Huntington Disease

Huntington disease, formerly called Huntington's chorea (kor-EE-uh), 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, meaning that is inherited by the child from a parent's genes.

Who Is at Risk for Huntington Disease?

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, later known as Huntington disease.

What Is Huntington Disease?

Huntington 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 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 disease usually first exhibits symptoms between the ages of 35 and 50. A person with the disease may live for another 10 to 20 years, but the condition is progressive (becomes worse over time). A less common form of Huntington disease known as early-onset or juvenile Huntington disease begins in childhood or adolescence, usually before the age of 20. Signs of the juvenile form include slow movements, clumsiness, frequent falling, rigidity, slurred speech, and drooling. School performance declines as thinking and reasoning abilities become impaired. Seizures occur in 30 to 50 percent of children with this condition. Juvenile Huntington disease tends to progress more quickly than the adult-onset form; affected people usually live 10 to 15 years after signs and symptoms appear.

What Are the Symptoms of Huntington Disease?

A person with Huntington 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 her balance while crossing the street.

What Is the Treatment for Huntington Disease?

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

WOODY GUTHRIE'S STORY

Woodrow Wilson (Woody) Guthrie (1912–1967) was a folksinger and songwriter who was well known during the 1930s and 1940s for his songs about 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 Yuh.” Woody Guthrie died of Huntington disease (HD).

When he first exhibited symptoms of the disease in the late 1940s, people thought he was drunk. Only after he was diagnosed as suffering from Huntington's chorea (as it was then known) in the 1950s was his behavior understood.

Although there is a predictive test for HD, Woody's son, folksinger Arlo Guthrie (b. 1947), opted not to be tested, and now is beyond the age range when the symptoms would usually first appear.

Offspring of someone with Huntington disease are advised to seek genetic counseling before deciding whether to have children of their own, as these children also could inherit the disease. A particular blood test can determine whether a person has the gene for Huntington disease.

Is There a Cure for Huntington Disease?

There was no cure as of 2016 for Huntington disease, but knowing if a person has the gene can influence that person's decision about having children who might develop this disease later in their lives. The test shows whether 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.

See also Genetic Diseases: Overview

Resources

Books and Articles

Guthrie, Woody. Bound for Glory. New York: Dutton, 1943.

Walker, Francis O. “Huntington's Disease.” Lancet, January 2007. http://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(07)60111-1.pdf (accessed July 17, 2015).

Websites

Genetics Home Reference. “Huntington Disease.” U.S. National Library of Medicine. https://ghr.nlm.nih.gov/condition/huntingtondisease (accessed May 6, 2016).

MedlinePlus. “Huntington's Disease.” U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/huntingtonsdisease.html (accessed May 6, 2016).

Organizations

Hereditary Disease Foundation. 3960 Broadway, 6th Fl., New York, NY 10032. Telephone: 212-928-2121. Website: http://www.hdfoundation.org (accessed July 17, 2015).

Huntington's Disease Society of America. 505 Eighth Ave., Suite 902, New York, NY 10018. Toll-free: 800-345-HDSA (4372). Website: http://www.hdsa.org (accessed July 17, 2015).

National Institute of Neurological Disorders and Stroke. PO Box 5801, Bethesda, MD 20824. Toll-free: 800-352-9424. Website: http://www.ninds.nih.gov (accessed July 17, 2015).

* central nervous system (SEN-trul NER-vus SIS-tem) is the part of the nervous system that includes the brain and spinal cord.

* dopamine (DOE-puh-meen) is a neurotransmitter in the brain that is involved in the brain structures that control motor activity (movement).

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)