ICD-11 classes
08 Diseases of the nervous system
Human prion diseases
8E03 — Variably protease sensitive prionopathy

ICD-11 8E03 — Variably protease sensitive prionopathy

A disease of the brain, caused by a mutation(s) in prion protein genes. This disease is characterised by deposition of abnormal prions in the brain leading to behavioural and mood changes, speech deficits, and progressive motor impairments. Confirmation is by pathological examination of the brain or identification of protease-sensitive prion proteins in a brain sample.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Human prion diseases #1434