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Why Prion Diseases Are a Mystery, and Why They Matter

Jennifer Fisher Wilson
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Potential Financial Conflicts of Interest: None disclosed.


Ann Intern Med. 2005;143(10):773-776. doi:10.7326/0003-4819-143-10-200511150-00037
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Prion diseases are rare in humans. Only about 300 Americans per year develop these fatal brain-wasting diseases, such as sporadic Creutzfeldt-Jakob Disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, or fatal insomnia. New variant CJD, from consumption of beef infected with bovine spongiform encephalopathy, is even more rare—only about 150 people worldwide have been infected so far, and the number of new cases is waning. Prion diseases that infect animals other than cows—scrapie in goats and sheep and chronic wasting disease in elk and deer—do not appear to infect humans.

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