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Prion diseases: Update on mad cow disease, variant creutzfeldt-jakob disease, and the transmissible spongiform encephalopathies

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Abstract

Transmissible spongiform encephalopathies (TSEs) are a group of progressive, fatal neurodegenerative disorders that share a common spongiform histopathology. TSEs may be transmitted in a sporadic, familial, iatrogenic, or zoonotic fashion. The putative infectious agent of TSE, the prion, represents a novel paradigm of infectious disease with disease transmission in the absence of nucleic acid. Several small but spectacular epidemics of TSEs in man have prompted widespread public health and food safety concerns. Although TSEs affect a comparatively small number of individuals, prion research has revealed fascinating insights of direct relevance to common illnesses. This paper reviews recent advances that have shed new light on the nature of prions and TSEs.

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Authors and Affiliations

  1. NCI, National Institutes of Health, Building 10, Room 12S245, 20892, Bethesda, MD, USA

    Frank Maldarelli MD, PhD

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  1. Jacqueline Janka MD
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  2. Frank Maldarelli MD, PhD
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Janka, J., Maldarelli, F. Prion diseases: Update on mad cow disease, variant creutzfeldt-jakob disease, and the transmissible spongiform encephalopathies. Curr Infect Dis Rep 6, 305–315 (2004). https://doi.org/10.1007/s11908-004-0053-y

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  • DOI: https://doi.org/10.1007/s11908-004-0053-y

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