Huntington's Disease Defined

Huntington's disease is an inherited, degenerative brain disease, which affects the mind and body. The disease usually begins during mid-life, and is characterized by intellectual decline, and irregular and involuntary movements of the limbs or facial muscles. Other symptoms of Huntington's disease include personality change, memory disturbance, slurred speech, impaired judgment, and psychiatric problems. Huntington's disease currently affects more than 25,000 Americans. The diagnostic process for Huntington's disease includes an evaluation of family medical history, recognition of typical movement disorders, and CAT brain scanning. A genetic marker linked to Huntington's disease has been identified on chromosome 4 and researchers are working on locating the gene itself. Although there is no treatment available to stop the progression of the disease, the movement disorders and psychiatric symptoms can be controlled by drugs.

Early Symptoms
By Huntington's Disease Society of America

Early symptoms of Huntington's Disease may affect cognitive ability or mobility and include depression, mood swings, forgetfulness, clumsiness, involuntary twitching and lack of coordination. As the disease progresses, concentration and short-term memory diminish and involuntary movements of the head, trunk and limbs increase. Walking, speaking and swallowing abilities deteriorate. Eventually the person is unable to care for him or herself. Death follows from complications such as choking, infection or heart failure.
HD typically begins in mid-life, between the ages of 30 and 50, though onset may occur as early as the age of 2. Children who develop the juvenile form of the disease rarely live to adulthood.

HD affects males and females equally and crosses all ethnic and racial boundaries. Each child of a person with HD has a 50/50 chance of inheriting the fatal gene. Everyone who carries the gene will develop the disease. In 1993, the HD gene was isolated and a direct genetic test developed which can accurately determine whether a person carries the HD gene. The test cannot predict when symptoms will begin. However, in the absence of a cure, some individuals "at risk" elect not to take the test.