ICD-11 classes
08 Diseases of the nervous system
Movement disorders
8A01 — Choreiform disorders
8A01.1 — Secondary Chorea
8A01.10 — Huntington disease

ICD-11 8A01.10 — Huntington disease

Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system. HD is an autosomal dominant disorder due to a mutation resulting in an increased number of triplicate cytosine-adenine-guanine repeats on chromosome 4. The manifestations include chorea, dementia and personality changes. In the Westphal variant dystonia and parkinsonism are prominent. Neuroimaging revels caudate atrophy. A genetic test is available and may facilitate presymptomatic detection.

The diagnosis excludes nothing.

It has no clarifying diagnoses.