Huntington's disease

Abstract

Huntington's disease is characterized by striatal degeneration, leading to progressive choreoathetoid movements, dementia and behavioral changes. It is inherited as an autosomal dominant illness with complete penetrance. New technology has localized the aberrant gene, and presymptomatic and even prenatal testing may eventually be available. Although no definitive treatment currently exists, judicious use of neuroleptics, appropriate referrals and a strong therapeutic alliance with the family may be helpful.