Huntingdon's disease is an uncommon disease that causes degeneration of the basal ganglia (paired nerve cell clusters in the brain). The disease is due to a defective gene and is inherited in an autosomal dominant manner (see genetic disorders).
Symptoms of Huntington’s disease do not usually appear until age 35 to 50, although they can develop in childhood. The main symptoms are chorea (rapid, jerky, involuntary movements) and dementia (progressive mental impairment). The chorea usually affects the face, arms, and trunk, resulting in random grimaces and twitches, and clumsiness.
Dementia takes the form of irritability, personality and behavioural changes (including outbursts of aggressive, antisocial behaviour), memory loss (especially short-term memory), and apathy. The disease progresses slowly; affected people live for about ten to 20 years after the onset of symptoms.
At present, there is no cure for Huntington’s disease. Treatment is aimed at reducing symptoms with drugs; for example, antipsychotic drugs may help to control the abnormal muscle movements. Speech therapy and occupational therapy may also be of benefit.
Genetic testing is available for people with family members affected by the condition. However, because there is no effective treatment for Huntington’s disease, this may raise difficult ethical questions.