Chorea is an irregular, rapid, uncontrolled, involuntary, excessive movement that seems to move randomly from one part of the body to another. The affected child often appears fidgety or restless and unable to sit still. The word "chorea" comes from the Greek word for dance. The jerky movements of the feet or hands are often similar to dancing or piano playing. When chorea is severe, the movements may cause motion of the arms or legs that results in throwing whatever is in the hand or falling to the ground.

Chorea is an abnormal voluntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement. The cause of sydenham chorea is only certain types of streptococci, called "Lancefield Group A beta-hemolytic." These particular germs seem to be able to create an immune response that attacks the body's own tissues along with the germs.

The disease was Huntington's Chorea, which is an inherited, degenerative disorder of the Central Nervous System, caused by a dominant gene. This means that everyone who inherits the gene from one of his/her parents WILL develop the disease, and the likelihood of doing so is therefore 50%. The specter of this cruel disease continues to hang over the entire Guthrie family.

Symptoms of Chorea

Swelling and inflammation (arthritis) of one or more joints that may be characterized by redness, warmth, tenderness, and pain (arthralgia) of affected joint regions. Arthritis and fever are the most common symptoms initially recognized in association with ARF.

Symptoms of muscular twitchings, among which are twitchings and spasms of the eyeballs and eyelids; it has angular choreic movements and spasmodic motions of the extremities, which are not confined to one side of the body, but affect the upper half on one side of the body and the lower half on the other.

Symptoms may appear gradually or suddenly, and may include muscle weakness, hypotonia (decreased muscle tone), and clumsiness. The symptoms vary in severity--from mild cases in which there is restlessness, facial grimacing, and a slight degree of incoordination of movements, to severe cases involving involuntary movements that incapacitate the child. 1

Causes of Chorea

Senile chorea, which is gradual in onset, is not associated with other causes of chorea, does not cause personality changes, and develops in people over the age of 60. At one time, senile chorea was thought to be a late-onset form of HC, but is presently considered to be the result of a different genetic mutation.

Chorea may also be caused by drugs (levodopa, anti-convulsants, anti-psychotics), metabolic disorders, endocrine disorders, and vascular incidents.

Huntington's chorea (HC), an incurable hereditary disorder caused by a mutation in a gene on the short arm of human chromosome 4. It is characterized by dementia and psychiatric disturbances as well as chorea.

Treatment for chorea

There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Treatment for Huntington's 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.