Bse Link To Human Disease

In 1990 the UK established the Spongiform Encephalopathy Advisory Committee to advise on the potential implications TSEs have on advised the government that it had become concerned about 10 cases of Creutzfeldt-Jakob disease with onset of symptoms in 1994 and 1995. These cases were unusual because they included patients younger than the expected (average age 27 vs 63 for sporadic CJD cases). In addition, the neuropathology and clinical course in these patients was distinguishable from that in other CJD patients. A review of medical histories, genetic analysis, and consideration of other possible causes of CJD "failed to explain these cases adequately" (4,5,11).

As many as 900,000 cattle might have been infected between 1984 and 1995, with 450,000 entering the human food chain prior to 1989 and 283,000 between 1989 and 1995 (6). Cattle are slaughtered at young ages and BSE's long incubation period would preclude most infected animals from showing any clinical signs. Cattle in the earlier stages of incubation are expected to have had much lower accumulations of infectious agent in their brain and spinal cord.

There have been no confirmed cases of v-CJD in people with occupations expected to have the highest risk of exposure, including farmers, abattoir workers, butchers, and veterinarians. The number of new cases of all types of CJD in the UK remains stable, at about one new case per million people per year. This rate is about the same in all countries, including Australia and New Zealand, where neither scrapie nor BSE have been identified. Currently 29 people, one in France and the remainder in the UK, are believed to have v-CJD (13). The proposed link between BSE and v-CJD was eventually substantiated (13,14). There are no predictions of the number of human victims that might eventually result.

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