What is chorea?

Chorea is a movement disorder that causes involuntary, irregular, unpredictable muscle movements. The disorder can make you look like you’re dancing (the word chorea comes from the Greek word for “dance”) or look restless or fidgety.

Chorea is a movement problem that occurs in many different diseases and conditions. Chorea itself isn’t life-threatening, but it could be a sign of a neurological disease such as Huntington’s disease. Doctors can prescribe medication to control the abnormal muscle movements. Depending on the underlying cause, chorea may be temporary or be ongoing and get worse over time.

At least two other movements related to chorea are seen in neurological diseases.

  • Athetosis is a continuous stream of slow, twisting or squirming-like motions usually involving the hands and feet.
  • Ballimus usually involves more intense movements such as wild flinging of one arm or leg. Usually the movements only affect one side of the body (hemiballism).

How common is chorea?

No one knows for sure how many people experience chorea. Chorea is usually a symptom of another disorder. About 30,000 people in the United States have Huntington’s disease (a genetic condition that causes chorea). Doctors estimate another 200,000 people have a risk of developing Huntington’s disease because their parents have the genetic condition. Chorea is the most common symptom of Huntington’s disease.

In the United States, about 4,000 kids a year develop Sydenham chorea after having rheumatic fever. Rheumatic fever is a serious complication of untreated strep throat. Girls are more likely than boys to get rheumatic fever. It usually occurs between 5 and 15 years of age.

Who is affected by chorea?

Chorea can occur in people of all ages. However, certain groups of people have an increased risk, including:

  • People with a family history of Huntington’s disease: If you have a parent with Huntington’s disease, there is a 50% chance you’ll inherit the disease. Usually, people develop symptoms of Huntington’s disease between ages 40 and 50. Huntington’s disease is the most common inherited type of chorea.
  • Children who have had rheumatic fever: Kids and adolescents can develop Sydenham chorea after rheumatic fever, which is a complication of untreated strep throat.
  • People with other medical conditions: Autoimmune diseases (such as lupus) and hormonal disorders like hyperthyroidism and metabolic disorders such as hypoglycemia can cause chorea. There is a long list of other disorders that can cause chorea.

How do people get chorea?

Chorea is a neurological symptom that originates in an area of the brain called basal ganglia, which are collections of nerve cells deep inside the brain that control movement. Dozens of genetic conditions, autoimmune diseases, metabolic disorders, infections and medications can affect basal ganglia and lead to the onset of chorea.

The most common causes of chorea are:

  • Huntington’s disease: People inherit this genetic disorder from their parents. In addition to chorea, it causes changes in personality and problems with speech, coordination, and memory. Symptoms of Huntington’s disease get worse over time (usually over 10 to 20 years).
  • Rheumatic fever: Around one to eight months after having rheumatic fever, children can develop Sydenham chorea (also called St. Vitus dance). Rheumatic fever is a complication of group A streptococcal infection, such as strep throat infection that wasn’t treated properly. Most of the time, kids with Sydenham chorea get better without treatment in less than two years.
  • Other infectious diseases (Rarely): Lyme disease, toxoplasmosis, HIV/AIDS, endocarditis, syphilis, encephalitis, meningitis, Legionnaire disease, Creutzfeldt-Jakob disease are among infectious diseases that can cause chorea.
  • Autoimmune diseases: People who have systemic lupus erythematosus (also called SLE or just lupus) can develop chorea. Multiple sclerosis, sarcoidosis, Sjogren syndrome, Behcet disease are among other autoimmune diseases that can cause chorea.
  • Stroke: Some people develop chorea if they have a stroke or a tumor that occurs in or near basal ganglia.
  • Pregnancy: Though rare, a type of chorea called chorea gravidarum can occur during pregnancy. (If pregnancy is the cause of the chorea, it may appear during the first three months of pregnancy and stop shortly after the birth of the baby.)
  • Endocrine and metabolic disorders: Hypoglycemia, hyperglycemia, hyperthyroidism, hypoparathyroidism, hyperparathyroidism, hyponatremia, hypernatremia, hypocalcemia, hypomagnesemia, polycythemia vera and hepatic failure are some of the conditions that can cause chorea.
  • Medications: Levodopa, neuroleptics, amphetamines, antihistamines, tricyclic antidepressants, anticonvulsants, cocaine, oral contraceptives are among the many medications known to cause chorea. Antipsychotic drugs can also cause chorea as part of a condition called tardive dyskinesia.
  • Toxins: Carbon monoxide poisoning, mercury, alcohol intoxication can cause chorea.
  • Older age: Sometimes chorea develops in older people without a known cause. This is called senile chorea and tends to affect the muscles in and around the mouth.

What are the symptoms of chorea?

The symptoms of chorea are much the same no matter what caused the movement disorder. The most common signs of chorea are:

  • Involuntary muscle movements: Also called fidgety movements or dance-like movements usually appear in the hands, feet, and face. They can affect the way you walk, swallow and talk. The movements may be fluid or somewhat jerky. They can make it look as if you’re dancing, playing the piano, or even writhing in pain. The movements often get worse if you’re stressed or anxious and go away when you’re sleeping.
  • Milkmaid’s grip: Rather than holding your hand steady when you try to shake someone’s hand, you may grip and release your fingers over and over again. Doctors call this symptom milkmaid’s grip because it looks like you’re milking a cow.
  • Jack-in-the-box tongue: When you try to stick out your tongue, it may slide in and out of your mouth. This sign of chorea is sometimes called jack-in-the-box tongue or harlequin tongue.
  • Speech problems: Kids and adults may develop slurred speech as the muscles involved in making speech lose coordination.
  • Headaches and seizures: Kids who develop chorea after rheumatic fever can have seizures and headaches. Some children also have behavioral and emotional issues.

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