Huntington’s disease

An uncommon disease in which degeneration of the basal ganglia results in chorea and dementia. Symptoms of Huntington’s disease do not usually appear until age 35–50. The disease is due to a defective gene and is inherited in an autosomal dominant manner (see genetic disorders). 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, memory loss, and apathy. At present, there is no cure for Huntington’s disease, and treatment is aimed at reducing symptoms with drugs.


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