Huntington's Disease

Huntington's disease (HD) is an inherited genetic disease that results in degeneration of nerve cells in in the brain, causing physical, mental, and psychiatric disorders.

The hallmark symptom of Huntington's disease (HD) is uncontrolled movement of the arms, legs, head, face and upper body. Those with Huntington's disease often experience alterations in mood such as depression, anxiety, and psychosis. Behavior changes usually occur before movement problems. As the disease progresses, the patient may have difficulty feeding himself or herself and swallowing.

Huntington disease is caused by a genetic defect on chromosome 4 and is passed down through families. The defect causes a part of DNA, called a CAG repeat, to occur many more times than normal.

Although there is not a cure for Huntington's disease, there are medications which can help manage some of the symptoms. These medications cannot slow down or stop the disease. Dopamine blockers may help reduce abnormal behaviors and movements. Drugs such as amantadine and tetrabenazine  are used to try to control extra movements.

Huntington disease is caused by a genetic defect on chromosome 4 and is passed down through families. The defect causes a part of DNA, called a CAG repeat, to occur many more times than normal. Those with Huntington disease may have a repeat of 36 to 120 times compared to a normal repeat of 10 to 28 times.

The number of repeats tend to get larger as the gene is passed down through families. The larger the number of repeats, the higher the chance of developing symptoms at an earlier age. As the disease continues to pass down within the family, the symptoms typically develop at younger and younger ages.

Huntington's disease is passed from parent to child through a mutation in the normal gene. If a parent has Huntington's disease, each child 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.

See a doctor for uncontrolled movements, changes in mood or thinking, or a family history of Huntington's disease. Genetic counseling and early support help with planning and care.