8E02.1

ICD-11 MMS 2026-01

Gerstmann-Straussler-Scheinker syndrome

Description

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.

Index terms

  • Gerstmann-Straussler-Scheinker syndrome
  • Gerstmann Straussler syndrome

Excludes

  • MB4C – Gerstmann syndrome

Exclusions from above levels

  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
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  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8E02.0 – Genetic Creutzfeldt-Jakob disease
  • 8E02.2 – Fatal familial insomnia
  • 8E02.Y – Other specified genetic prion diseases
  • 8E02.Z – Genetic prion diseases, unspecified

Foundation entity

Gerstmann-Straussler-Scheinker syndrome – http://id.who.int/icd/entity/406818835

Allowed postcoordination axes

Part of: 8E02: Genetic prion diseases › 08: Diseases of the nervous system