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Bovine spongiform encephalopathy (BSE), popularly referred to as mad cow disease, has caused such global controversy that one might expect it to be clearly proven as a very contagious and deadly human disease, but this expectation is far from true. Worldwide, fewer than 200 human cases have been blamed on infection with the BSE agent. Infection is thought to cause a human disease called variant Creutzfeldt-Jakob disease (vCJD). The human vCJD illness is somewhat different from the previously recognized classic Creutzfeldt-Jakob disease (CJD). Those affected with vCJD are younger, with a median age of 28 years (versus 68 years for CJD); the duration of illness is about twice as long at 13 to 14 months (versus 4–5 months for CJD); and patients exhibit more prominent psychiatric symptoms compared to dementia.

In all species, the disease causes some types of nervous system signs. In cattle, BSE usually occurs in cows over 4 years of age, causing difficulty walking, apprehension or the appearance of anxiety, loss of body weight, and hypersensitivity. All affected animals will die with or without treatment. The age at which the animal was affected and the clinical signs are useful tools in surveillance targeting high-risk animals.

A number of different mammals, including humans, have been identified with diseases similar to BSE, collectively termed transmissible spongi-form encephalopathy (TSE). Affected natural hosts include mink, cats (domestic and captive large cats), nyala and greater kudu (African antelopes), and North American cervids such as elk, mule deer, white-tailed deer, and red deer. In the United States and Europe, sheep with the TSE or scrapie have been recognized since the 1730s.

A Brief History

In 1986 and 1987, the BSE story began to unfold in the United Kingdom with the identification of a new and unique neurological syndrome in cattle. However, it was later discovered that the story had actually begun 10 to 20 years prior to that, with the implementation of feeding cattle a ration that included meat-and-bone meal (MBM) from cattle and sheep. This was a common practice due to the high protein content in MBM and the scarcity of high-protein crops such as soybean meal.

Once a nationwide reporting system was developed in the United Kingdom, many more cases were reported. The number of new BSE cases in cattle reported per week peaked at 800 in January 1993. Other countries began to report cases in 1989, with cases reported in the Falkland Islands and Ireland, then in Portugal and Switzerland in 1990. As of 2009, a few cases are still reported, but the epidemic seems to be nearing its end. The top 5 countries by reported cases are the United Kingdom (183,841), Portugal (875), Switzerland (453), Spain (412), and Germany (312). In North America, Canada has reported 15 cases, and the United States has reported only 3 domestic-born cases.

The first U.S. case of BSE was confirmed on December 25, 2003, and became known as “the cow that stole Christmas” for those in the U.S. Department of Agriculture (USDA) who were tasked with responding to the situation and communicating to the public regarding risk concerns. The second U.S. case, in June 2005, occurred in a 12-year-old, native-born cow from Texas. In March 2006, the third U.S. case was confirmed in a 10-year-old cow from Alabama. Genetic tests suggested that this last case was spontaneous, not resulting from the feeding of contaminated MBM. This was reassuring because the U.S. feed ban had been in effect since 1997.

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