Is There Any Treatment?

There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Treatment for Huntington disease is supportive, while treatment for Syndenham's chorea usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. Adjusting medication dosages can treat drug-induced chorea. Metabolic and endocrine-related choreas are treated according to the cause(s) of symptoms.

What Is the Prognosis?

The prognosis for individuals with chorea varies depending on the type of chorea and the associated disease. Huntington disease is a progressive, and ultimately, fatal disease. Syndenham's chorea is treatable and curable.

What Research Is Being Done?

The National Institute of Neurological Disorders and Stroke, a part of the National Institutes of Health, supports research on movement disorders such as chorea. The goals of this research are to increase understanding of these disorders and to find ways to prevent and treat them.

Additional Resources

Hereditary Disease Foundation
3960 Broadway, 6th Floor
New York, NY 10032
(212) 928-2121
Fax: (212) 928-2172
Internet: www.hdfoundation.org
E-mail: cures@hdfoundation.org

Huntington's Disease Society of America
505 Eighth Ave., Suite 902
New York, NY 10018
1-800-345-HDSA (1-800-345-4372) or (212) 242-1968
Fax: (212) 239-3430
Internet: www.hdsa.org
E-mail: hdsainfo@hdsa.org

Source: National Institute of Neurological Disorders and Stroke, National Institutes of Health

Updated: February 14, 2007

Huntington disease (HD) results from genetically programmed degeneration of brain cells, called neurons, in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. HD is a familial disease, passed from parent to child through a mutation in the normal gene. 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. A person who inherits the HD gene will sooner or later develop the disease. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene. Some early symptoms of HD are mood swings, depression, irritability or trouble driving, learning new things, remembering a fact or making a decision. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult and the patient may have difficulty feeding himself or herself and swallowing. The rate of disease progression and the age of onset vary from person to person. A genetic test, coupled with a complete medical history and neurological and laboratory tests, help physicians diagnose HD. Presymptomic testing is available for individuals who are at risk for carrying the HD gene. In 1 percent to 3 percent of individuals with HD, no family history of HD can be found.

Is There Any Treatment?

Physicians prescribe a number of medications to help control emotional and movement problems associated with HD. Most drugs used to treat the symptoms of HD have side effects such as fatigue, restlessness or hyperexcitability. It is extremely important for people with HD to maintain physical fitness as much as possible, as individuals who exercise and keep active tend to do better than those who do not.

What Is the Prognosis?

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 causes disease in the human body.

What Research Is Being Done?

Scientific investigations using electronic and other technologies enable scientists to see what the defective gene does to various structures in the brain and how it affects the body's chemistry and metabolism. Laboratory animals are being bred in the hope of duplicating the clinical features of HD so that researchers can learn more about the symptoms and progression of HD. Investigators are implanting fetal tissue in rodents and nonhuman primates with the hope of understanding, restoring or replacing functions typically lost by neuronal degeneration in individuals with HD. Related areas of investigation include excitotoxicity (overstimulation of cells by natural chemicals found in the brain), defective energy metabolism (a defect in the mitochondria), oxidative stress (normal metabolic activity in the brain that produces toxic compounds called free radicals) and tropic factors (natural chemical substances found in the human body that may protect against cell death).

Additional Resources

Hereditary Disease Foundation
3960 Broadway, 6th Floor
New York, NY 10032
(212) 928-2121
Fax: (212) 928-2172
Internet: www.hdfoundation.org
E-mail: cures@hdfoundation.org

Huntington's Disease Society of America
505 Eighth Ave., Suite 902
New York, NY 10018
1-800-345-HDSA (1-800-345-4372) or (212) 242-1968
Fax: (212) 239-3430
Internet: www.hdsa.org
E-mail: hdsainfo@hdsa.org

Source: National Institute of Neurological Disorders and Stroke, National Institutes of Health

Updated: February 13, 2007