Encephalopathy, Bovine spongiform
Transmissible spongiform encephalopathies (TSEs) are diseases characterized by spongy degeneration of the brain with severe and fatal neurologic signs and symptoms. Bovine spongiform encephalopathy (BSE) is one of several different forms of transmissible brain disease affecting a number of animal species. The disease has a long incubation period of 4-5 years, but ultimately is fatal to cattle within weeks to months from its onset. BSE was first recognized in November 1986 with the appearance in cattle of a newly recognized form of neurologic disease in the United Kingdom. BSE caught the world’s attention because of the possibility of crossing the species barrier and eventual transmission from cattle to humans, causing variant Creutzfeldt-Jakob disease (vCJD).
vCJD, which was first reported in March 1996 in the United Kingdom, is caused by the same agent as BSE in cattle. In contrast to the classical forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure, probably through food, to BSE. Recent studies have confirmed that vCJD is distinct from sporadic and acquired CJD.
From October 1996 to November 2002, 129 cases of vCJD had been reported in the United Kingdom, six in France and one each in Canada, Ireland, Italy and the United States of America. Insufficient information is available at present to make any precise prediction about the future number of vCJD cases.
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