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Dr. Coulthart is with the National Laboratory for Host Genetics and Prion Diseases, the Canadian Science Centre for Human and Animal Health, Health Canada, Winnipeg, Man. Dr. Cashman is with the Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ont.
Correspondence to: Dr. Michael B. Coulthart, National Laboratory for Host Genetics and Prion Diseases, Canadian Science Centre for Human and Animal Health, Health Canada, 1015 Arlington St., Winnipeg MB R3E 3R2; fax 204 789-5021; mike_coulthart{at}hc-sc.gc.ca and to Dr. Neil R. Cashman, Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park Cres. W, Toronto ON M5S 1A8; fax 416 978-1878; neil.cashman{at}utoronto.ca
Abstract
THE PRION DISEASES POSE UNIQUE SCIENTIFIC, medical, veterinary and regulatory challenges. Here, we summarize current information bearing on the natural history, pathobiology and epidemiology of these disorders and public policy responses to the potential threats to public health posed, particularly, by bovine spongiform encephalopathy and variant CreutzfeldtJakob disease (vCJD). Six years after the first case reports of vCJD, there is still no clear indication of the magnitude of the primary epidemic, or of the likelihood of lateral transmission of this untreatable disease by iatrogenic means, particularly by blood and blood products. However, the unsettling nature of the available evidence warrants prudence regarding public health policy and regulation, as well as a forward-looking approach to research.
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