Human prion diseases

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2019-04


Human prion diseases or transmissible spongiform encephalopathies are rare transmissible diseases affecting the central nervous system. The infectious agents are composed of an abnormal isoform of a host membrane protein called 'prion protein' (PrP). Their common features are a long duration of incubation and lesions limited to the central nervous system without inflammatory or immunologic reaction but with accumulation of an abnormal form of prion protein (PrPsc).

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