Huntington's Disease



Huntington' disease, formerly called Huntington's chorea (kor-EE-a), is a rare disease that causes part of the brain to deteriorate. A person with the disease has involuntary and strange movements. It is a genetic disorder that is passed from parent to child.

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Who Is at Risk for Huntington's Disease?

In the United States, only about 5 out of 100,000 people develop Huntington's disease. Because it is transmitted from parent to child, only children of a parent who has the abnormal gene are at risk, and they have a 50 percent chance of developing the disease.

What Is Huntington's Disease?

Huntington's affects the basal ganglia (GANG-lee-a, nerve cell bodies in part of the white matter of the brain). This part of the brain acts as an important pathway for the central nervous system * . Huntington's disease causes erratic movements, usually first affecting the face and speech. Memory, reasoning, and speech become affected. Eventually, the abilities to walk, swallow, and take care of oneself are lost. Males and females are equally affected. A person with Huntington's disease usually first has symptoms between the ages of 35 and 50. A person with the disease may live for a period of 10 to 20 years more, but the condition becomes worse over time.

What Are the Symptoms of Huntington's Disease?

A person with Huntington's disease may first make unusual or strange facial grimaces and become clumsy. Also, the person may become irritable or forgetful. The person may appear to be drunk even without having consumed any alcohol. The awkwardness that comes from the disease may put the person in danger, for example, by losing his balance while crossing the street.

What Is the Treatment for Huntington's Disease?

There is no cure for Huntington's disease, but there is medicine to control the erratic movements caused by the disease. This medicine blocks the production of dopamine * in the brain.

* central nervous system consists of the brain and the spinal cord. It controls and coordinates the activities of the entire nervous system.

* dopamine is a neurotransmitter (a chemical messenger) in the brain that helps to transmit messages between nerve cells in the brain.

George Huntington

The word "chorea" comes from the Greek language, by way of Latin, and means "dance." During the Renaissance, the Swiss physician Paracelsus (1493—1541) wrote about the uncontrollable rhythmic movements characteristic of several different movement disorders.

In 1872, the American physician George Huntington (1850—1916) described an inherited choreatic disorder that began late in life. Because of that research the disorder was named "Huntington's Chorea," now known as Huntington's disease.

Children of someone with Huntington's disease are advised to seek genetic counseling before deciding whether or not to have children of their own, as they also could get the disease. A particular blood test can determine whether or not a person has the gene for Huntington's disease.

Is There a Cure for Huntington's Disease?

There is no cure for Huntington's disease, but knowing if a person has the gene can influence a family as to whether to have children who might develop this disease later in their lives. The test will show whether or not the person has the gene that causes the disease. Sometimes people who are at risk may not want to know if they have the gene, and they take the chance of having children before the onset of the disease is noticed. They feel that knowing they have the gene will make it impossible for them to live a normal life.

Who Was Woody Guthrie?

Woody Guthrie was a folk singer who was well known during the 1930s and 1940s for his songs about the life of migrant workers and poor city people. Among his best known songs are "This Land Is Your Land" and "So Long, It's Been Good to Know You." Woody Guthrie died of Huntington's disease. When he first exhibited symptoms of the disease, people thought he was drunk. Only later was his behavior understood. His son Arlo Guthrie is also a famous singer. Arlo will not develop Huntington's disease because he did not inherit the gene from his father.

See also
Genetic Diseases

Resources

Caring for People with Huntington's Disease, on line at
http://www.edu/hospital/huntingtons/

Guthrie, Woody. Bound for Glory, 1943. An autobiography by the famous folk singer.

Huntington's Disease Society of America (HDSA), 140 West 22nd Street, 6th Floor, New York, NY 10011-2420. Telephone (212) 242-1968 or (800) 345-4372; e-mail: curehd@hdsa.ttisms.com; website: http://hdsa.mgh.harvard.edu

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