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Chorea, Athetosis, and Hemiballismus

by Hector A. Gonzalez-Usigli, MD, Alberto Espay, MD

Chorea is repetitive, brief, irregular, somewhat rapid involuntary movements that start in one part of the body and move abruptly, unpredictably, and often continuously to another part. Athetosis is a continuous stream of slow, flowing, writhing involuntary movements. Hemiballismus is a type of chorea, usually involving violent, involuntary flinging of one arm and/or one leg.

  • Chorea and athetosis are usually symptoms of another disorder, although chorea may develop on its own in older people or in pregnant women.

  • Chorea and athetosis can occur together, usually causing writhing, dancelike movements.

  • Hemiballismus affects a limb (the arm more often than the leg) on one side of the body, causing it to fling wildly.

  • For chorea and athetosis, treating the cause may help, as may antipsychotic drugs.

Chorea and athetosis, which may occur together as choreoathetosis, are not disorders. Rather, they are symptoms that can result from several very different disorders. Chorea and athetosis result from overactivity in the basal ganglia, the part of the brain that helps smooth out and coordinate movements initiated by nerve impulses from the brain. In most forms of chorea, an excess of dopamine , the main neurotransmitter used in the basal ganglia, prevents the basal ganglia from functioning normally. Drugs and disorders that increase dopamine levels or increase the sensitivity of nerve cells to dopamine tend to worsen chorea and athetosis.

Chorea and athetosis occur in Huntington disease, a hereditary degenerative disorder. Chorea may occur in Sydenham chorea (also called St. Vitus’ dance or Sydenham disease), a complication of rheumatic fever (a childhood infection caused by certain streptococci). Sydenham chorea is characterized by jerky, uncontrollable movements and can last for several months.

Chorea sometimes develops in older people for no apparent reason. This chorea, called senile chorea, tends to affect the muscles in and around the mouth. Chorea can also affect women during the first 3 months of pregnancy (a condition called chorea gravidarum), but it disappears without treatment shortly after they give birth. Rarely, a similar chorea develops in women taking oral contraceptives. Chorea can also result from lupus (systemic lupus erythematosus), overactivity of the thyroid gland (hyperthyroidism), a tumor or stroke affecting a part of the basal ganglia called the caudate nucleus, and certain drugs such as levodopa, phenytoin, and cocaine. In a few people, antipsychotic drugs can cause a chorea called tardive dyskinesia (characterized, for example, by puckering of the lips and tongue or by choreoathetosis).

Hemiballismus is usually caused by a stroke that affects a small area just below the basal ganglia called the subthalamic nucleus. This structure helps control voluntary movements.

Symptoms

Chorea typically involves the hands, feet, and face. The nose may wrinkle, the eyes may continually flit, and the mouth or tongue may continually move. The movements are not rhythmic, but they seem to flow from one muscle to the next and may seem dancelike. The movements may merge imperceptibly into purposeful or semipurposeful acts, sometimes making the chorea hard to identify.

Athetosis usually affects the hands and feet. The slow writhing movements often alternate with holding parts of the limbs in certain positions (postures) to produce a continuous, flowing stream of movement.

When chorea and athetosis occur together, the movements are writhing, dancelike, and slower than in chorea but faster than in athetosis.

Hemiballismus affects one side of the body. The arm is affected more often than the leg. Hemiballismus may be temporarily disabling because when a person tries to move the limb, it may fling out uncontrollably.

Treatment

Chorea in people who have hyperthyroidism usually lessens when that disorder is treated. Sydenham chorea and chorea caused by a stroke often gradually subside without treatment. If chorea is caused by a drug, stopping the drug may help, but the chorea does not always disappear.

Pregnant women with chorea may be treated with barbiturates during the pregnancy. However, after delivery, chorea lessens and eventually disappears on its own.

Drugs that block dopamine ’s action may help control the abnormal movements. These drugs include antipsychotic drugs (see Table: Antipsychotic drugs), such as fluphenazine, haloperidol, and risperidone. Drugs that reduce the amount of dopamine released, such as reserpine and tetrabenazine, may also help. However, improvement may be limited.

Hemiballismus usually goes away on its own after several days, but it sometimes lasts for 6 to 8 weeks. Antipsychotic drugs may help suppress hemiballismus.

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • No US brand name
  • DILANTIN
  • No US brand names
  • HALDOL
  • RISPERDAL