Young, Anne B. Department of Neurology, University of Michigan, Ann Arbor, Michigan.
Last reviewed:September 2017
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A hereditary disorder of the basal ganglia of the brain causing progressive motor incoordination, abnormal involuntary movements (chorea), and intellectual decline. Huntington's disease is a rare disorder that affects approximately five to ten of every 100,000 persons. The disease, which progresses gradually over 15 to 20 years, is invariably fatal. Inherited as an autosomal dominant mendelian trait, it inevitably develops in those who carry the gene if they live long enough. Men and women are affected equally. The average age at onset is between 35 and 40 years, but the disease can begin as early as 2 years or as late as 80 years (see illustration). The mutation rate is very low, and no confirmed new mutations have been reported. Pathologically, the nerve cells in the caudate nucleus and putamen degenerate; these regions are the most seriously damaged, although other regions of the brain are affected to a minor extent. See also: Brain; Degenerative neural diseases; Gene; Genetics; Human genetics; Mutation; Nerve; Nervous system disorders
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