a progressive hereditary disease associated with degeneration of nerve cells, particularly in the caudate nucleus of the
basal ganglia and in the
cerebral cortex. It is characterized by abnormalities of gait and posture, motor incoordination, and involuntary jerking motions (
chorea) as well as
dementia, mood disturbances, and personality and behavioral changes. The age of onset is usually between 30 and 50, but there is a juvenile form of the disease in which symptoms first appear before the age of 20. Huntington’s disease is inherited as an autosomal dominant trait (see
dominant allele); the single gene responsible is located on chromosome 4. Also called
Huntington’s chorea. [George Huntington (1850–1916), U.S. physician]