clinical features and course

Last reviewed 01/2018

The clinical manifestations of Huntington's disease are varied, with a minority of cases presenting in childhood - juvenile Huntington's disease - and some cases presenting in the elderly.

The majority of cases have an insidious onset, usually occurring between the ages of 30 and 50. The initial disturbances are subtle with changes in personality or behaviour.

The patient may become coarse, irritable and aggressive, and these changes in personality may be reflected by uncontrolled aggressive and sexual behaviour. A schizophreniform psychosis may also develop.

The first physical sign of illness is often the development of chorea, which gradually worsens as the disease progresses to affect walking and speech. Dementia becomes a prominent feature in the later stages and patients eventually develop akinesia and rigidity. This often leads to confinement to bed and emaciation.

Death occurs about 15 years after the onset of symptoms.

In the early stages of illness, insight is retained and this may lead to depression and a significant risk of suicide.