ICD-11 classes
06 Mental, behavioural or neurodevelopmental disorders
Neurocognitive disorders
Dementia
6D85 — Dementia due to diseases classified elsewhere
6D85.1 — Dementia due to Huntington disease

ICD-11 6D85.1 — Dementia due to Huntington disease

Dementia due to Huntington disease occurs as part of a widespread degeneration of the brain due to a trinucleotide repeat expansion in the HTT gene, which is transmitted through autosomal dominance. Onset of symptoms is insidious typically in the third and fourth decade of life with gradual and slow progression. Initial symptoms typically include impairments in executive functions with relative sparing of memory, prior to the onset of motor deficits (bradykinesia and chorea) characteristic of Huntington disease.

The diagnosis excludes nothing.

It has no clarifying diagnoses.