Huntington Chorea - Huntington Disease

Huntington disease is an inherited brain disease that affects movement, thinking, and personality.

What is going on in the body?

Huntington disease causes cells in certain areas of the brain to slowly destruct. It usually starts in middle adulthood.

What are the causes and risks of the disease?

Huntington disease is a genetic disorder. It is an autosomal dominant disorder that can be passed from affected people to their children.

What are the signs and symptoms of the disease?

Huntington disease may cause:

How is the disease diagnosed?

Diagnosis is made on the basis of the symptoms, and a physical examination. The diagnosis can be confirmed with a DNA test. Early testing of a fetus through amniocentesis can tell whether an unborn child will have the disease.

What can be done to prevent the disease?

Nothing can be done to prevent Huntington disease in a person who has already been born. Genetic testing can determine whether someone has the gene that causes Huntington disease. Genetic counseling may be useful for a person with a family history of Huntington disease.

What are the long-term effects of the disease?

Huntington disease gradually gets worse over time. Affected people may be unable to take care of themselves and may need to put in an institution. Death occurs in most affected people about 10 to 20 years after the disease is diagnosed.

What are the risks to others?

Huntington disease is not contagious. It is an autosomal dominant disorder that can be passed from affected people to their children. Autosomal dominant diseases require only one bad gene from one parent to pass on the disease to a child. With autosomal dominant conditions, the parent who passes on the bad gene also has the disease. Because the disease develops in middle adulthood, an individual may have children before he or she is diagnosed with the disease.

What are the treatments for the disease?

There is no cure and there are no treatments that slow the progression of Huntington disease. Medication may be used to treat psychosis, depression, or movement problems.

What are the side effects of the treatments?

The medications used for Huntington disease may cause fatigue, stomach upset, and new movement problems.

What happens after treatment for the disease?

The disease continues to progress and the person gets worse over time.

How is the disease monitored?

Symptoms are monitored by reports and physical examinations to determine the need for medications. Any new or worsening symptoms should be reported to the healthcare provider.

Author:Adam Brochert, MD
Date Written:
Editor:Smith, Elizabeth, BA
Edit Date:04/17/00
Reviewer:Ronald Jorgenson, DDS, PhD, FACMG
Date Reviewed:07/27/01

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