What is Huntington disease?

Huntington disease is a genetic neurodegenerative disease in which brain cells (called neurons) begin to deteriorate, causing physical, behavioral and cognitive problems. The disease is triggered by a single defective gene that causes nerve cells in the brain to deteriorate as the disease progresses. Since it is hereditary, each child of an HD parent has a 50-50 chance of inheriting the HD gene. A person who inherits the gene will eventually develop the disease.

 

Some early symptoms of HD include mood swings, depression and irritability. People sometimes also have 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.

 

Presymptomatic testing is available for individuals who are at risk for carrying the HD gene, and genetic counseling is available for those interested in getting the test.

 

There is no medical cure for HD, but a variety of drug, dietary and other treatments are available to treat symptoms. Through research, we hope to identify early changes caused by HD to better understand the disease and hopefully find more effective treatments through clinical trials.

 

(Source: National Institute of Neurological Disorders and Stroke)