Palaces For The People
Saturday, January 03, 2004

Bovine Spongiform Encephalopathy and
Variant Creutzfeldt-Jakob Disease:
Background, Evolution, and
Current Concerns

Paul Brown,* Robert G. Will,† Raymond Bradley,‡
David M. Asher,§ and Linda Detwiler¶
*National Institute of Neurological Disorders and Stroke, National Institutes
of Health, Bethesda, Maryland, USA; †National Creutzfeldt-Jakob Disease
Surveillance Unit, Western General Hospital, Edinburgh, Scotland; ‡Central
Veterinary Laboratory, New Haw, Addlestone, UK; §Center for Biologics
Evaluation and Research, Food and Drug Administration, Rockville,
Maryland, USA; ¶Animal and Plant Health Inspection Service, U.S.
Department of Agriculture, Robbinsville, New Jersey, USA
The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom,
which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion,
but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably
resulting from the consumption of beef products contaminated by central nervous
system tissue. Although averaging only 10-15 cases a year since its first appearance in
1994, its future magnitude and geographic distribution (in countries that have imported
infected British cattle or cattle products, or have endogenous BSE) cannot yet be
predicted. The possibility that large numbers of apparently healthy persons might be
incubating the disease raises concerns about iatrogenic transmissions through
instrumentation (surgery and medical diagnostic procedures) and blood and organ
donations. Government agencies in many countries continue to implement new
measures to minimize this risk.

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