Overview of Huntington's Disease
Huntington's disease (HD) is a fatal hereditary disease that destroys neurons in areas of the brain involved in movement, intellect, and emotions. The course of Huntington's is characterized by jerking uncontrollable movement of the limbs, trunk, and face (chorea); progressive loss of mental abilities; and the development of psychiatric problems.
Huntington's disease progresses without remission over 10 to 25 years and patients ultimately are unable to care for themselves. Huntington's disease usually appears in middle age (30-50 years), but can develop in younger and older people.
Juvenile HD (also called Westphal variant or akinetic-rigid HD) develops before the age of 20, progresses rapidly, and produces muscle rigidity in which the patient moves little, if at all (akinesia).
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