Diagnosis and treatment of Huntington's disease

Huntington's disease (HD), formerly known as Huntington's chorea, was described in 1872 by George Huntington, an American physician. Huntington's disease is a disorder characterized by chorea and dementia usually beginning between the ages of 35 and 45 years and gradually worsening, ending in death an average of 15 years after onset. The most prominent neuropathologic finding is atrophy of the caudate nucleus and the putamen. Huntington's disease is the most frequent inherited neurologic disorder with a prevalence of 4 to 8 per 100,000. At least twice that many persons, the offspring of affected persons may develop HD at some time. This article reports the authors' experience of the systematic examination and treatment of 50 HD patients and more than 100 persons at risk.