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Huntington Disease

Alternate Names

  • Huntington chorea

Definition

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.

Risks

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.

Prevention

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.

Diagnosed

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.

Long Term Effects

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.

Other Risks

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.

Treatments

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.

Side Effects

What are the side effects of the treatments?

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

After Treatment

What happens after treatment for the disease?

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

Monitor

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 professional.

Sources

Huntington Disease Foundation of America

Hereditary Disease Foundation

Cecil Textbook of Medicine, 1996, Bennett et al.

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