ICD-11 classes
01 Certain infectious or parasitic diseases
Human prion diseases
8E02 — Genetic prion diseases
8E02.1 — Gerstmann-Straussler-Scheinker syndrome

ICD-11 8E02.1 — Gerstmann-Straussler-Scheinker syndrome

A disease caused by inheritance of mutation(s) in normal prion protein genes. This disease is characterised by cerebellar ataxia, decreased coordination, dysmetria, or dysarthria, and is fatal. Confirmation is by pathological examination of the brain and genetic testing.

The diagnosis includes nothing.

It excludes 1 item.

  • Gerstmann syndrome (MB4C)

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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