Prions
Mad Cow Home ... Best Links ... Search this site

Coastguard officer is 25th BSE victim
Mad cowboy: the rancher who won't eat meat, new book by Howard Lyman
The Trembling Mountain: Kuru, Cannibals and Mad Cow Disease, by Robert Klitzman, M.D
Mad cows and Milkgate, by Virgil Hulse, M.D.
Mad Cow U.S.A. : Could the Nightmare Happen Here? gets 5 stars at amazon.com
Chronology of mad cow disease (BSE)
Prusiner at Inquiry
EU vets examine British beef plan, postpone vote
British farmers demand active moves for beef ...


Coastguard officer is 25th BSE victim

Thu, Jun 18, 1998 PA News
A Coastguard officer has become Britain's 25th victim of the human version of "mad cow" disease, it was confirmed today.

Tony Barrett, 45, from Brixham, south Devon, died of Creutzfeldt-Jakob Disease, which has been linked to BSE-infected cattle. His devastated widow Sandra, said: "I just hope the current beef ban won't be lifted until it's totally safe."

Mrs Barrett is determined that her husband's death about 10 days ago should be seen as a warning to other families and as a message to the Government and food suppliers. She says she will be willing to give evidence about his illness to the current inquiry into the BSE crisis and has attacked scientists and food producers who are trying to tamper with natural products.

Mr Barrett's death certificate confirms new variant CJD as the cause of death, but how he came to contract it is a mystery. The 17-stone, 6ft 2ins family man had a normal, healthy mixed diet, said his widow. "This really brings the dangers home," she said.

At the end, Mr Barrett, who dedicated his life to saving others in the busy search and rescue service, could not recognise his wife and two children. "He was just so frightened. I don't want anyone else - any other family - to go through the same torture.

"I can't think of anything worse than watching the rapid deterioration of the husband and father you love, losing all his faculties, all because someone wants to make a profit out of cheap cattle food."

She added: "I don't want to frighten people. The early symptoms could be a variety of illnesses. Tony was treated for multiple sclerosis for a long time. Then he went for psychiatric tests during a long period of mis-diagnosis.

"Then the consultant broke the news it was new variant CJD - the first they had seen in he West County. "We were numb. There's no cure, but at least it had a name." Daughter Paula, 23, said: "I was always moaning at Dad for being so loud and cheerful first thing in the morning.

"Now I'd give anything to have him cracking jokes and being jolly at 7am. We'll never get over the horrible way he died."

The Trembling Mountain: A Personal Account of Kuru, Cannibals and Mad Cow Disease [Plenum, 1998]

Robert Klitzman, M.D.
Assistant Professor
College of Physicians and Surgeons
Columbia University
Several reviews are posted at amazon.com under the book's page. Discover Magazine also reviewed the book in its July issue. The Associated Press is also now carrying a review. The book sells for $19.57 in hard cover.

"The key word here is personal. Physician Robert Klitzman tells us his life story and humanizes what could easily have been a tabloid-size horror story of Stone Age cannibals and rotten-brained cows. Vivid portraits of the men and women he helped and worked with lift this book above mere sensationalism, showing one people's tragedy in the hopes that others can be averted.

Kuru is a fatal disease formerly epidemic among the Fore people of New Guinea, with symptoms including involuntary laughing, dementia, and loss of motor control. Traced to their ritual cannibalism, it was found to be caused by nonliving crystal-like proteins in the brain. Klitzman traveled to New Guinea before attending medical school to work with these people and quickly learned how little Western medicine could do for the afflicted--he could only make their deaths as comfortable as possible. His despair is palpable.

Fortunately, most Fore have been convinced to give up the most dangerous of their ancestral practices, and the disease has largely abated. But mad cow disease (and others like it), caused by the same class of protein as kuru, remains a threat to Westerners--a threat Klitzman would rather we not face. His very personal story forces us as readers to examine our own lives and our own ancestral practices, perhaps to make some changes ourselves." --Rob Lightner

From Booklist , 05/15/98

"Psychiatrist Klitzman's early association with D. Carleton Gajdusek, who won the Nobel Prize for his work on kuru and Creutzfeldt-Jakob disease, aka mad cow disease, led to a year in Papua New Guinea studying kuru. Klitzman's work there produced valuable epidemiological findings on transmission and onset. To accomplish it, he slogged through mud, strained up and down hills, and pushed through jungles. His descriptions of conversations with patients, their family members, and elders illustrate their fears of spirits and magic as well as his own frustrations with his guides, who were as greedy and untruthful as some people he had known in the States."

"His presentation of other researchers, with their respective quirks, and of missionaries, all of whom were sure of their own sect's rightness and other sects' wrongness, is also memorable. Out of his experience, Klitzman learned to rely on himself and decided to go to medical school. Striking a much more somber note are his highly pessimistic observations about the kuru-like and increasingly prevalent mad cow disease." William Beatty

Mad Cows and Milkgate

3 June 98 Virgil M Hulse M.D.
fax: 541 488 5368; phone 541 482 2048
ISBN: 0-9654377-0-1 $20.00  338pp.
Hulse's background is preventative medicine, public health, and food science. He has followed BSE and other issues involving human health and modern agriculture for many years. It has a chapter on the Oprah lawsuit that he attended for 10 days as expert witness and is quite up-to-date on this and other aspects of spongiform encephalopathies present in the United States.

There are other chapters, each scary in its own way, on bovine AIDS and bovine leukemia virus, bovine growth hormone, Crohn's disease, E. coli, and Salmonella. The prose is clear and non-technical; the tone is moderate. Covers more bovine-to-human diseases than excellent books such as Mad Cow USA by Stauber and Shelton. Hulse raises some interesting questions to which no one has satisfactory answers. The book would make for a good web site. Recommended reading for non-specialists.

New Howard Lyman book to appear

MAD COWBOY Plain Truth from the Cattle Rancher Who Won't Eat Meat 
By Howard F. Lyman With Glen Merzer 
Publication Date: June 3, 1998
Price: $23.00/ Pages: 223
ISBN: 0-684-84516-4
A detailed description of the book is available online along with excerpts.There is a separate page with background information on the First Amendment vs veggie libel laws and information about the live online chat with Howard Lyman on 2 June 98.

The book may be easily purchased online.

Mad Cow U.S.A. : Could the Nightmare Happen Here?

by Sheldon Rampton, John C. Stauber
 Price: $17.47You Save: $7.48 
Common Courage Press; ASIN: 1567511112
Review at amazon.com:

"Mad Cow U.S.A. is not the book to read before you go out for a steak. In fact, it's not really a book to read before eating anything; this chronicle of government cave-in to pressure from the food industry just might scare away your appetite.

Authors Sheldon Rampton and John Stauber argue that both the American and British governments colluded with beef producers to suppress important facts about interspecies transmission of bovine spongiform encephalopathy, or "mad cow disease"--facts that might have prevented gruesome deaths.

Could a British-style BSE epidemic happen in America? In a 1996 TV talk show, Oprah Winfrey attempted to ask the same question, only to find herself slapped with a lawsuit by a group of Texas cattlemen. Their grounds: the so-called agricultural product disparagement laws currently on the books in 13 states; these laws prohibit people from questioning the safety of any agricultural product, shifting the legal burden of proof from the food industry to its watchdogs.

What happens when anyone who speaks out about problems with our food supply can be sued into silence? Rampton and Stauber fear grave consequences for public health, and they make a convincing case against these laws--and, inadvertently, for vegetarianism."

Fatal Protein: The Story of CJD, BSE and Other Prion Diseases

 by R M Ridley & H F Baker 
 Reviewed by Adriano Aguzzi in Nature 27 May 98 321 

Chronology of mad cow disease (BSE)

Reuters Friday, June 12, 1998
The European Commission on Wednesday proposed lifting a ban on mainland British beef exports more than two years after it was imposed over mad cow disease. Here is a chronology of related scientific and political events beginning with the discovery in Britain of a new cattle disease more than a decade ago:

NOVEMBER 1986 - Mad cow disease or BSE (bovine spongiform encephalopathy) first identified by British government's Central Veterinary Laboratory.

JUNE 1987 - Ministers informed of new cattle disease. Not known at this stage if it was transmissable.

DECEMBER 1987 - Scientific tests conclude meat and bone meal derived from ruminants was the only viable cause of BSE.

JUNE 1988 - Britain announces ban on sale of certain animal feed to ruminants.

JULY 1988 - Britain announces introduction of a slaughter policy.

JULY 1989 - EU bans export of British cattle born before July 18, 1988 and offspring of infected or suspect animals.

NOVEMBER 1989 - Britain bans use of certain specified bovine offals (SBO) for human consumption.

DECEMBER 1989 - Government extends ruminant animal feed ban, original time limit is removed.

APRIL 1990 - Establishment of the Edinburgh-based Spongiform Encephalopathy Advisory Committee (SEAC) to spearhead research.

MAY 1990 - A cat contracts a spongiform encephalopathy.

MARCH 1991 - First case of BSE in offspring born after the 1989 ruminant feed ban.

JULY 1993 - 100,000th confirmed case of BSE in Britain.

JULY 1994 - European Commission puts restrictions on British exports of beef- on-the-bone - they must only come from cattle certified to be from holdings BSE-free for at least six years.

NOVEMBER 1995 - On advice from SEAC, British government suspends use of bovine vertebral columns in the manufacture of mechanically recovered meat.

MARCH 20, 1996 - SEAC says special unit looking into human Creutzfeldt-Jakob Disease (CJD), identifies previously unrecognised and consistent disease pattern. SEAC says the most likely explanation is a link to exposure to BSE before the SBO ban in 1989. Government says it will consult on further control measures on deboning of carcasses.

MARCH 27, 1996 - EU imposes ban on exports of beef from Britain. Bans export of live bovine animals, semen, embryos, meat of bovine animals; products from bovines liable to enter animal or human food chain, and materials destined for use in medicinal products, cosmetics and pharmaceuticals, and mammalian- derived meat and bone meal.

MAY 1996 - British government announces ``policy of non-cooperation'' with EU. Prime Minister John Major establishes crisis committee to co-ordinate strategy of paralysing EU business until the ban is lifted.

JUNE 1996 - Framework for lifting the export ban agreed by EU heads of state at Florence summit. Britain ends non-cooperation policy.

AUGUST 1996 - Ban on feeding animals meat and bone meal becomes fully effective.

DECEMBER 1996 - Britain publishes Export Certified Herds Scheme (ECHS), which relies on documentation proving an animal was from a herd free of BSE. Also announces selective cull of cattle most at risk from BSE.

JULY, AUGUST 1997 - Illegal shipments of British beef found in various EU countries. Germany leads call for tighter British controls on exports.

SEPTEMBER 1997 - European Court of Justice Advocate General upholds the legality of European Commission's decision to impose the worldwide beef export ban on Britain.

OCTOBER 1997 - Britain formally proposes Date-Based Scheme, which covers mainland Britain and is applicable to meat from animals born after August 1, 1996.

NOVEMBER 1997 - EU scientists give favourable opinion on revised ECHS, stressing cattle database as a positive factor, effectively limiting the scheme's scope to Northern Ireland.

JANUARY 1, 1998 - Britain bans sale and import of all beef-on-the-bone after fresh scientific evidence from SEAC shows small chance of BSE transmitted in nerve tissue, including spinal column and bone marrow.

FEBRUARY 1998 - EU scientists raise few problems over the Date-Based Scheme, but some question whether a calf's mother should be kept alive for six months after the calf goes to slaughter. This would reduce the risk of maternal transmission.

MARCH 16 1998 - EU farm ministers approve the ECHS, meaning Northern Ireland ban can be lifted.

JUNE 1 1998 - European Commission sets June 1 date for the first legal exports of beef from Northern Ireland.

JUNE 10 1998 - European Commission proposes to lift the ban for the rest of Britain under the Date-Based Export Scheme, which permits exports of meat from animals born after August 1 1996 - the date meat and bone meal ban became fully effective.

Prusiner at Inquiry

New Statesman 12 June 1998 FOOD Emily Green
He was regarded as a guest of such import, the enquiry covened on Saturday to hear him.

Using a paper napkin, in fastidious circular motions, Professor Stanley B Prusinerwiped the condensation from the top of a Diet oke can. He reached for a glass, but thought better of pouring. This was not a time to dice with effervescence. He was the focus of some 60 or 70 pairs of eyes. He had been flown into London last week by the British government to give evidence before the inquiry into BSE. Indeed, the neurologist from the University of California at San francisco was regarded as a guest of such import, the inquiry team convened on a Saturday to hear him.

Britain had not always been so accommodating. During the late eighties, the Ministry of Agriculture had refused to provide Prusiner with BSE cow-brain samples. Later, it had turned down several of his grant applications. Yet while the UK sank deeper into the mire of BSE, he went on to receive a series of prestigious awards, culminating last year with the Nobel Prize for Medicine. An obvious question before the inquiry is: did scepticism over Prusiner's line of science contribute to the BSE disaster?

Prusiner contends that BSE, CJD and a host of similar fatal neurological disorders are caused by rogue proteins, which in 1982 he named prions. These, he speculates, are unlike other known disease agents, such as viruses, in that prions propagate without a disease-specific nucleic acid DNA or RNA. Rather, prions are a normal mammalian protein which, in a complicated chemical translation, sometimes corrupt. This might happen through genetic predisposition, spntaneous freak or contact with alien prions. Once corruption is initiated, a domino effect results in fatal neuro-degeneration.

Sixteen years on, the prion hypothesis remains unproven. Awkwardly for Prusiner's hosts at the inquiry, the most convincing evidence against it had come before them three days earlier from Dr Moira Bruce, a biologist at the Neuropathogenesis Unit in Edinburgh.

For the last 30 years, she has strain-typed what Prusiner calls "prion diseases" in sheep by taking homogenate from affected animals and passaging it through experimental mice. This identified 20 different strains, each with distinct incubation times and brain lesion profiles. Working with BSE Bruce has found startling matches using homogenate taken from cows, antelopes, cats, sheep, pigs and, eventually, humans. These matches amount to the BSE "fingerprint" that finally convinced the government and scientific community that BSE and nv-CJD are caused by the same agent.

That there is a distinct BSE "fingerprint" suggests that the BSE agent has a disease-specific genetic component. In other words, that it is not a prion.

Last Saturday, when asked about Bruce's results, Prusiner was in a quandary. If he suggested that they were unreliable, it would cast doubt on the association between British bovine offal and Creutzfeld-Jacob disease. If he credited them, he could undermine the prion. In the event he concluded: "I do not know quite what it means at this point."

The following day, the _Independent on Sunday_ carried the headline, "Nobel winner challenges CJD link to beef"; in the _Observer_ it was, "Tories blundered over BSE research". As for which scientist is right, time will tell.

Presently, we know only for whom the civil service sits on a Saturday.

Transcipts of Professor Prusiner's and Dr. Bruce's appearance are online.

[The reporter has an agenda here: Prusiner's protein-only model is actually completely compatible with strain types. Reading the transcript, Prusiner's expressed concern actually centered on why these 25 succumbed and not other young people with far worse exposures and the same genetics at codon 129. (Earlier testimony had pooh-poohed regional clustering and water supply.) Without understanding this critical issue, it is impossible to understand transmission, much less estimate the scope of the epidemic.

The real question that should have been put to Prusiner is why no one has sequenced the promoter regions of the nvCJD victims to see if they are over-producers. My view is that the English have indeed done this long ago and are not releasing the results. -- webmaster]

EU vets examine British beef plan, postpone vote

Fri, Jun 12, 1998 Reuters Financial Report
BRUSSELS - European Union veterinary experts took a first look on Friday at a proposal to lift the ban on British beef exports, but postponed a decision until at least next week, EU officials said. On Wednesday, the European Commission proposed easing the worldwide embargo on British beef, imposed more than two years ago at the height of the mad cow disease crisis.

"The Commission presented the scheme and (EU) member states asked their initial questions," an EU official told Reuters. "They said it was too early to give their opinions on it."

The plan, known as the Date-Based Export Scheme, would allow a resumption in shipments of meat from animals born after August 1996 -- the date when a ban on feeding meat and bone meal to cattle became fully effective. Chief veterinary officers from the 15 EU states are expected to give the plan a second reading next Thursday, although a vote then cannot be assured.

Such is the proposal's political sensitivity that the vets are likely to pass a final decision to EU farm ministers. There is a small chance that the proposal will be discussed by EU farm ministers at a meeting starting on June 22, officials said.

Britain will have to convince its more sceptical EU partners, such as Germany, that strict controls are in place to prevent any non-eligible meat >from reaching their markets. And British farmers will have a hard fight ahead of them -- not only will they have to gain the trust of continental consumers, but the pound's recent strength has left them almost priced out of foreign markets.

EU ministers have already approved a separate scheme -- the Export Certified Herds Scheme -- for Northern Irish beef. The province has an extensive database, which can track cattle movements and ensure animals are >from herds free of BSE (bovine spongiform encephalopathy) or mad cow disease. The EU imposed the ban on British beef exports in March 1996 after the government in London admitted a possible link between BSE and a new form of the human brain-wasting disorder Creutzfeldt-Jakob Disease.

British farmers demand active moves for beef ...

COMTEX Newswire Sun, Jun 14, 1998
LONDON, June 14 (Itar-Tass) - Thousands of British farmers gathered for a rally and a march in Cardiff on Sunday to urge more active moves of the British government for the lift of the international embargo on the British beef exports. The embargo was introduced by the European Union over two years ago for fear of the mad cow disease spread. The farmers chose Cardiff as the location of their protests because the town would host a two-day EU summit on Monday.

Head of the National Farmer Union Ben Jill said at the rally the summit would give the British government a perfect chance to press on the EU partners for the embargo lift. The embargo had inflicted considerable losses on the British farmers and resulted in the large-scale slaughter of cows aging over 30 months, which were potential sources of the disease.

The beef exports embargo had been recently lifted for Northern Ireland. However, it remained valid for other areas of the United Kingdom. In the words of Jill, there are no scientific grounds for keeping the embargo and farmers no longer want to tolerate delays, which have political reasons. [This amazingly ignores recent test results from Switzerland described in a British journal - webmaster]



Mad Cow Home ... Best Links ... Search this site