coverage of the diseases, the survey found that many consumers are confused. In fact, 19% of those surveyed incorrectly thought that BSE and FMD were the same, 27% thought there was a direct link between the two, and 46% thought that cows with FMD would infect humans.

The shoppers also said they would respond similarly if either of the diseases were found in the U.S., despite the fact that FMD can't infect humans. If FMD were to occur in this country, 71% of those surveyed said they would eliminate or reduce ground beef from their diet, and 80% said they would do the same in the case of BSE. Also, approximately half of the respondents said they would eliminate or reduce their consumption of other animal products such as other meat, chicken, milk and cheese if BSE or FMD were to occur here.

Consumer education needed from a trusted source

Snyder says that in addition to the fight to prevent BSE and FMD from entering our food supply, we need a national consumer education campaign to help clear up the confusion. "We devote a lot of energy to controlling the spread of BSE and foot-and-mouth, but little to control the psychological impact on consumers," he told @griculture Online. "If we don't address that psychological impact, the industry will be hurt."

When it comes to consumer education, the Porter Novelli survey shows the U.S. government may be best suited for the role of educator, since respondents most trust agencies such as USDA, FDA and CDC to assure them that they and their family could not be infected by eating animal products.

This surprised Snyder, who says doctors -- the second most trusted group -- are usually listed as the most trusted source of medical and safety information.

"I think that's a message to agencies like the USDA, FDA and CDC that maybe they should step up," Snyder says. However, he says it may be difficult for government agencies to address the situation without causing consumers undue concern. He says, "At what point do you get in front of it and not enflame it?"


25 Apr 01 - CJD - Asia livestock output seen to rise with FMD and Mad Cow diseases

By Melody M. Aguiba

Manila Bulletin--Wednesday 25 April 2001


The outbreak of madcow and of foot and mouth disease (FMD) in Europe will bring about double growth in livestock production in Asia and give many opportunities not only for big livestock raisers but also for small farmers.

Hank Fitzhugh, director general of the International Livestock Research Institute (ILRI), said during the Asian Agriculture Congress at the Westin Philippine Plaza that the reduced supply of beef and pork in the world market will intensify trade even among Asian countries. He said however that Asian countries must be warned of the transfer of disease in livestock among each other as UK's FMD itself must have come from Asia.

"It will create opportunity for small farmers to generate more income. My expectations is that this will bring in more meat from within Asia as well as for regional trade and be able to manage increases in meat production in this region. There will be much more than doubling of production in meat and milk in Asia, not imports but production in Asia by the year 2020. The Philippines is definitely included. The situation (outbreak of FMD) in UK is usual because of its very strong Asia connection. The strain of foot and mouth disease that came to UK originally came from Asia, and was probably brought in through import from Asia so we also have to be concerned about what's happening not only in our country but in other countries," said Fitzhugh.

Among the countries that will particularly benefit from reduced meat supply from the UK are India which is supporting buffalo meat, Thailand, Malaysia, and Vietnam. In the Philippines, small livestock raisers should take advantage too of the opportunity. "By controlling diseaseses and by better feeding, increases in the volume of these producing countries Thailand, Malaysia, Vietnam will increase the amount available for export. India is one of the countries in Asia that is exporting. Of course you have a major neighbor to the south, Australia, which is looking to this very closely. This should open up real opportunities here in the Philippines not only for much of the beef here is produced from relatively large farmers and producers, but there is really opportunity here for the small scale producers.


25 Apr 01 - CJD - Mad Cow disease theory challenged

by Penny Fannin, Science Reporter

The Age--Wednesday 25 April 2001


An amateur British scientist's belief that Mad Cow disease is caused by cattle being exposed to the metal manganese and a common insecticide has gained acceptance from members of Britain's scientific community.

Mark Purdey, an organic farmer, has spent 15 years collecting evidence that the British Government's explanation for Bovine Spongiform Encephalopathy (BSE) - that it is caused by animals being fed infected meat and bone meal from sheep infected with scrapie - is wrong.

It is widely believed that variant Creutzfeldt Jakob Disease - the human form of Mad Cow disease - is caused by eating BSE-infected beef. But Mr Purdey believes high levels of manganese in the environment and exposure to the pesticide phosmet don't just affect cows, but also make people susceptible to CJD.

His findings were detailed in a BBC Panorama special on the ABC's Four Corners last night.

David Brown, a CJD researcher at Cambridge University, believes Mr Purdey's theory has merit. "I think the general view of what caused BSE is wrong," he told Panorama.

When BSE was identified in 1986, Mr Purdey noticed that areas where the disease was emerging broadly corresponded with those where organophosphate pesticides such as phosmet had been used against the pest warble fly.

In experiments, Mr Purdey and Dr Stephen Whatley at the Institute of Psychiatry found that phosmet increased the number of prions - proteins produced in human and animal brains. Usually, prions exist for a few hours but in diseases like BSE they become almost indestructible, building up in brain cells until the cells die.

Mr Purdey believes these higher prion numbers, coupled with increased manganese in the environment, could cause BSE. He studied the environment in so-called cluster areas of spongiform encephalopathies, including Colorado and Iceland. In each he found high levels of manganese and low levels of copper.

Dr Brown found that prions starved of copper and dosed with manganese change their shape to the dangerous form of the prion.

Dr Steven Collins, coordinator of Australia's CJD case registry, said Mr Purdey's ideas were known but had not gained wide acceptance.

He said they needed to explain why outbreaks were recorded among zoo animals that were fed protein and meat meal. "He has to have a whole explanation. It has to cover all dimensions of the epidemic. Why has the epidemic diminished? Has there been a change in pesticide use or manganese levels?"


25 Apr 01 - CJD - Conference confronts Mad Cow

By Kathleen Curry

Charlotte.com---Wednesday 25 April 2001


Food-borne diseases get spotlight at gathering of U.S. meat industry

Food safety wasn't supposed to dominate the first day's agenda of the 2001 Meat Conference, which began Sunday in Charlotte.

But with terms like "Mad Cow" and "foot-and-mouth disease" filling press reports, the annual gathering of U.S. meat producers, processors and sellers couldn't push it off their plates.

"We didn't have this topic in the original brochure, but as the crisis of BSE (Bovine Spongiform Encephalopathy, or Mad Cow) and foot-and-mouth disease escalated in Europe, we all began getting questions and we knew we had to address it," said Joe Luter IV, co-chairman of the conference and a senior vice president for Smithfield Packing Co. in Smithfield, Va.

So the conference asked a leading U.S. expert on the subject, Dr. William Heuston, to tell them what to tell their customers.

"I've been talking about (BSE) for 12 years and for the first 10 years the only people who wanted to talk or hear about this were a few cattle producers," Heuston told a packed auditorium.

"I am thrilled to be invited here (and) very heartened by this change," Heuston said.

That change comes as customers bombard grocers and meat producers with questions about food safety.

Mad Cow disease has been headline news for several years; and an occasional e.coli breakout makes the news. Then, in recent months, Europe's struggle with a mass breakout of foot-and-mouth disease in cattle has pushed concern to a fever pitch.

"First, if there is only one thing you take away, let it be this: BSE is not the same thing as foot-and-mouth disease," said Heuston, chair of the University of Maryland's Department of Veterinary Science. Foot-and-mouth is harmless to humans. But Mad Cow, or BSE, is linked to a human brain wasting illness, variant Creutzfeld Jakob Disease (vCJD), that has killed an 101 people - 97 in Great Britain, three in France and one in Ireland - since 1995.

The confusion is causing wrong information to become fact to many, Heuston said.

"No BSE or variant CJD have ever been identified in the United States," he said. "And the possibility that we will ever see any BSE here is very low."

Largely because U.S. cattle are farmed and fed using very different - and safer - methods than are common in Great Britain, BSE is much harder to spread in the U.S. It is not contagious and is only spread through fodder.

Foot-and-mouth disease is very contagious, but is no danger to humans. Infected animals must be destroyed, however.

Other speakers noted that food-safety problems aren't limited to the very rare BSE. Salmonella and campylobacter in poultry and pork, along with staph contamination of all meat, are bigger problems now.

Dr. Lester Crawford, former head of the U.S. Department of Agriculture's Food Safety and Inspection Service, considers lack of communication about food-borne illnesses the key.

"For many years, talking about food safety was a negative thing in the industry, a definite no-no," said Crawford, now director of the Georgetown Center for Food and Nutrition Policy.

"Food safety is the most important issue we face," said Brad Graham, vice president of perishables for the Harris-Teeter grocery chain. Graham also co-chaired the industry's conference. Graham said he gets several calls a week from customers about food safety. "Our customers are concerned."

Frank talk about food-borne illness beyond BSE, including salmonella and campylobacter - a potentially much bigger pathogen - should begin, he said.

A raft of new regulations and technologies have helped U.S. meat producers and sellers to grab more control, Crawford said. For example, DNA "fingerprinting" technology has been a breakthrough, helping inspectors nail down the origin of any tainted bacteria, from the farmer or processor to grocer or food preparer.

"What we do have now is much more accurate. But the news is not all good," Crawford said.

According to the Centers for Disease Control in Atlanta, Crawford said, food-borne illnesses contributed to more than 76 million illnesses annually, including approximately 5,000 deaths and 325,000 hospitalizations.


25 Apr 01 - CJD - State of European research on BSE and related diseases

EU

European Commission--Wednesday 25 April 2001


-------------------------------------------------------------------------------- DN: IP/01/586 Date: 2001-04-23

TXT: FR EN DE ES IT PDF: FR EN DE ES IT Word Processed: FR EN DE ES IT

IP/01/586

Brussels, 23 April 2001

State of European research on BSE and related diseases

Today, a group of leading experts established by Philippe Busquin, European Commissioner for research, presents a comprehensive inventory of research on Transmissible Spongiform Encephalopathies, such as BSE, in order to give a clearer picture on research currently undertaken in Europe. The inventory that is published today identifies strengths and weaknesses of European research in this field. For example, it calls for increased co-ordination, networking, better communication and exchange of results between the various national efforts. It shows that the European research effort is handicapped by several factors such as the lack of well characterised sample materials, limited availability of animal models and cell lines and lack of trained scientists to carry out the research. The group was set up by Philippe Busquin following a suggestion made by Member States' research ministers in their meeting of 16 November 2000.

Commissioner Philippe Busquin said: "The inventory will help to promote complementarity and increased coherence into European research efforts in a field of research which is crucial for the well-being of European citizens. The creation of the inventory is a practical example of how the European Research Area, which is being realised by the Commission and the Member States, can help to bring forward a combined and stronger response in Europe."

All crises, in particular those related to human health, call for a quick assessment of the situation and the establishment of a common action plan highlighting research priorities, based on the full solidarity of all parties concerned. This is especially true in the case of BSE. The research community active in this field has been directly challenged and encouraged to join forces and provide the scientific knowledge that is needed to better cope with the problem, but also to advise on how to deal more effectively with similar epidemics in the future. Moreover, as most European countries are concerned by TSE, common public health measures, analytical standards and diagnostic procedures must be agreed.

This is why the Commission has already taken several initiatives in this area, through its EUR 50 million Action Plan on TSE launched in 1996.

As a result, a considerable research effort on TSE is underway in Europe, underlining the public concern and economic cost and the need to answer some of the fundamental issues as to the agent and its spreading.

This research is however uneven and variable, reflecting different approaches and scales of the problem in the various Member States. So far, no complete picture did exist as to what research is carried out by whom in the different parts of Europe.

On the basis of the inventory's conclusions, the Commission's Research DG intends to inject further EU funds in relevant research activities. A forthcoming call for proposals will address some of the major issues such as better co-ordination of the research efforts, opening up of ongoing research capacities in Europe and will focus on a limited number of issues such as research leading to the development of live tests, inactivation of prions, animal TSE transmission and the risk to humans.

Interestingly enough, several priorities identified by the experts such as networking and better coordination correspond to the objectives of the European Research Area. The European Research Area intends to ensure co-ordination of national programmes and complementarity between research agendas. In a very specific context, the BSE crisis shows how urgent and important it is for the Union to realise the European Research Area.

Mad Cow disease - Some figures

It was in 1986 that the Central Veterinary Laboratory in the UK first identified two animals infected with an unknown form of Bovine Spongiform Encephalopathy (BSE) in two British herds. By the end of 1989, 10 000 cases had been recorded.

Emergency measures, including the ban on animal-derived feed - quickly established as the origin of the catastrophe - had little effect. The situation worsened. By 1992 the epidemic had struck 37,000 cattle throughout the UK.

Between 1987 and 2000 almost 180 000 cattle were infected in the United Kingdom, compared with 1325 cases recorded to date elsewhere in Europe (mainly in Ireland, Portugal and France). 97 people have been identified as having the new variant of the Creutzfeld-Jacob disease (CJD) in the United Kingdom, compared to three cases in France and one in Ireland.


25 Apr 01 - CJD - Emu farmers gain from European meat scares

Ananova

PA News--Wednesday 25 April 2001


German importers may have help guaranteed the future of America's fledgling Emu farm business.

They have placed an initial order for 250,000 kilos of emu meat to keep up with public demand caused by the BSE and foot-and-mouth meat scares.

It is said to be the largest order ever received by the emu meat industry in the US.

Donna Womack, the vice-president of the American Emu Association, says the order may mean the emu industry in Texas is now there to stay.

She added that orders were also coming in from other European countries following the German order.

In the US, emus are usually bred for the cosmetics industry which uses their oil. The industry has been doing poorly in recent years and many farmers have given up production.

Emu meat is low fat and full of protein and is said to taste like a cross between beef and chicken.

Ostrich farmers in the US have also increased production to meet the growing demands for meat from Europe.


25 Apr 01 - CJD - EU ministers prolong ban on Meat and Bone Meal-based animal feed

Ananova

PA News--Wednesday 25 April 2001


LUXEMBOURG (AFX) - EU agriculture ministers have agreed to prolong the ban on animal feed derived from meat or bone products which was set to expire at the end of June, said EU sources.

The European Commission proposed extending the ban, set up to prevent the spread of Mad Cow disease or BSE, judging a provisional lifting "premature" after the findings of its enquiry.

A representative of the current EU presidency, Sweden, said that ministers had agreed on a continued temporary ban, but that no timescale had been fixed.


25 Apr 01 - CJD - Scientists 'develop quick BSE test'

Ananova

PA News--Wednesday 25 April 2001


Austrian scientists say they have developed a BSE test which works within minutes on live animals.

The group, from the University of Linz, claims the new test is revolutionary and would eventually detect disease in humans and food or drink.

The test needs only one drop of an animal's blood to detect catalysts and harmful substances which show BSE.

Upper Austria government spokesman Josef Fill said the method gives an "exact and reliable picture" within minutes of catalysts and harmful substances in an animal's blood.

The head of the university's biophysics department, Hans-Georg Schindler, the test allows scientists to see even the smallest molecules in the blood in a way which was not possible before.

Schindler claimed the test would put an end to mass slaughter of cattle and other animals and would be ready in six months. He said international approval and licensing would take another year.

"In about one and a half to two years, it may be used on a broad basis and would be a first step in cases of suspected BSE," he said.

Mr Schindler said human tests would be possible in five to 10 years, and that by making pathogens in blood visible, a number of other illnesses could be diagnosed at a very early stage using similar techniques.


25 Apr 01 - CJD - Leather Furniture Makers Feel Pinch

Associated Press

JS Online---Wednesday 25 April 2001


HIGH POINT, N.C. - When Leathercraft president Jack Donahoe sits down with buyers this week at the world's largest furniture trade show, he's keeping an eye on the price of animal hides in Europe and South America.

Like many of his counterparts, Donahoe is concerned about the impact of foot-and-mouth and Mad Cow diseases on furniture manufacturers who rely on an ample, affordable supply of animal hides.

``I heard meat consumption is down 60 percent in Germany,'' he said. ``The fact is that if people choose not to eat beef, even the healthy cows won't go to slaughter. This will reduce the supply and increase our costs.''

Another manufacturer, American Leather, is telling buyers it is increasing prices for leather sofas and other pieces by 4 percent. More price hikes could follow if the efforts to control the livestock diseases reduce supplies further.

American Leather, a high-end manufacturer, has benefitted from the skyrocketing popularity in leather furniture, which has grown from about 4 percent of the total U.S. market 20 years ago to about 25 percent.

``The real worry is there's no precedent for this problem,'' said American Leather sales and marketing chief Cary Benson, who said his company's business is expected to grow this year by 10 percent. ``That's why we've decided to be totally up front with our clients.''

Leather furniture isn't the only industry feeling the pinch. Goods that rely on meat byproducts range from shoes and handbags to face cream and soap.

Furniture industry analyst Jerry Epperson said the price of hides rises and falls depending on the number of livestock that go to slaughter.

``It's a real concern anytime people stop buying meat,'' he said. ``It's a big deal for us.''

Leather furniture makers depend on a steady supply of top-quality hides to keep up with the demand for their products, he said.

``I've read somewhere that two companies, Klaussner Furniture and Natuzzi, use six times as much leather as Nike,'' Epperson said.

Epperson said many consumers don't realize there's a large difference in the way officials are trying to rein in Mad Cow disease and foot-and-mouth disease. With foot-and-mouth disease, the entire animal must be destroyed. In Mad Cow disease, the meat is destroyed but the hides can be saved and later sold.

``You just might have a Mad Cow sofa in your house right now and you don't know it,'' he quipped at a press briefing on the opening day of the furniture market last Thursday.

Nevertheless, Epperson and the industry executives said, it's consumer fear about Mad Cow disease that's reducing demand for meat, which cuts down on the supply of hides.

The situation changes almost on a daily basis, with leather tanneries reviewing their pricing as the availability of hides ebbs and flows. Furniture manufacturers are absorbing some of the price increases but part of it will certainly be passed to the consumer.

``We're figuring around $50 more per sofa at the wholesale level and $100 at retail,'' said Donahoe, whose company makes sofas that sell for about $2,000 and above. He predicted wholesale prices will continue to rise over the next six months unless conditions change dramatically for the better.

``All we can do right now is react,'' he said. ``This first round of price increases could be the tip of the iceberg.''

Bans imposed by nations around the world because of Mad Cow and foot-and-mouth diseases have halted 94 percent of the European Union's beef exports and 73 percent of its pork exports. They also have slowed Europe's weekly livestock slaughter from a half million head to just 350,000 head, according to Hidenet.com, an Internet market report.

The supply of leather is down by as much as 75 percent in Britain, lifting the price of local leather by about 20 percent. Worldwide, the supply squeeze has pushed up cowhide prices 15 percent.

Donahoe, who has been studying the markets closely, said he's hopeful the efforts to control the livestock diseases will succeed sooner than later. But he warned that the problems the disruptions in supply created may stick around.

``It's hard to replenish the cattle supply if a lot of the animals are destroyed,'' he said. ``It takes a long time to get it back.''

And what if consumer demand for meat remains low even after the livestock diseases are gone?

``It's scary,'' Epperson said.