Huntingtons Disease

By - 2006-01-10 Find more articles like this in our Disability Articles category.

Email article

Print

What is Huntington's Disease?

Huntington's disease (Huntington's chorea) is a progressive, degenerative disease that causes certain nerve cells in your brain to waste away.

As a result, you may experience uncontrolled movements, emotional disturbances and mental deterioration.

The disorder was documented in 1872 by American physician George Huntington. The name chorea comes from the Greek word for "dance" and refers to the characteristic and incessant quick, jerky, involuntary movements.

Huntington's disease is an inherited condition resulting from a single abnormal gene.

Signs and symptoms usually develop in middle age, and men and women are equally likely to develop the disease. Younger people with the disease often have a more severe condition and symptoms may progress more quickly. Rarely, cases may occur in children.

Psychopathological symptoms vary more than cognitive and physical symptoms, and may include anxiety, depression, a reduced display of emotions and decreased ability to recognize negative expressions like anger, disgust, fear or sadness in others, egocentrism, aggressive behavior, compulsivity which can cause addictions such as alcoholism and gambling, or hypersexuality.

Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations.

An estimated one in 10,000 Americans has the disease, with about 30,000 known cases in the United States. About 150,000 Americans may be at risk of inheriting Huntington's disease from a parent.

At this time, there is no way to stop or reverse the course of HD. Now that the HD gene has been located, investigators are continuing to study the HD gene with an eye toward understanding how it cause disease in the human body.