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Chorea, Athetosis, and Hemiballismus

By

Alex Rajput

, MD, University of Saskatchewan;


Eric Noyes

, MD, University of Saskatchewan

Reviewed/Revised Feb 2024
VIEW PROFESSIONAL VERSION
Topic Resources

Chorea is characterized by random, flowing involuntary movements that cannot be suppressed. They start in one part of the body and move abruptly, unpredictably, and often continuously to another part. Chorea typically involves the face, mouth, trunk, and limbs. Athetosis is a continuous stream of slow, flowing, writhing involuntary movements. It usually affects the hands and feet. Hemiballismus is a type of chorea, usually involving violent, involuntary flinging of one arm and/or one leg. Movements are wider and more intense than chorea.

  • Chorea and athetosis are usually symptoms of another disorder, although chorea may develop on its own in older adults 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 medications.

Chorea and athetosis, which may occur together as choreoathetosis, are not disorders. Rather, they are symptoms that can result from several very different disorders.

Causes

Chorea and athetosis result from overactivity in the basal ganglia, the part of the brain that helps initiate and smooth out and coordinate intended (voluntary) 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. Medications, other substances, and disorders that increase dopamine levels or increase the sensitivity of nerve cells to dopamine tend to worsen chorea and athetosis.

Locating the Basal Ganglia

The basal ganglia are collections of nerve cells located deep within the brain. They include the following:

  • Caudate nucleus (a C-shaped structure that tapers to a thin tail)

  • Putamen

  • Globus pallidus (located next to the putamen)

  • Subthalamic nucleus

  • Substantia nigra

The basal ganglia help initiate and smooth out muscle movements, suppress involuntary movements, and coordinate changes in posture.

Locating the Basal Ganglia

Chorea may also occur in the following:

Chorea sometimes develops in older adults for no apparent reason. This chorea, called senile chorea, tends to affect the muscles in and around the mouth. If such movements develop, people should see a doctor.

Hemiballismus is usually caused by a stroke that affects a small area just below the basal ganglia called the subthalamic nucleus. The subthalamic nucleus 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 random, and they seem to flow from one muscle to the next. They 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. The movements cannot be suppressed and may be violent.

Diagnosis

  • A doctor's evaluation

  • Sometimes tests to identify the cause

The diagnosis of chorea, athetosis, or hemiballismus is based on symptoms and observation by a doctor. The doctor also asks which medications and other substances a person is taking to check for medications and other substances that may be causing the symptoms.

Tests may be done to identify the cause. These tests may include

  • Blood tests to measure levels of thyroid hormones and/or blood sugar

  • Imaging of the brain, such as magnetic resonance imaging (MRI) or computed tomography (CT), to check for tumors or evidence of a stroke

  • Sometimes other tests, depending on what cause is suspected

Treatment

  • Treatment of the cause

  • Medications to help control abnormal movements

Chorea in people who have hyperthyroidism or hyperglycemia 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 medication or other substance, stopping it may help, but the chorea does not always disappear.

If pregnant women have severe chorea, they may be treated with barbiturates during the pregnancy. However, after delivery, chorea lessens and usually disappears on its own.

If people have chorea and athetosis, treatments that help relieve the chorea tend to also help relieve athetosis.

Medications that block dopamine’s action may help control the abnormal movements. These medications include antipsychotic medications Antipsychotic drugs Schizophrenia is a mental disorder characterized by loss of contact with reality (psychosis), hallucinations (usually, hearing voices), firmly held false beliefs (delusions), abnormal thinking... read more , such as fluphenazine, haloperidol, and risperidone. Medications that reduce the amount of dopamine released, such as reserpine, deutetrabenazine, 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. If severe, it is treated with antipsychotics. Antipsychotics may help suppress hemiballismus. If medications are ineffective, deep brain stimulation may be used. For this procedure, tiny electrodes are surgically implanted in the basal ganglia. The electrodes send small amounts of electricity to the specific area of the basal ganglia thought to be responsible for hemiballismus and can thus help lessen symptoms.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Intropin
NATPARA
INBRIJA, Larodopa
Dilantin, Dilantin Infatabs, Dilantin-125, Phenytek
GOPRELTO, NUMBRINO
Prolixin, Prolixin Decanoate
Haldol, Haldol Decanoate
PERSERIS, Risperdal, Risperdal Consta, Risperdal M-Tab, Rykindo, UZEDY
No brand name available
Austedo, Austedo XR
Xenazine
NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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