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Another Miami meat worker dies of CJD
250,000 cattle needlessly infected
Cheap meat blamed for mad cow in humans
CJD victim's father appears before BSE inquiry
22,000 hemophiliacs file suit over tainted blood in Canada
Protesters at first court test of bone-in beef ban
Ministry 'amended report' on early BSE case
Scientist was horrified by inaction over BSE
Study of nuns and their brains sheds new light on Alzheimer's

Second Miami meat worker dies of Creutzfeldt-Jakob Disease

 Reuters North America Thu, Mar 12, 1998 By Jim Loney
MIAMI - Medical investigators are looking into the death of a Florida meat warehouse laborer who died of the brain-wasting Creutzfeldt-Jakob Disease, authorities said Thursday. Ozzie Hyman, 66, of Miami died March 5 of the degenerative disease, a rare affliction that affects just one in one million people, officials said.

Hyman was not a victim of the variant strain of Creutzfeldt-Jakob Disease (CJD) linked by British researchers to bovine spongiform encephalopathy (BSE), better known as mad cow disease, officials said.

"It was a person who died of Creutzfeldt-Jakob Disease. It is a disease that people like to connect or somehow relate with mad cow disease," said Dr. Eleni Sfakianaki, medical executive director of the Dade County Health Department. "This is a human case."
Sfakianaki said the variant type of CJD has not appeared in the United States. "This is a typical, regular CJ disease," she said. "It is a very rare disease, the incidence is about one in one million people. "There is no reason for people to panic, no reason to be alarmed at all."

In a March 6 statement, the Dade County Medical Examiner's Office said: "The relationship of this condition to his (Hyman's) occupation, a meat warehouse laborer, is under investigation and remains to be determined."

Testing to date found Hyman was a victim of sporadic Creutzfeldt-Jakob disease and not the variant strain of CJD linked by British researchers to bovine spongiform encephalopathy (BSE), known as mad cow disease, officials said.

"The clinical course (progression of the disease) doesn't fit the variant disease that was described in England," Dr. Bruce Hyma, a Miami-Dade County associate medical examiner, told Reuters. "His clinical course indicates CJD."
Concerns the Miami death might renew fears that eating beef could lead to CJD contributed to lower cattle prices early Thursday at the Chicago Mercantile Exchange, traders said. Prices recovered at the exchange later when traders learned the county health department had not attributed Hyman's death to the CJD strain linked to mad cow disease.
"The impact (to cattle prices) is negligible until something is confirmed that would link that death to mad cow disease," said Chuck Levitt, senior livestock analyst at Alaron Trading Corp in Chicago.
Dade County authorities launched an investigation in part to quell perceptions of a public health threat and partly because Hyman worked in a meat warehouse, Hyma said. "It brings up the question -- is there a relationship? From all the information I've seen so far, he would be a sporadic case of CJD. He just happened to have that occupation," he said.

Brain tissue samples were being sent to Case Western University in Cleveland, Ohio, for examination by experts in Creutzfeldt-Jakob disease and the U.S. Centers for Disease Control and Prevention has been notified. The first case of BSE was confirmed in Britain in 1986, a result of contaminated cattle feed, British researchers say. Mad cow disease resulted in the slaughter of thousands of cattle in Britain and abroad and prompted the European Union to impose a beef export ban on Britain in March 1996. In Texas, U.S. talk show queen Oprah Winfrey prevailed last month in a court battle with cattle ranchers who claimed her comments on mad cow disease caused beef prices to plunge.

After the Miami death, Dr. Eleni Sfakianaki, medical executive director of the Dade County Health Department, said the variant strain linked by British researchers to mad cow disease had not appeared in the United States. Hyman's death was "typical, regular CJ disease," she said. "There is no reason for people to panic."

British microbiologist John Pattison, whose research linked mad cow disease to the human CJD variant, played down the Florida case, saying: "They've had such unproven reports from Florida before."

The earlier Miami meat worker case

AP US & World Thu, Mar 12, 1998  By CATHERINE WILSON
MIAMI (AP) -- The death of a meat warehouse worker from a rare human equivalent of mad cow disease has medical investigators searching for any link between his occupation and his illness. Investigators also are pursuing an earlier report of the death of another Miami meat worker from the same disease, Dr. Steven Wiersma, deputy state epidemiologist, said Thursday.

"We don't feel there's a lot of reason for alarm that this occupation could be at higher risk," he said.

Dr. Lawrence Schonberger of the federal Centers for Disease Control and Prevention in Atlanta, said, "I don't think it's a public health concern," but that the CDC would help if needed.

Meat handlers have shown no increased rate of either the classic form of the degenerative brain disease or a deadly variant blamed in Britain on contaminated beef. But the death of Ozzie Hyman, 65, is getting a closer look.

Hyman worked for the Jacksonville-based Winn-Dixie supermarket chain from August 1976 until he left on sick leave last Oct. 29, said company spokesman Mickey Clerc. Hyman died at home March 5, the Miami-Dade County medical examiner's office said. The company released no job description, but Wiersma said his occupation was listed as meat stocker.

About one in 1 million people die each year worldwide from the classic form of Creutzfeldt-Jakob disease. Based on his age and symptoms, Hyman's death initially appears to be from classic CJD, Wiersma said. Beef contaminated with mad cow disease was identified as the most likely cause of the deadly variant that killed 27 Britons. That variant has not been known to have occurred in the United States. Wiersma said only unusual cases of Creutzfeldt-Jakob disease are investigated. Florida recorded 14 deaths from the disease in 1996, the last year for which figures are available.

Experts: deaths not mad cow disease; two meat workers died of cjd, which is similar to famous ailment

Sun-Sentinel (Fort Lauderdale, FL) March 13, 1998
NANCY McVICAR; Staff Writer; Sun-Suntinel Staff Writer Deborah Ramirez and  Maya Bell of The Orlando Sentinel
The deaths of two Miami-Dade County meat industry workers from a disease similar to mad cow disease within a few months of each other is intriguing, but likely just a coincidence and is no cause for alarm, health experts said on Thursday. ''No evidence exists to link the human form of the disease to the bovine form. There is no evidence that it is transmissible from cow to human,'' said Dr. Bruce Hyma, Miami-Dade associate medical examiner, who is investigating the most recent death. Nevertheless, Hyma is sending tissue samples from the brain of Ossie Hyman, 66, a meat warehouse worker, to Case Western University in Cleveland for further study.

And Dr. Steven Wiersma, deputy state epidemiologist for the Florida Department of Health, said his office is interested in the cases, but he does not think they are related to mad cow disease. Hyman died last week of Creutzfeldt-Jakob disease, also known as CJD, a degenerative brain disease often compared to mad cow disease, because they have similar symptoms and are thought to be caused by the same kind of infectious agent _ a prion, which is neither a virus nor a bacteria, but an abnormal protein.

Last October, Tomas J. Rodriguez Sr., 69, who had worked as a butcher in Miami-Dade for 18 years until he retired in 1993, also died of CJD, said his son Tomas Rodriguez Jr. The diagnosis was confirmed by a brain biopsy, he said. Hyma said he had heard about the Rodriguez case, but because he died in a hospital and the cause of death had been established, it did not become a medical examiner's case. The state records about 15 cases of CJD a year, which is about the national norm of one case per million population.

Hyma and other health experts said two cases involving meat workers are unusual, but most likely are coincidence. ''If this were something transmitted from meat in the United States, we would see these cases all the time, and this is only the second case I've been involved with in 11 years,'' Hyma said. He said he is sending Hyman's brain tissue specimen to Case Western ''so it can be properly investigated and all the rumors can be laid to rest, because the rumors are running rampant.'' Case Western has a laboratory that does research on diseases such as CJD and mad cow.

Reuters news service reported that cattle prices at the Chicago Mercantile Exchange dropped Thursday because of news reports about Hyman's death. Prices recovered after Miami-Dade health officials said the case was not linked to mad cow disease.

But Hyman's wife is suspicious that he may have been exposed to something on the job. ''He was a man in good physical condition. He didn't take any medication. He had the blood pressure of a young man,'' said Willie Hyman, his wife, who trained as a nurse. Hyman called in sick only four times during his 21 years as a butcher, so when he told her he wasn't feeling well and had trouble walking on the morning of Sept. 26, she immediately called a doctor. Tests were ordered, among them a brain scan, and doctors diagnosed CJD. He lived about five months. Willie Hyman has hired an attorney to help investigate her husband's death, but Wiersma said he didn't think his job was a factor.

Dr. Ermius Belay, an expert on CJD at the federal Centers for Disease Control and Prevention in Atlanta, said he was aware of the two cases and that they should be investigated, but the fact that the two men handled meat ''is not a big deal. There is nothing to be alarmed about.'' He was critical of some news reports about the cases and said they did not distinguish between the new variant of the disease found only in Great Britain, and the classic form of the disease that has been around since it was identified in the 1920s.

Health officials in the United Kingdom announced in March 1996 that they had identified 10 cases of a new type of the disease in young people and that they probably had contracted the disease from eating beef tainted with bovine spongiform encephalopathy, or BSE, the scientific name of mad cow disease. There have been no cases of the new form of the disease in the United States because there has been no mad cow disease in this country nor any mad-cow tainted beef imported into this country, he said. Belay said the new form of the disease is easily discernible from classic CJD. Its onset is in younger people. Classic CJD occurs most often in people older than 55. And patients with the new form of CJD most often sought treatment first in psychiatrists' offices. Only in later stages did they develop the neurological symptoms common to classic CJD memory loss, speech abnormalities, imbalance, unsteady gate and involuntary muscle movements. Their brain tissue also appears markedly different, he said, so it is not difficult to distinguish between CJD and the new variant at autopsy.

After the announcement in Great Britain, the CDC established a surveillance program to ensure that if any cases did occur in the United States, they would be quickly reported, Belay said. Case Western has long had a lab doing research on prion diseases _ prions are the protein agents thought to cause CJD and similar diseases _ and the lab is cooperating with the federal Centers for Disease Control and Prevention. The American Association of Neuropathologists also has been asked to report any cases that appear to be the new variant of CJD, he said. In addition, in five areas of the country, state health departments have been asked to track any cases of CJD in people under 55 and send clinical and pathology reports to the CDC, Belay said.

All CJD cases in the country are tracked through death certificates and are compiled in a database at the National Center for Health Statistics, part of the CDC, he said. Little is known about its transmission, but human-to-human transmission has occurred inadvertently through corneal and other transplants. [Misleading: 'all CJD cases' means those that happen to be diagnosed and reported on the death certificate. CJD is a non-reportable disease. -- webmaster] ''The data suggests the death rate has been quite stable over the years,'' Belay said. There is still about one case of CJD per million people. Wiersma, of the state health department, said his office has been tracking CJD cases in the state and has not found any cases of the new type. ''We're requiring that (brain) specimens from people under 55 go to the Case Western lab,'' he said. ''It's another good way to make sure we don't have any cases here.''

Wiersma said reports of a cluster of CJD cases in the Tampa area 11 in a 16-month period are being investigated, but it could be that the deaths occurred there because there is a doctor in the area who is a specialist in treating them. ''First of all, I don't think it's been confirmed. And one of the issues in looking at clusters is finding out where these people came from,'' Wiersma said. ''The fact that he's seeing a lot of cases there may reflect that a lot of people are going there for help.'' Wiersma said the state is re-reviewing data on cases in the state ''to make sure there is not an increasing trend.'' Sun-Suntinel Staff Writer Deborah Ramirez and Maya Bell of The Orlando Sentinel contributed to this report.

250,000 cattle `needlessly infected'

 PA News Thu, Mar 12, 1998 By Andy Gales, PA News
An expert will tell the BSE inquiry that 250,000 cattle were needlessly infected with the disease, it emerged tonight. Professor Roy Anderson of Oxford University's Centre for Infectious Diseases says herds were given contaminated feed despite mounting concern over the safety of offal--based foodstuffs.

Between 1989 and 1991 he claims to have made several approaches to Ministry of Agriculture officials in a bid to understand the epidemic but was denied access to BSE data.

His research showed that the meat and bone meal feed ban already in place was not effective as new cases of mad cow disease continued throughout the early 1990s.

In a prepared statement, Prof Anderson will say: "If this had been known at the time, and if measures to prevent the continued use of contaminated feed had been put in place, the size of the epidemic would have been significantly smaller, by about one quarter of a million infected cattle." Prof Anderson will read the statement to the inquiry in London on Monday.

Cheap meat reportedly to blame for mad cow in humans

March  12, 1998 Reuters News Service  by June Preston 
A British scientist whose research linked mad cow disease to a deadly strain of a brain-wasting disorder in humans said on Wednesday that cheap hamburger was to blame for the outbreak.

Microbiologist John Pattison, whose work led researchers to conclude a variant strain of Creutzfeldt-Jakob Disease (CJD) in humans was tied to bovine spongiform encephalopathy (BSE) in cattle, told Reuters ground beef was to blame for the deaths of 24 people in the United Kingdom in late 1995 and early 1996.

"In the U.K., the big outlets like Burger King and McDonalds actually use minced muscle meat in their burgers," he said. "But if you buy very cheap frozen burgers or you buy something out of a van, I have no idea what's in those."At the lower end of the market, it's very hard to know what's in the food. That includes cheap hamburger, possibly the hot dog and some of our meat pies. They're not governed by any sort of regulations or recipe or whatever," he said.
Scientists from 70 countries were in Atlanta for a conference on emerging infectious diseases, and on Wednesday morning Pattison told them he fears human-to-human spread of the CJD variant is possible through contaminated blood transfusions.But he later told Reuters the greatest culprit of all in transmission of the disease to humans is hamburger.

The first cases of variant CJD diagnosed involved teen-agers, leading scientists to wonder what dietary preferences might contribute to the disease. Pattison said cheap ground beef was the common bond.

"It's a common thing, really," he said. "We have this huge drive toward cheap. The (profit) margins are small. Some people make a living off of small margins by putting everything in, every part of the animal. And, even though it's on the label, we buy it anyway," he said.
Pattison chairs Britain's Spongiform Encephalopathy Advisory Committee. His recommendations led to the British government's controversial ban of beef on the bone in December. Earlier in the day, Pattison told fellow scientists that although variant CJD has not claimed a single fatality in Britain since 1996, it could resurface through blood transfusions
."We continue to use whole blood products in the U.K," he said. "There is no thought being given to importing blood, because there is a huge counterbalance to be considered involving other diseases that would be far worse than the danger of CJD."
It is not impossible that CJD could be transmitted through the blood supply because it has a long incubation period and consumption of the affected cattle did not stop until 1996, he said.The first case of BSE was confirmed in Britain in 1986, a result of contaminated feed. Since then, 170,000 cattle have died of the disease, according to Pattison's report.

He said the likely culprits in the continued BSE spread were family farmers who gave their cattle feed intended for poultry and pigs. BSE cases among cattle have dropped dramatically since meat products were banned from all feed, Pattison said, but it has not been eliminated."The BSE epidemic is under control," he said, "However, there are still going to be about 1,500 to 2,000 cases in the U.K. this year."

A couple of interesting points from Wednesday's Inquiry transcript:

1.  Sir Richard Southwood: (p.45 line 8):

 8  You will also see in  our report we were concerned about the amount of
 9   bonemeal, and meat and bonemeal imported from the
 10   United States both because we had noticed that the
 11   amount of scrapie in the United States had increased
 12   rather violently in the previous period.
The Human BSE Foundation: mentioned in the Inquiry, this is apparently a new support group for affected families. The CJD Support Network seems to be a different and older organization. Gillian Turner is the National CJD Co-ordinator. It is at Birchwood, Heath Top, Ashley Heath, Market Drayton, Shropshire, TF9 4QR.

CJD victim's father appears before BSE inquiry

 PA News  Mon, Mar 9, 1998  By John von Radowitz, Science Correspondent
The father of a tragic young victim of the human illness linked to "mad cow disease" will give evidence at the BSE inquiry today.

Vegetarian Clare Tomkins, 24, had not eaten meat for 12 years before being diagnosed as having the new variant form of CJD. She is now dying from the disease which most experts believe was triggered by BSE passing to humans through infected beef.

Her father, Roger, a company director, and GP, Dr Nick Cheales, will be among the first witnesses to give evidence on the second day of the BSE inquiry in London today. Clare, from Tonbridge, Kent, was looking forward to a happy future with her fiance Andrew Beale when she was struck down by the disease. The couple were engaged in 1994.

Roger Tomkins said his daughter Clare had been a "stunning strawberry blonde" who enjoyed life to the full, loved animals and was looking forward to getting married. But in the space of just six months she was reduced to a tragic wreck of a human being who could not control her movements, cowered in fear from members of her family and howled at night like an injured animal.

She showed the first symptoms in January, 1996, when dark depressed moods began to replace her normally bright, vibrant personality and she lost enthusiasm for her work at a local pet shop. At first it was thought she was suffering from a psychiatric complaint. But over a period of two and a half months doctors came to the conclusion that her condition was not psychological but neurological.

He told the BSE inquiry, sitting in London, that Clare, 24, had been a strict vegetarian since the age of 13 because of her love of animals. The father described how Clare's symptoms progressed from her suffering depressed moods and a bad taste in her mouth to the point today where she is permanently bedridden and has to be cared for night and day at home.

As he gave his evidence, relatives of other new variant CJD victims attending the hearing wept openly.

At the end, Mr Tomkins - who kept his composure throughout - was congratulated on his courage by inquiry chairman Lord Justice Phillips and given a spontaneous round of applause.

Mr Tomkins from Tonbridge, Kent, said he first noticed that Clare was not herself in October 1996. She had returned from a holiday with her fiance Andrew Beale in an uncharacteristically depressed mood.

The petite blond, 5ft 2in tall, began to lose weight, dropping from seven stone to six stone and complained of a nasty taste in her mouth. She became increasingly depressed to the point where she was crying for no reason and could no longer face her job in the pet department of a local garden centre.

The disease was eventually confirmed from a tissue sample by experts at St Mary's Hospital, London, which specialises in the investigation of CJD. Clare's case is significant because of her vegetarian background. If her condition was caused by eating BSE-infected beef, she must have contracted the disease before 1986, the year in which the first case of BSE in cattle was confirmed.

She is also one of an unusually large number of people from rural Kent to have fallen victim to the new form of CJD. [According to David Body, solicitor for the families of nvCJD victims, there have been 27 cases, ie 4 unannounced. If any of these are near Ashford, Kent this theory would be proven --- webmaster]

The first cow identified with BSE was found on a Kent farm in 1985. New variant CJD has claimed at least five victims from the county. The area contains a landfill site where cattle with BSE were buried and a rendering plant which has been criticised for spreading effluent including boiled cattle remains on the land.

The region was also the site of Britain's worst incident of pesticide poisoning in 1963. A factory producing organo-chlorine pesticides accidentally contaminated two acres of farmland, killing cattle, pets and wildlife. Organo-chlorines are chemically similar to organo-phosphate pesticides which some claim to be the cause of BSE.

The latest official figures issued by the Department of Health this month show that so far there have been 23 definite and probable cases of new variant CJD. According to David Body, solicitor for the families of nvCJD victims, there have been 27 cases.

Yesterday Lord Justice Phillips, heading the inquiry, was granted an extra six months in which to complete the investigation before submitting his report. He had told Prime Minister Tony Blair he needed more time to consider the growing mountain of evidence. The delivery date for the report is now June 30, 1999.

BSE experts `horrified' at cattle slaughter methods

 PA News Tue, Mar 10, 1998 By John von Radowitz, Science Correspondent
Members of a working party appointed by the Government to look at the dangers from mad cow disease were "horrified" at the way cattle were being slaughtered, the BSE inquiry will hear today. They feared the greatest risk to humans might be from baby foods containing cattle offal.

And they encountered resistance from the then Government to proposals to contain the spread of the disease and carry out further research. Professor Sir Richard Southwood was asked to chair the committee in April 1988 by the Chief Medical Officer at the time, Sir Donald Acheson. In a statement to the inquiry, he said when the group first met in Oxford on June 20, 1988 they were told cattle showing symptoms were entering both the animal and human food chains. John Wilesmith, head of epidemiology at the Ministry of Agriculture's Central Veterinary Laboratory, told them "the head is taken off with a chain saw".

Sir Richard said in his statement: "It is fair to say that we were all horrified. "We considered that it was essential that arrangements were put in hand immediately to prevent any part of an animal suspected of having BSE entering the food chain." The very next day he wrote to the Permanent Secretary at the Ministry of Agriculture calling for a thorough programme of research and the destruction of carcasses of infected animals. He also expressed the committee's doubts about the temporary nature of the order banning ruminant remains in cattle and sheep feed, which had just been passed.

He said: "I think we all felt - without evidence - that its `temporary' nature was a concession to the industry, and that this was dangerous as it would encourage complacency." After another, strongly worded recommendation to continue the feed ban indefinitely, the Government announced it would be extended for a year. Later, the ban was made permanent. Sir Richard said his team was "extremely anxious" about the risks to man and discussed the sale of offal. He recalled consulting local butchers on the sale of ox brains.

"We thought that the greatest risk might be in baby foods that are homogenised and might `contain anything'," said Sir Richard, in his statement. "By analogy with some other diseases it might be that babies were more susceptible (it also seemed as if cattle were mostly infected when calves)."

He said the committee made a recommendation which eventually led to the 1989 ban on specified high-risk offals being used for human consumption. The group also considered the case for insisting that any food containing bovine offal should be labelled. But they were advised this would be difficult to enforce and at the time Britain was having problems with European Union proposals on food labelling.

Sir Richard said the committee had been concerned about the slow progress of Government research into BSE, particularly in respect of investigating the possibility of the disease passing from cow to calf. "The failure to support or undertake more research was regrettable," he said in his statement. Sir Richard will give evidence today along with two members of his committee, Professor Sir Anthony Epstein, from Bristol University, and Dr W B Martin, director of the Moredun Research Institute, Edinburgh.

Hemophiliacs file suit over tainted blood in Canada

Reuters News Service TORONTO March 10, 1998 
[There are uncanny similarities here with CJD in France and elsewhere-- webmaster]

Some 22,000 hemophiliacs who became infected with hepatitis C through blood transfusions have brought a multi-billion dollar class action lawsuit Tuesday against Canada's federal and provincial governments and the Red Cross.Toronto lawyer David Harvey, who represents the hemophiliacs, said the suit seeks C$3.8 billion ($2.7 billion) in damages and he claimed it was the largest tainted-blood class action lawsuit in the world.

"They (the Canadian government) have forced our hand and forced us into the courts which is a place we all agree we don't want to be," Harvey said. Hepatitis C is a degenerative liver disease that can lie dormant for years before carriers become aware they are infected and can be fatal in about 20 percent of cases. The class action suit contends various methods to screen blood for hepatitis C were available but not used in Canada during the periods covered by the lawsuit. It charges that Canada's federal government, its 10 provincial governments and the Red Cross were negligent for not implementing the screening.

The suit also alleges the Red Cross, which manages the blood-supply system, should not have pooled batches of plasma from as may as 20,000 donors and exposed recipients to these pooled transfusions.The court action in Ontario Court in Toronto follows an independent probe by the Canadian government into how the blood supply was contaminated.

The Royal Canadian Mounted Police are investigating the role of various health officials in the country's tainted blood scandal which may result in criminal trials, similar to those against health officials in blood cases in France and Japan.The Canadian government was expected to offer C$1 billion ($707 million) in compensation to 20,000 victims infected between 1986 and 1990, a time when the United States screened donors for hepatitis C but Canada did not.

That period is covered by another C$3.5 billion ($2.5 billion) suit but Tuesday's class action suit covers the periods prior to 1986 and after 1990. "The government is not going to follow the path of compassion but instead will offer compensation to about half the people affected," Harvey told reporters after filing the suit. Added hepatitis C victim Mike McCarthy of the Canadian Haemophilia Society: "They've let me down and thousands of other Canadians down ... I'm 38 going on 68."

Protesters at first court test of bone-in beef ban

March 11 1998 BY GILLIAN HARRIS Times
MORE than 100 farmers gathered outside Selkirk Sheriff Court yesterday to protest against the first prosecution for serving beef on the bone. The noisy crowd surged forward to cheer Jim Sutherland, 44, a hotelier from the Borders, as he arrived.

He is charged with serving prime Aberdeen Angus on the bone at a dinner held in deliberate defiance of the ban imposed two months earlier. David Kidd, Mr Sutherland's solicitor, told the court that the ban, imposed by Jack Cunningham, the Agriculture Minister, to prevent the remote risk of infection with "mad cow" disease, was "manifestly absurd".

Sheriff James Paterson adjourned the case until April 6, when five days have been set aside for a preliminary hearing into the legality of the regulations. After the hearing, Mr Sutherland said: "My lawyers will seek to challenge the legitimacy of the regulations."

His comment brought applause from the farmers, many wearing badges that read "I Am Backing Scotch Beef". Tom Douglas, a farmer from Selkirk, said: "The support for Jim is immense." About 170 opponents of the beef ban, mostly local farmers, attended the dinner held by Mr Sutherland at the Lodge Hotel, Carfraemill, near Lauder, on December 22. They were not charged for the meal but donated #1,700 to charity.

The dinner was interrupted by two health inspectors from Scottish Borders Council who arrived to charge Mr Sutherland with contravening the Food Safety Act. If found guilty, Mr Sutherland could be fined up to #5,000 or be jailed for up to six months. Alan Coomber, landlord of the Bell Inn at Iden, East Sussex, is the first person in England to be charged with breaking the ban. No court date has been set.

Ministry 'amended report' on early BSE case

March 11 1998 Times BY MICHAEL HORNSBY  Day two of Inquiry transcripts
A VET was forced by a senior Ministry of Agriculture official to amend the wording of a research paper on sick cows he had delivered in early 1987, the BSE inquiry was told yesterday.

Colin Whitaker, who works in Ashford, Kent, based the paper on his examination of the cows at Plurenden Manor Farm, at High Halden, Kent, which are now accepted as having been early cases of BSE. Mr Whitaker planned to present the findings at a conference of the British Cattle Veterinary Association in Nottingham. In the paper he referred to a "new scrapie-like syndrome".

However, the ministry had asked to see a copy of the report before it was presented and an unnamed official had insisted the words "scrapie-like" be taken out. [The change affected slide 17 in his talk. -- webmaster]

Another vet who dealt with what is now thought to have been the first outbreak of BSE said that he had been baffled by the symptoms, which were unlike anything he had seen. David Bee told the inquiry that he had been called to Pitsham Farm at Midhurst, West Sussex, in December 1984. The first cow he looked at was suffering from an arched back and weight loss and went on to develop tremors and loss of co-ordination, dying two months later.

"She was a mystery," he said. Others died and, after a post-mortem on one, Carol Richardson, a pathologist at the Central Veterinary Laboratory at Weybridge, Surrey, which is run by the Ministry of Agriculture, diagnosed a "moderate spongiform encephalopathy", but her superiors failed to act on her finding.

Scientist was horrified by inaction over BSE

March 12 1998 BY MICHAEL HORNSBY, Times AGRICULTURE CORRESPONDENT
THE scientist first appointed by the Government to investigate "mad cow" disease said yesterday that he had been horrified to discover how little had been done to protect the public from possible infection. Sir Richard Southwood, Professor of Zoology at Oxford University, also said that he had been concerned that the Ministry of Agriculture would be reluctant to take the necessary precautions because they might cost too much.

Appearing before the public inquiry into BSE, Sir Richard recalled that one day in April 1988, he had heard of BSE for the first time when he had been rung up Sir Donald Acheson, then the Chief Medical Officer. Sir Donald had told him: "I will tell you in great confidence that there is a potentially dangerous disease and I want you to be chairman of a committee to investigate."

In February of the following year, his group produced the first official report on BSE, about which little was then known. Sir Richard, accompanied by three members of his group, told the inquiry they had been concerned about the paucity of research funding and the inadequacy of compensation to farmers with BSE-infected cows. The group had first met in Oxford on June 20, 1988, and had learnt from a Ministry of Agriculture epidemiologist that only the heads of cattle showing symptoms of BSE were being removed and that the rest of the carcass was still being used for human food.

"It is fair to say we were all horrified," Sir Richard said. "We considered it was essential that arrangements were put in hand immediately to prevent any part of an animal suspected of having BSE entering the food chain." As a result of the working party's alarm, the compulsory slaughter and destruction of all cattle showing any signs of BSE was introduced in August 1988, nearly two years [make that 4 years -- webmaster] after government veterinary scientists had first confirmed the existence of BSE.

Sir Richard told the inquiry that Sir Derek Andrews, then the Permanent Secretary at the Ministry of Agriculture, had "expressed the hope to me that any recommendations we would make 'would not lead to an increase in public expenditure' ". This is disputed by Sir Derek, but a copy of the minutes of the meeting, cited yesterday at the inquiry, appeared to show that worries about cost had been one of the main reasons why the Government had not introduced a compulsory slaughter policy sooner.

The minutes report Sir Derek as expressing concern that "compulsory slaughter might well lock us into a very large public expenditure commitment, particularly if there was no prospect of eradication, because the current legislation required us to compensate".

Sir Richard said the group was worried that the Government was prepared initially to pay farmers compensation equal only to 50 per cent of market value for suspected BSE cattle that had to be slaughtered. William Martin, director of the Moredun Research Institute in Edinburgh from 1977 to 1985, a member of the group, said it was feared this had led many farmers to send sick cattle to market to get the full value.

Study of nuns and their brains sheds new light on Alzheimer's

Scripps Howard MILWAUKEE  March 14, 1998
When scientists examined the brain of an 86-year-old nun soon after her death, they found it was riddled with signs of Alzheimer's disease. Twisted fibers of protein called tangles and collections of protein masses called plaques littered the top of her brain, called the neocortex. Indeed, few brains they had ever examined among Alzheimer's disease patients had so many of these protein abnormalities. In addition, genetic tests showed the woman had two copies of the APOE4 gene.

Studies had previously shown that people with two copies of the gene have a roughly two-fold risk of developing Alzheimer's compared to someone without the gene.But the biggest shock came when the scientists matched the brain with the identity of the nun.What they found was that the nun, at the time of her death, had as quick and agile a mind as any nun of her age group -- 80s -- in the study.

During a mental capacity test not long before her death, the nun from the School Sisters of Notre Dame not only quickly identified the season, month and exact date, but within several minutes correctly estimated the time of day, although she had no access to a watch or clock."At first we didn't think we had the right brain," said David A. Snowdon, director of the Sanders-Brown Center on Aging in Lexington, Ky. But checking confirmed the identity.

The nun had an "Alzheimer's brain" but not a single symptom of Alzheimer's disease. At about the same time, the scientists also were examining the brain from another nun of similar age. She, too, had tangles and plaques but not nearly as many as the other nun. She also had only one copy of the APOE4 gene, not two. But when this second nun had been given the memory test shortly before she died, she was unable to answer any of the questions correctly and she couldn't even remember she was being tested.

The explanation? The second nun previously had suffered two strokes."It was a double whammy," said Snowdon. Actually more than a double whammy. What the two cases showed to researchers is that Alzheimer's is a disease with many contributing factors. And when just two -- strokes and the brain abnormalities in the second case -- combine, the result can be synergistic."It is not one plus one equals two," Snowdon said. "It is more like one plus one equals six or seven."

The factors that may contribute to Alzheimer's, hastening its onset and determining how serious it will be, are the goals of the now-famous Nun Study, of which Snowdon is the primary investigator.The study is examining the lives of 678 nuns, based on autobiographies they wrote when they entered the order at age 19; observations and tests in later years; and examination of their brain tissue after death. All the nuns are from the School Sisters of Notre Dame, an order founded in Munich that sent its first sisters to the United States in 1847 and set up its first U.S. motherhouse in Milwaukee in 1850....

Head injuries at some point in life also have been shown to increase the risk of dementia later on, as do depression and exposure to heavy metals.The point: Snowdon maintains that Alzheimer's is less a consequence of simple aging as it is of progression of a disease process."Whether you are going to get it is a long chain of events that can include lifestyle, diet, smoking, adequate medical care. If you layer all that on top of an Alzheimer's brain, we think that can trigger the symptoms," he said. "Keeping it at bay is really the issue."Which means, in his view, that Alzheimer's can be prevented in many cases.It will be up to the Nun Study, work at Marshfield and similar studies to tease out exactly what can be done to reduce the risk.

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