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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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