Learning to Live at Risk for Huntingtonʼs Disease

Huntington's disease (HD) is an autosomal dominant inherited disorder of the central nervous system. Consequently, a child of an affected parent has a 50% risk of developing the disease. This article provides an overview of HD by providing a brief history of the disease and describing progressive symptoms of chorea, dystonia, incoordination and decreased mental acuity. Steps in diagnosis, treatment and prognosis are outlined. Because persons at risk for HD have usually witnessed the deterioration of an HD parent, they are often dominated by fears of developing HD themselves. Life's choices, such as whether or not to have children, then become dilemmas. The importance of maintaining a positive life perspective, while making practical choices about employment, marriage, children and other concerns needs to be addressed.