Huntington’s Chorea

Chorea is an abnormal involuntary movement  derived from the Greek word “dance”. It is characterized by brief, abrupt, irregular, unpredictable, non-stereotyped movements. In milder cases, they may appear purposeful; the patient often appears fidgety and clumsy. They can affect various body parts, and interfere with speech, swallowing, posture and gait.

Chorea may worsen with anxiety and voluntary movements, and subsides during sleep. They may occur with athetosis, a more distal, slower, writhing, abnormal movement, and is known as choreoathetosis. In more severe choreiform movements, they appear wild, violent, and may involve flinging of a body part and induce injuries, and is known as ballism. Chorea may also occur with other abnormal movements such as dystonia.

There is a wide range of seemingly unrelated causes, from pregnancy (chorea gravidarum) to inherited forms such as Huntington’s disease and benign hereditary chorea, infection/immune-related such as Sydenham’s chorea and systemic lupus erythematosus, focal vascular lesions in the basal ganglia, drugs such as levodopa, neuroleptics and oral contraception, various metabolic and endocrinological disorders such as hyperthyroidism, hypo/hyperparathyroidism and hypo/hyperglycemia. Its pathophysiology involves a functional dysregulation of the basal ganglia motor circuit, where the final  thalamo-cortical output is increased, resulting in increased movement and chorea.

Treatment of chorea usually entails addressing its root etiology. The most common symptomatic treatment of chorea  include the use of neuroleptic agents, other dopamine depleters such as tetrabenazine, and sometimes benzodiazepines.

Movement Disorders Organization      -

​​The Alzheimer’s & Parkinson’s Association
of the Treasure Coast