MAD COW DISEASE IS OUT THERE!    STAY INFORMED!

( Bovine Spongiform Encephalopathy - BSE )

20 Dec 00 - CJD - BSE meat from foreign cattle may be ending up in burgers

By David Brown, Agriculture Editor

Telegraph... Wednesday 20 December 2000

A legal loophole still leaves the British public at risk from BSE tainted French beef.

Prof Harriet Kimbell, a member of the Spongiform Encephalopathies Advisory Committee (SEAC) and senior government adviser, disclosed that beef from cattle more than 30 months old could be imported from France, Germany, the Republic of Ireland and other countries affected by Mad Cow disease and processed into products sold in this country.

She suggested that the committee had been misled into thinking that consumer safeguards in Britain were stronger than they are. Until now, the Government has restricted its warnings to the possibility of risks from meat products manufactured abroad and from rogue traders.

She said: "The regulations are not as we thought they were. It is highly likely that meat from cattle over 30 months old is being sold in this country ." It is illegal in Britain to use any beef from cattle over 30 months of age for human consumption. Until yesterday it was generally understood that all meat from older cattle could only be brought into Britain if it was destined for re-export.

Prof Kimbell said that she and other members of SEAC were surprised to discover that the ruling applied only to fresh meat. Manufacturers could import beef and process it, after which it was legal to sell it here. So far there are 88 known and probable cases of new variant Creutzfeldt-Jakob disease (vCJD) in Britain. Five victims are still alive. Four cases are know to have occurred abroad - one in Ireland and three in France.

All the major supermarkets have denied that they sell French beef or products made from it but about 5,000 tons a year are imported here. Thousands of tons of Irish beef are also imported.

The Food Standards Agency, the body responsible for protecting the public from BSE and other food-borne dangers, admitted last night that it has known about this loophole for some time . It has so far advised the Government that there is no need to ban imports of French beef to protect consumers.

The Tories immediately demanded a statement from Nick Brown, Minister of Agriculture, who was at a meeting of EU farm ministers in Brussels last night, and from the Food Standards Agency.

Tim Yeo, shadow minister of agriculture, said: "This is a very serious disclosure. It is ridiculous that we have rules in the United Kingdom to destroy all cattle more than 30 months old to protect people from BSE and then import meat from the same category of cattle which is then sold to our consumers."

A spokesman for the Ministry of Agriculture said: "This is a matter for the Food Standards Agency."

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Mon, Dec 11, 2000 • AP  • WorldStream

About 2 out of 1,000 "at-risk" cows actually have mad cow disease

About two out of 1,000 French-raised cows considered to be at risk for mad cow disease are infected with the fatal brain-wasting ailment, according to a government study published Monday.

The national food safety agency, known by its French initials AFSSA, launched a broader screening program in August and has since carried out 15,000 tests on animals considered to have a higher-than-average risk for the disease: cows that died naturally, and cows that had to be killed after an illness or accident.

On Monday, AFSSA said it had discovered 32 cases of mad cow disease since launching the program, in which the cow's brain is probed for traces of an infectious protein, prion.

"We're not surprised,"Didier Calavas, a member of AFFSA's epidemiology unit, said at a news conference. "The results line up with our expectations."

In 1999, the agency had forecast that it would discover up to 3 cases per 1000 tested in the new screening program. Animals born in or after 1996 could not be studied, as it takes time for prion to develop in the brain. In the study, AFSSA discovered that cows killed after being injured in accidents had a slightly higher level of risk than the others screened, with 3 out of 1000 infected. The group said it would take steps to pull such animals from the food chain.

The final results of the study are to be published in spring 2001. France has been hit by a new wave of mad cow panic since it was discovered in October that potentially infected beef wound up on supermarket shelves. The meat was quickly pulled, but consumer confidence was shaken.

Since then, many school cafeterias have taken beef off their menus, and the government has taken stringent measures to wipe out risks, including banned several specialties such as the T-bone steak. At the same time, more cases of mad cow disease have been reported here this year after the government began screening a wider sample of cows. In total, about 125 cases have been discovered this year, compared to 31 cases in 1999.

Experts believe eating infected meat can cause people to contract the brain-wasting ailment Creutzfeldt-Jakob Disease. Two people have died of the disease in France, compared to about 87 in Britain, where the disease was identified in 1995.

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Spiroplasma proposed as cause of Mad Cow Disease

Organophospates and BSE: Mark Purdy

Theories of Mad Cow Disease

JAMA August 14, 1996 - DC Capital Conference spring 1996

A dissenting view on the cause of mad cow disease came from Frank O. Bastian, MD, profesor of pathology at the University of South Alabama College of Medicine, Mobile, who outlined his theory that mad cow disease is caused by a conventional microorganism, a spiroplasma. Bastian spoke at a symposium held by the National Foundation for Infectious Diseases.

Bastian regards the prion theory as a red herring. The cause of transmissible spongiform encephalopathies (TSEs), he says, is a conventional microorganism--a mollicute or, more specifically, a spiroplasma. "The infection-related protein is produced by the host in response to the infection," he says. He bases his conclusion on 3 premises:

1. Twenty years of his research indicates a role for spiroplasma. The evidence includes the following: spiroplasma-like inclusions were seen in brain biopsies from patients with CJD (Arch Pathol Lab Med. 1979;103:665-669); spiroplasma internal fibril proteins are identical morphologically to those seen in TSEs; the spiroplasma proteins show immunological cross reactivity with the TSE proteins (J Clin Biol. 1987;25:2430-2431); and spiroplasma, when inoculated into rodents, produces a similar neuropathology (Amer J Pathol. 1984;114:496-514).

Spiroplasmas, discovered in 1976, do not have a cell wall; they blend with the host's cell membranes. A labeling marker would help identify them, but developing one is difficult because spiroplasmas are hard to cultivate. No more than half the known strains are culturable, says Bastian.

2. The PrP in these TSEs is a product of the infection. "It is a modified host protein and may play a role in the pathogenesis of the disease, but it is not the agent because there are numerous reports that the protein can be separated from infectivity. Also, it is found in inclusion body myositis, a condition unrelated to CJD," says Bastian.

3. The immune system is involved. The gene for the host protein is located on the chromosome in the region of the major histocompatibility complex (MHC) in the mouse. "Occasionally, you see elevation of immunoglobulins; there are morphological alterations of the leukocytes; there is leukopenia," Bastian explains, "and autoantibodies are characteristically seen in the late stages of both experimental and naturally occurring infection. There is partial MHC restriction in both human and animal disease."

The immune reaction seen in these spongiform diseases can be explained by superantigen activity, Bastian says. He notes that, normally, an antigen is presented to the cell surface in the MHC and interacts with the T-cell receptor--the antigen lying in a groove in the T-cell-MHC sets in motion the standard reaction. A superantigen, on the other hand, binds outside the groove of the T-cell and interacts with the MHC. This results in some immunoglobulin production, but only transiently. The major effect is clonal deletion of T cells, resulting in a state of immune tolerance. Autoantibodies can also form.

Bastian proposes that, in spongiform diseases, PrP presumably acts as a superantigen. The question, he says, is what is producing the superantigen. He proposes that it is a bacterium. He says he is especially excited by the fact that the mollicutes cause superantigen production, adding that it is noteworthy that inclusion body myositis, a condition in which prions are seen, is an established superantigen disease.

Bastian is concerned because of recent reports that disease has been transmitted to macaques given BSE-containing contaminated material (Nature. 1996;381:743-744). The pathology in the monkeys is identical to that seen in the recent human cases of CJD in Great Britain and appears to confirm that these cases were the result of transmission to humans of BSE, Bastian says.

He also notes that investigators have just reported transmitting scrapie to mice from hay mites gathered from farms in Iceland where scrapie is endemic (Lancet. 1996;347:1114). Bastian says he is virtually certain that these hay mites contain spiroplasma, noting that the investigators have not so far found PrP in the mites.

"Clearly, the agent has become so virulent that it can easily jump species barriers. This makes it very dangerous," says Bastian, "and shows the need to intensify research in this area."

To control CJD, Bastian said the first step is to stop feeding offal to cattle and second, "we must develop a preclinical method of detecting the causative agent."

The key to accomplishing this is looking for a conventional invasive organism, rather than a host protein, in these infected tissues, Bastian said. "Once we have developed a method of detecting the organism, we will be in a position to do surveillance studies, find out where the disease is, and deal with any potential problems that might arise." He added, "Perhaps success in this area will also allow us to eradicate scrapie, which we've known about for 200 years."

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Mark Purdey: Organophosphate theory of BSE

Purdey, Mark

Med-Hypotheses. 1996 May; 46(5): 445-54

This paper elucidates the flaws in the official hypothesis that bovine spongioform encephalopathy originated from alterations in the way that scrapie-contaminated cattlefeeds were manufactured in the UK. An alternative hypothesis is proposed that cites exposure of the bovine embryo to various specific high-dose lipophilic formulations of organophosphates, such as the high-dose phthalimide containing organophosphate phosmet, (which were applied compulsorily and exclusively in the UK during the 1980s/early 1990s) as the primary trigger that initiated the deformation of prion protein and the onset of the bovine spongioform encephalopathy epidemic. The multi-site binding metabolites of these organophosphates penetrate the fetus, covalently phosphorylaing various active sites on fetal prion protein.

The extra charged phosphate groups left on aged prion protein blocks both proteases and chaperones from accessing their catalytic/bonding sites, creating the undergradable, misfolded isoform of prion protein, PrPsc. The resulting abnormally phosphorylated PrPsc aggregates to freshly synthesized PrPc, transforming it into same; due to a system of positive feedback invoked by the organophosphate-induced blockage of a prion protein-specific protein kinase. Both the timing, distribution and dynamics of usage of these specific organophosphates correlates with the epidemiology of bovine spongioform encephalopathy as well as accounting for the 23,000 cattle that have developed the disease, yet were born after the 1988 ban on scrapie-contaminated cattlefeed.

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Friday December 22 11:58 AM ET

UN Health Agency Sees Global Mad Cow Risk

By Stephanie Nebehay GENEVA (Reuters) - The World Health Organization (WHO) on Friday expressed concern about what it called ``exposure worldwide'' to mad cow disease and its fatal human form, new variant Creutzfeldt-Jakob Disease (vCJD).

The United Nations health agency said it would convene a major meeting of experts and officials from all regions on the neuro-degenerative diseases striking cattle and humans. It will be held in Geneva in late spring, probably in May.

WHO officials spoke after an informal meeting of experts reviewed scientific evidence on a variety of issues amid growing consumer concern in countries including Germany and Canada. Experts' concerns center on British meat and bone meal exports in the 10-year period between 1986, when bovine spongiform encephalopathy (BSE ) surfaced in Britain, and 1996, when an export ban was imposed on British beef. There are also wider concerns about European Union exports.

``Our concern is that there was sufficient international trade in meat and bone meal and live cattle that there actually has been exposure worldwide already,'' Dr. Maura Ricketts, of WHO's animal and food-related public health risks division, told a news conference.

BRITAIN'S TOLL:

180,000 BSE, 87 vCJD CASES Since 1986, 180,000 BSE cases have been confirmed in British cattle, with 1,300 to 1,400 cases elsewhere in Europe -- all but several dozen cases in four countries (France, Ireland, Portugal and Switzerland), according to WHO. Small numbers of cases have been reported in Canada, Argentina, Italy and Oman, but in each of these countries this was only in imported British bovine, it added. In all, 87 cases of vCJD have been reported in Britain, three in France and one in Ireland, according to the agency. ``We know potentially contaminated materials were exported outside the European Community...We are trying to identify the countries that we should put our largest effort into,'' Ricketts said. ``The only way to know whether or not different countries are at risk is to ask them...These countries themselves have the information that is required to determine if they are at risk. ``We are concerned some countries which received materials do not have surveillance systems to detect the disease in animals or the human population,'' she added.

``Countries of the world need to be developing surveillance systems for these diseases.'' But Ricketts, a Canadian, conceded it would be difficult to trace exported beef and meat products, often repackaged or transformed before being re-exported with new labeling. ``It become very difficult, the trail grows cold,'' she said.

Extra Vigilance On Rendered Materials

Experts reviewed issues including: slaughterhouse practices; ''chronic low dose exposure'' of humans to BSE; mechanically-recovered meat which may contain infected nervous tissue; exposure of sheep and pigs to BSE; testing; and meat and bone meal. ``We thought we had to review how feed moves around the world because of the importance of cattle feed in the transmission of BSE,'' Ricketts said. ``We felt we had to review these tissues that are called 'specified risk materials' and include brains, eyes, the spinal column, parts of the gut content...to find how these materials are being sold for human consumption.'' ``We are very interested in the movement of 'rendered' materials around the world since it is quite possible that rendered materials contain infectivity,'' she added.

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By Leroy Sievers

Coming up on Nightline Swearing Off Beef

W A S H I N G T O N, Dec. 26 — Turkey. Roast turkey. Turkey sandwiches. Turkey casserole. Turkey surprise. It probably hasn't happened quite yet, but I guarantee that within the next few days, all of us will start to get really sick of turkey.

Now, I love turkey sandwiches after the holidays, but even those start to be too much after a week or so. And all the while, the remains of the turkey sit in the refrigerator, silently challenging even the most creative cook to come up with new ways of disguising the leftovers. Because, let's face it, for all the chicken we eat, all of "the other white meat," fish, vegetable burgers, and all of that, this is still a beef-eating country.

Beef just seems more American. I don't remember the movie cowboys that were my boyhood heroes sitting down around the campfire to a hearty meal of free-range chicken. They ate beef. And lots of it. Now, I really don't eat a lot of steaks, not very much roast beef, but I do love the occasional double cheeseburger. With fries, but that's a whole different issue. My guess is that Americans eat less beef than they used to, I think chicken may be No. 1, but think about what it would be like if we were all afraid to eat beef.

What if eating a cheeseburger was as dangerous as eating the poisonous blowfish that is a delicacy in Japan, but that prepared incorrectly can lead to death? That's sort of the fear sweeping through Europe right now. Mad cow disease attacks the nervous systems of cows, and has apparently spread into humans. The initial medical opinion was that people got the disease by eating the brains, or perhaps bone marrow, of infected cows.

That seems easy enough to avoid. Mad cow disease or not, facing a world where it was not safe to eat brains does not make me lie awake at night. But there is enough uncertainty, enough fear, that people all over Europe are swearing off beef. Now that would seem to be good news for American ranchers. Mad cow disease has not appeared in North America, so you would think that the market for U.S. beef would be huge.

But the fear in Europe continues. I think people are probably suspicious, it would be easy to just say that the meat they were being served came from the U.S, when it fact it didn't. And in all seriousness, the disease, when contracted by people, is truly devastating. It's fatal to the person that has it, and like all diseases that strike the unsuspecting, it's devastating to their families as well.

This has been one of those stories that has been hovering around our attention for a while. One of those stories you scan over in the newspaper, without really paying attention. Well, it really is front-page news overseas, so we figured we should take a look at it tonight. I will warn you that some of the images are disturbing, but this is a story that will just keep building. The hope is that the disease can be kept out of this country, but if it ever does make it here, our lives, or at least our diets, will change forever. We'll need to come up with a lot more ways to cook turkey.

Leroy Sievers is executive producer of Nightline.

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Has the United States imported cattle from the United Kingdom?

Yes. Between 1981 and 1989, 496 cattle were imported from the United Kingdom and the Republic of Ireland. These U.K. imports have been traced, and there are only 4 cattle still alive in the United States (as of February 1999). All of these animals have been under quarantine since April 1996. APHIS is currently attempting to purchase these cattle for diagnostic research purposes. In July 1989, the importation of live ruminants from the United Kingdom was banned.

In addition, 2 head of cattle imported from Belgium in 1996 are now under quarantine. APHIS, in cooperation with the States and industry, continues to purchase these animals for diagnostic purposes. No evidence of BSE has been found in any of these imported animals.

Can we account for all of the U.K.-imported cattle?

All but 32 animals have been traced. All cattle of unknown status would be greater than 10 years of age and would have a reduced likelihood of developing BSE at this late date.

STATEMENT OF CRAIG REED, ADMINISTRATOR, USDA'S ANIMAL AND PLANT HEALTH INSPECTION SERVICE

December 19, 2000

"As of December 7, 2000, the U.S. Department of Agriculture has prohibited all imports of rendered animal protein products, regardless of species, from Europe. This decision followed the recent determination by the European Union that feed of non-ruminant origin was potentially cross-contaminated with the bovine spongiform encephalopathy (BSE) agent. The restriction applies to products originating, rendered, processed or otherwise associated with European products. USDA is taking this emergency action to prevent potentially cross-contaminated products entering the United States. The same type of rendered product from ruminant origin has been prohibited from BSE infected countries since 1989 and the entirety of Europe since 1997. "USDA bases all decisions regarding imports on sound science and extensive research. Although no case of BSE has ever been diagnosed in this country, we continue to take aggressive measures to protect the United States from BSE introduction and to ensure the security of U.S. export markets. These measures include active surveillance, testing, prevention, education, and emergency preparation. Since 1989, the United States has banned the import of live ruminants (cattle, sheep, goats) and most ruminant products from countries where BSE has been reported. In 1998, USDA entered into a cooperative agreement with Harvard University's School of Public Health to analyze and evaluate the Department's efforts to prevent BSE. A report is expected to be issued early next year."

Sources:

http://www.aphis.usda.gov/oa/bse/

http://www.aphis.usda.gov/lpa/press/2000/12/reed.12.19.txt