ICD-11 classes
08 Diseases of the nervous system
Human prion diseases

ICD-11 Human prion diseases

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).

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis contains 6 clarifying diagnoses:

1. 8E00 — Sporadic Creutzfeldt-Jakob Disease
2. 8E01 — Acquired prion disease
   It contains 4 clarifying diagnoses.
3. 8E02 — Genetic prion diseases
   It contains 6 clarifying diagnoses.
4. 8E03 — Variably protease sensitive prionopathy
5. 8E0Y — Other specified human prion diseases
6. 8E0Z — Human prion diseases, unspecified

The diagnosis is coded elsewhere:

• 01 Certain infectious or parasitic diseases #1
• Human prion diseases #1434