Chorea is an involuntary movement disorder that causes irregular muscle movements. It occurs due to the overactivity of dopamine in the brain, which can have many causes. Treatment and outlook both depend on the underlying cause.

Chorea is a type of dyskinesia, which is a group of neurological disorders attributed to the overstimulation of dopamine receptors in areas of the brain that control movement.

Chorea presents as a symptom of a variety of conditions. The treatment and outlook will depend on the underlying cause of chorea. In all cases, doctors will tailor treatment to the specific context.

This article will define chorea and list the causes, treatment options, and outlook for the condition.

A healthcare professional analyzing samples to diagnose the cause of chorea.Share on Pinterest
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According to the National Institute of Neurological Disorders and Stroke (NINDS), chorea is an involuntary movement disorder that causes irregular muscle movements.

The word “chorea” comes from the Greek word for dance. This is because it can make people appear as if they are dancing.

How common is it?

It is unclear how many people experience chorea, as it is usually a symptom of other disorders.

It is a primary symptom of Huntington’s disease, which affects approximately 40,000 people in the United States.

A secondary type of chorea, called Sydenham chorea, affects 20% of children and adolescents who have had rheumatic fever.

However, the incidence of rheumatic fever is largely unknown as experts no longer keep a record of it. The Centers for Disease Control and Prevention (CDC) uses data from the 1980s to show variation across the U.S., where Tennessee reported 0.6 cases per 100,000 people and Utah reported 3.7 cases per 100,000 people.

Learn more about Huntington’s disease and chorea.

Individuals with chorea will experience uncontrolled movements and be unable to maintain steady movements, postures, or positions.

A person’s movements may be:

  • spontaneous
  • nonrepetitive
  • irregularly timed
  • abrupt

The movements can vary in severity, ranging from a mild exaggeration of movement to a continuous flow of unstable movements.

Other symptoms can include:

  • Milkmaid’s grip: This means that a person is not able to coordinate the hand muscles and will squeeze and release the hand as if they were milking. They may also constantly drop objects.
  • Inability to stick out the tongue: When a person with chorea attempts to stick out their tongue, it will dart in and out of the mouth.
  • Hypotonia: A few people with chorea may have low muscle tone.
  • Athetosis: This is a slower form of chorea in which the movements will have a writhing or twisting motion.
  • Ballism: This is a severe form of chorea, in which a person violently flings parts of their body, most often the limbs. It usually affects one side of the body, but in rare cases, it may affect both sides.

As chorea is itself a symptom of other conditions, those who are experiencing it will also have other symptoms associated with the overarching issue.

According to NINDS, chorea develops due to overactivity of the neurotransmitter dopamine in the areas of the brain responsible for movement.

This can develop as a result of:

  • Huntington’s disease, of which chorea is a primary symptom
  • Wilson’s disease
  • spinocerebellar ataxias, a group of inherited conditions that affect movement

Other causes include:

Infectious causesrheumatic fever
Lyme disease
• subacute sclerosing panencephalitis, a neurological condition that affects the central nervous system
Endocrine and metabolic conditionshypoglycemia
Drugs and medications• levodopa, medication to treat Parkinson’s disease
• neuroleptics
oral birth control
Toxinsalcohol intoxication
carbon monoxide poisoning
Vascular conditionsmultiple sclerosis
Churg Strauss
systemic lupus erythematosus
Behçet’s disease
Neurologic conditionsstroke
Malignant causesbrain tumor

During pregnancy, people can also develop a type of chorea called chorea gravidarum. Symptoms resolve after delivery.

Treating chorea usually means addressing the underlying cause.

In some cases, the symptoms may resolve without treatment, such as with pregnancy. In other cases, a person may require an adjustment to their current medication.

Sydenham chorea will usually resolve without treatment. However, if it is severe, a doctor may prescribe sedatives, such as benzodiazepines and barbiturates, or antiepileptic medications, such as valproic acid.

As chorea is associated with dopamine activity in the brain, there are pharmacological options. Some medications, such as tetrabenazine, work to deplete the amount of dopamine in the brain. Antipsychotics can physically block dopamine receptors.

For some people, deep brain stimulation (DBS) may be a beneficial treatment option. During the procedure, the surgeon implants electrodes in the brain to regulate abnormal impulses. However, doctors still consider this an experimental surgery and only an option in severe cases.

The outlook will be dependent on the underlying cause of chorea.

Temporary causes, such as medication or pregnancy, usually result in a complete recovery once the underlying cause resolves.

However, this is not always the case. According to NINDS, most children will also recover from Sydenham chorea, but 33% will experience a return of symptoms between 18–30 months after the first episode.

There also appears to be an association between recurrent Sydenham chorea and pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection (PANDAS).

This is when a child develops obsessive-compulsive disorder or tic disorders suddenly after an infection with strep throat or scarlet fever.

Chorea, as a result of Huntington’s disease, has a poor outlook. Most people with this condition will live for 10–30 years after a diagnosis.

As chorea has a range of possible causes, doctors will gather as much information as possible about the individual and the chorea. This will include asking questions about:

  • family history
  • medical history
  • other symptoms
  • symptom duration

To determine the cause of the chorea, doctors can also order various tests:

  • Blood tests: These can help check for any sign of infection or metabolic issues.
  • Imaging tests: These can include MRI and CT scans to identify any brain issues.
  • Genetic testing: This can help check whether the gene for Huntington’s disease is present.

Chorea is a movement disorder that occurs due to overactive dopamine activity in the brain. It can cause uncontrolled movements that appear to be spontaneous, abrupt, and irregularly timed.

Chorea is a primary symptom of Huntington’s disease, but it can develop as a symptom of many other conditions.

Treatment is available to address the symptoms of chorea. However, treatment of the underlying condition is usually more effective.

Some underlying causes are curable and may result in a full recovery.