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8E02.1 Gerstmann-Straussler-Scheinker syndrome

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

exclusions

• Gerstmann syndrome  (MB4C)

postcoordination

synonyms

• Gerstmann-Straussler-Scheinker syndrome
• Gerstmann Straussler syndrome

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