Huntington's chorea, also termed Huntington's disease, is an inherited central nervous system (CNS) disease that usually has its onset between ages 25 and 55. It is characterized by involuntary muscular twitching and progressive deterioration of intellectual abilities.

Deterioration of the cerebral cortex and basal ganglia cause chronic progressive chorea and dementia. There is a causative gene inherited as an autosomal dominant trait that has been identified and parents that possess the disease have a fifty-percent chance of passing it on to their children.

Classic signs and symptoms encompass bizarre, involuntary dance-like movements and mental deterioration, ending in dementia. Initially, the patient is usually clumsy, irritable, or impatient with episodes of anger and periods of suicidal depression, indifference, or euphoria. The movements may begin on one side of the body and are more prominent in the face and arms than in the legs. As the disease progresses, the movements progress from a mild fidget to constant movement and dysarthria. As the disease progresses, judgment and memory become impaired, and hallucinations, delusions, and paranoid thinking may occur. Later, emotional symptoms may become less, but eventually dementia does occur. In addition, later stages involve movements of all muscles of the body. Movements are constant; speech becomes incomprehensible; chewing and swallowing are difficult; and walking is impossible. In the late stages, the patient may take on the appearance of emaciation and exhaustion. Eventually, the patient becomes totally dependent.

There is no definitive test for Huntington's disease; however, there are specific tests that can detect it, e.g., deoxyribonucleic acid (DNA) analysis. In addition, as the disease progresses, computed tomography (CT) scan displays characteristic changes.

No cure currently exists for this condition. Supportive and protective therapy form the basis for treatment, depending on the patient's symptoms. Tranquilizers and antipsychotic drugs help to control the choreic movement and ease discomfort and depression but mental deterioration continues. Tranquilizers tend to increase rigidity so alternative drugs may be used. Psychotherapy may also be instituted to decrease anxiety and stress. Mental deterioration may indicate the need for institutionalization.

Complications that may develop from this disorder include choking, aspiration, pneumonia, heart failure, and infection.

• May be entitled to special monthly compensation where the Veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Special Monthly Compensation (SMC).

• None.