Huntington's Disease - 101

Huntington's Disease (HD) is an inherited brain disorder for which, at present, there is no cure. The disease currently affects 30,000 Americans, with 150,000 more at risk.

Traditionally this condition was known as Huntington's Chorea. The name Huntington's is used as a tribute to George Huntington, the American doctor who first reported his description of the disorder in 1872. Chorea is the Greek word for dance, which reflects the involuntary movements associated with HD. The term Huntington's disease is now commonly used to refer to the illness.

Huntingtons Disease transcends the boundaries of race, sex, ethnic background and can be passed from one generation to the next by either the mother or the father. The gene that causes HD is called a dominant one. This means that each child of a parent with HD has a 50/50 risk of having inherited the gene which causes HD. Sometimes a parent may die before the age when his or her HD symptoms would have appeared. This gives the wrong impression that the illness has skipped a generation. Although each child has a 50/50 chance of escaping or inheriting the disease from an HD parent, in some families all children may inherit it. It is like flipping a coin - there is equal chance to get heads or tails regardless of the results of the last toss. It is the same chance for each child regardless of whether or not any of his/her brothers or sisters are affected. It is also not determined by birth order or by who looks/acts most like the affected parent.

What are the earliest symptoms?

Early symptoms may appear as slight physical, cognitive or emotional change.

PHYSICAL: "Nervous" activity, fidgeting, a twitch in the extremities, or excessive restlessness. Some clumsiness, alterations in handwriting or difficulty with normal daily physical skills such as driving.

COGNITIVE: In addition to the initial physical symptoms of HD, there are often very subtle cognitive signs as well. There may involve little more than a reduced ability to organize routine matters or to cope effectively with new situations. There may be a loss of short-term memory. Work activities may become more time consuming. Decision-making and attention to details may be impaired. Making choices that involve more than two items becomes very stressful.

EMOTIONAL: Early emotional symptoms may be equally subtle. There may be an accentuation of certain aspects of the individuals normal make-up such as more periods of depression, apathy, irritability, and impulsiveness or there may be a change in personality. Rarely, a person may become delusional or unrealistically paranoid.

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