Human prion diseases
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
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).
sections/codes in this section (8E00-8E0Z)
- Sporadic Creutzfeldt-Jakob Disease (8E00)
- Acquired prion disease (8E01)
- Genetic prion diseases (8E02)
- Variably protease sensitive prionopathy (8E03)
- Other specified human prion diseases (8E0Y)
- Human prion diseases, unspecified (8E0Z)
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