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What Causes Chorea?

What is chorea?

Chorea is a movement disorder that causes involuntary, unpredictable body movements. Chorea symptoms can range from minor movements, such as fidgeting, to severe uncontrolled movements of the arms and legs. It can also interfere with speech, swallowing, posture, and gait.

What are the symptoms of chorea?

Chorea symptoms usually depend upon the condition causing it. A common symptom is "milkmaid’s grip." People with this condition don’t have strong hand muscles and will squeeze and release their hand, as if milking. Another symptom is involuntarily sticking out the tongue.

Chorea movements can be fast or slow. A person may appear to be writhing in pain and have no bodily control.

Conditions associated with chorea and its symptoms include:

Huntington’s disease

Huntington’s disease is an inherited disease. It causes the breakdown of nerve cells in your brain. People with Huntington’s disease can experience chorea symptoms such as involuntary jerking or writhing. Milkmaid’s grip is also a common symptom. Chorea is more common in people with adult-onset Huntington’s disease. Over time, symptoms may get worse and movements may affect the legs and arms.


This condition is a very rare genetic disorder. It’s characterized by misshapen red blood cells. It causes neurological abnormalities and affects brain functioning. Chorea for this condition commonly involves abnormal arm and leg movements, shoulder shrugs, and pelvic thrusts. It can also involve rapid, purposeless movements of the face.

People with this form of chorea can also exhibit dystonia. This is characterized by involuntary muscle contractions of the mouth and face, such as:

  • teeth grinding
  • involuntary belching
  • drooling or spitting
  • lip and tongue biting
  • difficulty with speech or communication
  • vocal tics, such as grunting, involuntary speaking, or slurred speech

Sydenham’s chorea

This condition mainly affects children and adolescents, and follows a streptococcal infection. It can also be a complication of rheumatic fever. This type of chorea mainly affects the face, arms, and hands. It can impede voluntary movements, making it difficult to perform basic tasks such as getting dressed or feeding yourself.

It can also lead to:

  • frequently dropping or spilling items
  • abnormal gait
  • muscle weakness
  • slurred speech
  • diminished muscle tone

People with this chorea type often display milkmaid grip. Another common symptom is called "harlequin tongue." When a person with this symptom tries to stick their tongue out, the tongue pops in and out instead.

What are risk factors for chorea?

People with a history of rheumatic fever are more likely to experience chorea. This risk factor makes pregnant women more likely to have chorea gravidarum.

Other risk factors are related to risks for a specific disease. For example, Huntington’s disease is a hereditary disorder that may cause chorea. A person with a parent who has Huntington’s disease has a 50 percent risk of inheriting the disease, according to the Mayo Clinic.

What causes chorea?

Chorea is associated with a number of additional causes, some temporary and some chronic. These causes include:

  • AIDS
  • genetic conditions, such as Huntington’s disease
  • immune conditions, such as systemic lupus erythematosus
  • infection-related conditions, such as Sydenham’s chorea
  • medications, including levodopa and neuroleptics
  • metabolic or endocrine disorders, including hypoglycemia
  • pregnancy, known as chorea gravidarum

How is chorea diagnosed?

Because many conditions cause chorea, your doctor must take a thorough medical history to determine potential causes. To diagnose chorea, your doctor may ask:

  • When did the symptoms begin?
  • What makes the symptoms better or worse? Do your chorea symptoms tend to worsen when you’re stressed?
  • Do you have a family history of hyperthyroidism or Huntington’s disease?
  • What medications are you taking?

Some laboratory tests can indicate chorea. For example, a low copper level in your body can indicate Wilson disease, a genetic disorder that causes chorea. Tests for spiky erythrocytes or red blood cells can indicate chorea-acanthocytosis. Blood tests for parathyroid hormones or thyroid hormones can indicate metabolic or endocrine-related chorea.

For Huntington’s disease, imaging studies, such as MRI scans, can show brain activity to indicate the disease.

How is chorea treated?

Treatment of chorea depends on the type of chorea you have. It aims to treat the underlying condition, which will help with chorea symptoms. For example, Sydenham’s chorea may be treatable with antibiotics. Huntington’s disease chorea can be treated with antipsychotic drugs, as well as other medications. Chorea due to Parkinson’s disease has no cure but symptoms can be controlled.


Most medications for chorea affect dopamine. Dopamine is a neurotransmitter, or brain chemical, that controls movement, thinking, and pleasure in your brain, among other things. Many movement disorders are associated with dopamine levels. These disorders include Parkinson’s disease and restless legs syndrome.

Some medications block dopamine receptors so your body can’t use the chemical. Many of these are antipsychotic drugs that seem to reduce chorea. These drugs include:

Other drugs reduce the amount of dopamine in the brain, such as reserpine and tetrabenazine (Xenazine). Medications known as benzodiazepines, such as clonazepam (Klonopin), may also help to reduce chorea.

Anticonvulsants, which reduce spontaneous movements, can also reduce chorea symptoms.


Deep brain stimulation is a surgical approach that shows promise for chorea treatment. This treatment involves implanting electrodes in your brain to regulate nerve impulses. If chorea doesn’t respond to medications, your doctor may recommend deep brain stimulation. This procedure does not cure chorea but can reduce its symptoms.

Home care

Chorea increases a person’s likelihood for falls. Home care measures include installing non-slip surfaces on stairs and in bathrooms to prevent injury. Talk to your doctor about other ways to modify your home for safety.

What is the outlook for chorea?

The outlook for chorea depends on the condition causing it. Antibiotics can cure Sydenham’s chorea, while there is no cure for Huntington’s disease. Women with chorea gravidarum typically stop having symptoms within six weeks after giving birth. People with metabolic or endocrine-related chorea typically stop having symptoms once a doctor treats the imbalance. Whatever the condition causing chorea, your doctor will develop a treatment plan to help you manage and control your symptoms.

Content licensed from:

Written by: Rachel Nall
Medically reviewed on: Apr 28, 2016: University of Illinois-Chicago, College of Medicine

This feature is for informational purposes only and should not be used to replace the care and information received from your health care provider. Please consult a health care professional with any health concerns you may have.
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