ICD-11 code : L1-8E0;
Preferred Label : Human prion diseases;
ICD-11 definition : 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).;
ICD-11 synonym : TSE - [transmissible spongiform encephalopathy]; transmissible spongiform encephalopathy; Prion disease of central nervous system;
ICD-11 acronym : TSE;
ICD-11 "other" category code : 8E0Y;
ICD-11 "unspecified" category code : 8E0Z;
Origin ID : 1965146397;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
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).
