8A01.10 Huntington disease

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

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 reveals caudate atrophy. A genetic test is available and may facilitate presymptomatic detection.

inclusions

• Huntington chorea

postcoordination

synonyms

• Huntington disease
• Huntington chorea
• chronic progressive chorea
• chronic progressive hereditary chorea
• HC - [Huntington chorea]
• hereditary chorea
• progressive hereditary chorea

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