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- W1601009770 abstract "Prion diseases, also referred to as transmissible spongiform encephalopathies (TSEs), are rare but important causes of central nervous system (CNS) disease in humans and other mammals. Human prion/TSE diseases include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia. A novel addition to this intriguing group of disorders referred to as variant CJD (vCJD) has been attributed to transmission of bovine spongiform encephalopathy (BSE; mad cow disease) to humans. Although it was feared that vCJD might become epidemic, the number of reported cases remains low and does not appear to be increasing. The prevailing theory for the pathogenesis of these disorders is the “prion hypothesis,” which attributes disease to the accumulation of proteinaceous infectious particles (prions) composed of an abnormal form of a natural host protein (prion protein [PrP]) that is devoid of nucleic acid. A small group of contrarian scientists has continued to argue that TSEs may be the result of infection with a virus, with mutant PrP being either a component of the infectious agent or a by-product of infection. The 3 articles reviewed address questions regarding the TSE agent and discuss the risk factors and diagnosis of CJD and vCJD." @default.
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- W1601009770 date "2007-01-01" @default.
- W1601009770 modified "2023-09-23" @default.
- W1601009770 title "Infectious disease - developments in the field of Creutzfeldt-Jakob disease." @default.
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