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Huntington's Disease

Huntington's disease is a movement disorder characterized by chorea and dementia. It is inherited in an autosomal dominant manner and occurs throughout the world, in all ethnic groups, with a

Clinical Findings

Clinical onset is usually between 30 and 50 years of age. The disease is progressive and usually leads to a fatal outcome within 15B20 years. The initial symptoms may consist of

Chorea developing with no family history of choreoathetosis should not be attributed to Huntington's disease, at least not until other causes of chorea have been


There is no cure for Huntington's disease, progression cannot be halted, and treatment is purely symptomatic. The reported biochemical changes suggest a relative underactivity of neurons containing gamma-aminobutyric acid (GABA) and acetylcholine or a relative overactivity of dopaminergic neurons. Treatment with drugs blocking dopamine receptors, such as phenothiazines or haloperidol, may control the

Offspring should be offered genetic counseling. Now that the gene responsible for