Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal, neurodegenerative disease of cattle, which infects by a mechanism that surprised biologists on its discovery in late 20th century. While never having killed cattle on a scale comparable to other dreaded livestock diseases, BSE has attracted wide attention because it seems that people can contract the disease as well. It is thought to be the cause of variant Creutzfeldt-Jakob disease (vCJD), sometimes called new variant Creutzfeldt-Jakob disease (nvCJD), which is a human brain-wasting disease.

Infectious agent

Unlike the other kinds of infectious disease which are spread by microbes, the infectious agent in BSE is a specific type of protein. Mutated prion proteins can carry the disease between individuals and cause deterioration of brain tissue. BSE is a type of transmissible spongiform encephalopathy (TSE) that can arise in animals carrying a rare mutant prion allele, which expresses prions that contort by themselves into the disease-causing shape. Oddly, most TSEs occur sporadically in animals that do not have a prion protein mutation. Transmission can occur when healthy animals consume tainted tissues from other animals infected with the disease. In the brain, these proteins cause native cellular prion proteins to deform into the infectious state, which then goes on to deform other prion protein in an exponential cascade. These mutated prion proteins aggregate to form dense plaque fibers, which lead to the microscopic appearance of "holes" in the brain, degeneration of physical and mental abilities, and ultimately... death.

Origin: The BSE Epidemic in British cattle

The British BSE cattle epidemic was recognised in 1986. It was first believed to have originated in sheep, in which the related prion disease 'scrapie' is common. However, sheep and cattle TSEs are quite different and it is now thought more likely that BSE could have originated with a case of sporadic BSE in a single bovine. Prior to the BSE epidemic, cattle were fed with meat and bone meal, a high-protein substance obtained from the remnants of butchered animals, including cows and sheep. This practice allowed the accumulation of prions over many generations. As more animals became ill, more infectious tissue got into the feed, and the number of cases reached epidemic proportions. The tissues that contain most of the pathogenic molecules are those of the brain and the nervous system, although infectious amounts have been shown experimentally to be present elsewhere, such as in blood. The use of meat and bone meal as a protein supplement in cattle feed was widespread in Europe prior to about 1987.

Currently, soybean meal is the primary worldwide plant-based protein supplement fed to cattle. However, soybeans do not grow well in Europe, so cattle farmers throughout Europe turned to the less expensive animal byproduct feeds as an alternative. A change to the rendering process in the early 1980s may have resulted in a large increase of the infectious agents in the cattle feed. A contributing factor seems to have been a change in British laws that allowed a lower temperature sterilization of the protein meal. While other European countries like Germany required said animal byproducts to undergo a high temperature steam boiling process, this requirement had been eased in Britain as a measure to keep prices competitive.

Following an epidemic of BSE in Britain, 157 people (as of 2004) acquired and died of a disease with similar neurological symptoms subsequently called vCJD, or (new) variant Creutzfeldt-Jakob disease. This is a separate disease from 'classical' Creutzfeldt-Jakob disease, which is not related to BSE and has been known about since the early 1900s. Of the 157 cases of vCJD in humans so far, 148 occurred in the United Kingdom, 6 in France, and one in Italy. Three cases of vCJD occurred in people who had lived in or visited Britain--one each in Ireland, Canada and the United States. There is also some concern about those who work with (and therefore inhale) cattle meat and bone meal, such as horticulturists, who use it as fertilizer. Up to date statistics on all types of CJD are published by the UK CJD Surveillance Centre in Edinburgh. For many of the vCJD patients, direct evidence exists that they had consumed tainted beef, and this is assumed to be the mechanism by which all affected individuals contracted it. Disease incidence also appears to correlate with slaughtering practices that led to the mixture of nervous system tissue with hamburger and other beef.

It is estimated that 400,000 cattle infected with BSE entered the human food chain in the 1980s. Although the BSE epidemic was eventually brought under control by culling all suspect cattle populations, people are still being diagnosed with vCJD each year (though the number of new cases currently seems to be dropping). This is attributed to the long incubation period for prion diseases, which are typically measured in years or decades. As a result the full extent of the human vCJD outbreak is still not fully known.

In 2004 researchers reported evidence of a second contorted shape of prions in a rare minority of diseased cattle. In other words, this implies a second strain of BSE prion. Very little is known about the shape of disease-causing prions, because their insolubility and tendency to clump thwarts application of the detailed measurement techniques of structural biology. But cruder measures yield a "biochemical signature" by which the newly discovered cattle strain appears different from the familiar one, but similar to the clumped prions in humans with traditional CJD (Creutzfeldt-Jakob Disease).The finding of a second strain of BSE prion raises the possibility that transmission of BSE to humans has been underestimated, because some of the individuals diagnosed with spontaneous or "sporadic" CJD may have actually contracted the disease from tainted beef. So far nothing is known about the relative transmissibility of the two disease strains of BSE prion. In 2005 a controversial paper in The Lancet suggested that BSE might have originated in British cattle when they ate imported animal feed that included infected human remains from Hindu funeral ceremonies in India.

UK epidemic and UK licensed medicines

During the course of the investigation into the BSE epidemic, an enquiry was also made into the activities of the Department of Health and its Medicines Control Agency. On May 7, 1999 in his written statement number 476 to the BSE Inquiry, David Osborne Hagger reported on behalf of the Medicines Control Agency that in a previous enquiry the Agency had been asked to:

"... identify relevant manufacturers and obtain information about the bovine material contained in children’s vaccines, the stocks of these vaccines and how long it would take to switch to other products."

It was further reported that the:

"... use of bovine insulin in a small group of mainly elderly patients was noted and it was recognised that alternative products for this group were not considered satisfactory."

A medicines licensing committee report that same year recommended that:

"... no licensing action is required at present in regard to products produced from bovine material or using prepared bovine brain in nutrient media and sourced from outside the United Kingdom, the Channel Isles and the Republic of Ireland provided that the country of origin is known to be free of BSE, has competent veterinary advisers and is known to practise good animal husbandry."

In 1990 the British Diabetic Association became concerned regarding the safety of bovine insulin and the government licensing agency assured them that:

"... there was no insulin sourced from cattle in the UK or Ireland and that the situation in other countries was being monitored."

In 1991 a European Community Commission:

"... expressed concerns about the possible transmission of the BSE/scrapie agent to man through use of certain cosmetic treatments."

Sources in France reported to the British Medicines Control Agency:

"... that there were some licensed surgical sutures derived from French bovine material."

Concerns were also raised:

"... regarding a possible risk of transmission of the BSE agent in gelatin products."

BSE in North America

As of January 2005, five BSE-infected cattle have been identified in North America. The first was in 1993, involving an animal born in Britain. The second was reported in Canada on May 20, 2003. It occurred in a single older cow that may have contracted the disease from contaminated feed in earlier years. The animal was destroyed after being declared unfit for consumption. The United States also issued a temporary ban on all Canadian beef.

On December 23, 2003, the first case of BSE in the United States was found in a single Holstein cow in Mabton, Washington, although trace-backs later revealed that this cow originated from a Canadian herd. Agriculture Secretary Ann Veneman called the discovery "a clear indication that our surveillance and detection program is working." However, the United States tested only 20,526 cows in 2003 out of the roughly 35 million slaughtered. Current tests reveal the presence of misshaped prions when they are abundant, but it is not known how far the disease must progress in an individual to transmit it to others. Therefore, it is possible that even among those cattle that are tested and classified as negative, a proportion nevertheless may be contagious. As a result, U.S. authorities have very little idea of how many American beef cattle might have the disease.

The government has banned the use of "downer cows" for human consumption. While the Washington cow that tested positive for BSE was reportedly unable to stand, veterinarians say the condition was unrelated to BSE. Furthermore, there is some dispute as to whether the cow was a downer or not. Therefore it is not clear how effective the ban is in reducing the number of infected cattle consumed. Only 200,000 cows slaughtered in 2003 were downers. The meat of the BSE-positive cow went to market, but some of it was successfully recalled before it was sold to consumers. U.S. authorities called for a switch to the testing procedure that is used in the United Kingdom, which yields its results in one day. Until the switch, U.S. surveillance relied on a test that gave results only after two weeks, after which time the meat from an animal usually has all been sold.

Shortly after the U.S. discovery of BSE in 2003, Japan and South Korea instituted temporary bans on the import of U.S. beef until the authorities can be assured of its safety. Since Japan and South Korea are the first- and third-largest importers of US beef, respectively, the economic impact of their bans is significant both for American cattle ranchers and for Japanese and Korean beef consumers. Notwithstanding, Japanese beef exports, chiefly the expensive wagyu, have been banned in the United States since Japan experienced its first case of BSE in January 2001.

Japan lifted its ban on US beef in December 2005. It was, however, quick to reinstate the ban. Brooklyn-based Atlantic Veal & Lamb inspectors failed to notice there was bone material included in a shipment of veal to Japan. Japanese inspectors found material from cattle backbone in three of 41 boxes in a 858-pound shipment of beef from Atlantic Veal & Lamb. No case of variant Creutzfeldt-Jakob disease has occurred in North America so far, except among those who have traveled to Europe.

On June 10, 2005, the USDA reported a possible case of BSE in the United States. Tests carried out at the USDA laboratory in Ames, Iowa indicated the presence of BSE, and after subsequent confirmation from the Weybridge Veterinary Laboratory in the United Kingdom, the USDA acknowledged the second case of BSE on June 24. Trace-backs revealed that this cow originated from a herd in Texas, making it the first BSE cow native to the United States.

On March 13, 2006, the U.S. Department of Agriculture confirmed that a 10-year-old, nonambulatory cow in Alabama tested positive for BSE. Despite this the government still plans to scale back testing of animals from about 1000 to 110 daily. The USDA and the National Cattlemen's Beef Association have repeatedly insisted that the testing in place is sufficient.

Husbandry practices in the United States relating to BSE

Soybean meal is cheap and plentiful in the United States. As a result, the use of animal byproduct feeds was never common, as it was in Europe. However, U.S. regulations only partially prohibit the use of animal byproducts in feed. In 1997, regulations prohibited the feeding of mammalian byproducts to ruminants such as cows and goats. However, the byproducts of ruminants can still be legally fed to pets or other livestock and poultry such as pigs and chickens. In addition, it is legal for ruminants to be fed byproducts from some of these animals. [9] A proposal to end the use of cow blood, restaurant scraps, and chicken litter* (fecal matter, feathers) in January 2004 was eventually scrapped, despite the efforts of some advocates of such a policy, who cite the fact that cows are herbivores, and that blood and fecal matter could potentially carry BSE.

In February 2001, the USGAO reported that the FDA, which is responsible for regulating feed, had not adequately policed the various bans. Compliance with the regulations was shown to be extremely poor before the discovery of the Washington cow, but industry representatives report that compliance is now 100%. Even so, critics call the partial prohibitions insufficient. Indeed, US meat producer Creekstone Farms alleges that the USDA is preventing BSE testing from being conducted.

* The term "chicken litter" also includes spilled chicken feed as well as fecal matter and feathers. It is still legal in the United States to use ruminant protein to feed chickens. Thus, ruminant protein can get into the food chain of cattle in this round about way.

BSE statistics by country The following table summarizes reported cases of BSE and of vCJD by country. BSE is the disease in cattle, whilst vCJD is the disease in people.

Country	BSE cases	vCJD cases
Austria	2	0
Belgium	125	0
Canada	4	1
Croatia	0	0
Czech Rep	9	0
Denmark	13	0
Falkland Is	1	0
Finland	1	0
France	891	11
Germany	312	0
Greece	1	0
Hong Kong	2	0
Ireland	1353	2
Israel	1	0
Italy	117	1
Japan	20	1
Lichtenstein	2	0
Luxembourg	2	1
Netherlands	75	1
Oman	2	0
Poland	21	0
Portugal	875	1
Slovakia	15	0
Slovenia	4	0
Spain	412	0
Sweden	1	0
Switzerland	453	0
UK	183803	160
US	3	0
Total	188515	168 (+ 6 results pending)

The figures given above for BSE are certainly too low, and most likely by a considerable amount. The tests used for detecting BSE vary considerably as do the regulations in various jurisdictions for when, and which cattle, must be tested. For instance, in the EU the cattle tested are older (30 months+), while many cattle are slaughtered earlier than that. At the opposite end of the scale, Japan tests all cattle at the time of slaughter. Tests are also difficult as the altered prion protein has very small levels in blood or urine, and no other signal has been found. Newer tests are faster, more sensitive, and cheaper, so it is possible that future figures may be more comprehensive. Even so, currently the only reliable test is examination of tissues during an autopsy.

It is noticeable that there are no cases reported in Australia and New Zealand where cattle are mainly fed outside on grass pasture and, mainly in Australia, non-grass feeding is done only as a final finishing process before the animals are processed for meat.

As for vCJD in humans, autopsy tests are not always done and so those figures too are likely to be too low, but probably by a lesser fraction. In the UK anyone with possible vCJD symptoms must be reported to the UK Creutzfeldt-Jakob Disease Surveillance Unit and so it is unlikely that any cases would be missed. In the U.S., the CDC has refused to impose a national requirement that physicians and hospitals report cases of the disease. Instead, the agency relies on other methods, including death certificates and urging physicians to send suspicious cases to the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University in Cleveland, which is funded by the CDC.

Credit: the majority of this page was taken from a Wikipedia search on BSE. Since Wikipedia is comprised of many different authors, I figured it would provide the most comprehensive body of basic data on the disease.

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