Information about Huntington's

Huntington's disease is a rare hereditary disorder of the central nervous system characterized by uncontrollable movement and dementia. (In the past, the disease was called Huntington's chorea, from the Greek word meaning "dance.") The illness begins gradually, usually between the ages of 30 and 40, and people can live with the disease for up to 20 years.

Early signs of Huntington's disease include changes in behavior and unusual, fidgety movements. Symptoms may be mild enough for the disease to go unnoticed for several years.

Eventually, however, twisting and jerking movements that spread to the entire body are followed by memory loss, confusion, and hallucinations.

Huntington's disease directly affects the parts of the brain that control coordination. Studies show a striking decrease in brain levels of the neurotransmitter gamma-aminobutyric acid (GABA), but it is unclear whether this change is a cause or effect of the disease.

In 1993, scientists identified the gene defect that causes Huntington's disease. The gene is dominant, meaning that children with a parent who carries the defective gene have a 50% chance of developing Huntington's disease and that everyone who has the Huntington's gene will eventually develop the disease.

Gene therapy could one day correct the defective gene, but currently no treatment is available.

Publication Review By: Peter V. Rabins, M.D., M.P.H.

Published: 09 Mar 2011

Last Modified: 23 Jun 2011