Chorea

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.

How is chorea diagnosed?

Because so many conditions and disorders can cause chorea, your doctor will ask a range of questions about your family history, the medications you take, and any symptoms you’re having besides uncontrollable movements. Your doctor will also conduct a physical exam and ask how often and how long your uncontrolled muscle movements have been happening and whether they get worse during times of stress.

To determine the cause of chorea, your doctor may order:

• Blood tests to check for infections, lupus, thyroid levels or other endocrine or metabolic abnormalities.
• Imaging studies such as magnetic resonance imaging (MRI) scan or a computed tomography (CT) scan to look for changes in your brain.
• Genetic testing to see if you have the gene for Huntington’s disease.

How do I know if I have chorea?

If you have irregular muscle movements that you can’t control, you should see your doctor. Your risk of chorea is higher if you have an autoimmune disease, a family history of Huntington’s disease, or if you’ve had rheumatic fever.

What are the treatments for chorea?

Some people may not be aware of their chorea; others may view their mild symptoms simply as a nuisance or perhaps as a social embarrassment. However, if your chorea is severe or disabling or interferes with the quality of your life, you should see your doctor for treatment.

The type of treatment depends on what is causing the movement disorder.

• If Huntington’s disease is the cause, although doctors can’t cure the disease, they can prescribe medications that may be able to control the movements. Doctors prescribe deutetrabenazine (Austedo®) or tetrabenazine (Xenazine®) to control muscle movements of Huntington’s chorea.
• If chorea is part of a tardive dyskinesia syndrome, the medications valbenazine (Ingrezza®) and deutetrabenazine are usually considered.
• Amantadine is sometimes tried if levodopa (Parkinson medication) is the cause of the chorea. Antipsychotics are sometimes considered if you have both chorea and psychiatric conditions present, such as agitation, irritability, anxiety, depression, suicide and apathy.
• Kids who have Sydenham chorea usually get better in less than two years without any treatment at all. If the chorea is severe, doctors can prescribe medication (for example, corticosteroids) to help control the movements. Doctors also prescribe antibiotics to kill the rheumatic fever infection.
• If chorea is caused by a medication, stopping the medication may help lessen the abnormal muscle movements.
• If chorea is caused by endocrine or metabolic disorders (such as hyperthyroidism, hypoglycemia, hypoparathyroidism, hypocalcemia, hypomagnesemia), treating those conditions usually lessens the abnormal muscle movements.
• The use of deep brain stimulation to reduce chorea has been tried in some patients but is considered an experimental treatment for this disorder. Deep brain stimulation may be an option if chorea is severe and all other treatments have been tried and failed.

What are the side effects of the treatment for chorea?

Deutetrabenazine and tetrabenazine can cause suicidal thoughts and actions in people with Huntington’s disease. Before taking these drugs, it’s very important to tell your doctor if you’re depressed. People who have a history of depression should not take these medications.

Taking corticosteroids for a long time can weaken your bones and lead to a condition called osteoporosis. People have also reported weight gain and high blood pressure after long-term use of corticosteroids.

All medications have potential side effects. Talk to your doctor about the risks and benefits of all medications considered to treat your chorea. You and your doctor can decide which medications or treatment plan may be best suited for you based on your symptoms and the cause of the chorea.

What are the complications associated with chorea?

Depending on the cause of chorea, complications can be severe. Huntington’s disease is an ongoing disease that gets worse over time and has no cure. Although there is no cure for lupus, doctors can help you manage the disease with medications. Children who develop Sydenham chorea after rheumatic fever usually recover without long-term medical problems.

What can I do to help relieve symptoms of chorea?

Only a doctor can help you relieve the symptoms of chorea. You should talk to your doctor about medications that can control the muscle movements.

How can you prevent chorea?

You can’t prevent some of the diseases and conditions that cause chorea, such as Huntington’s disease and lupus. To avoid rheumatic fever, children with a sore throat should get tested for strep. If your child’s doctor prescribes antibiotics for strep throat, be sure to take them as directed and finish all the medicine.

What is the outlook for patients who have chorea?

The outlook depends on what’s causing the involuntary muscle movements. People with Huntington’s disease can live 15 to 25 years after they develop symptoms. The condition slowly gets worse over time and makes it very difficult to do everyday activities like dressing, bathing and eating.

Children who develop Sydenham chorea after rheumatic fever usually recover within 2 years. Some children may have lingering behavioral or emotional issues, such as obsessive compulsive disorder (OCD). These issues can last a lifetime.

Chorea itself usually can be managed with currently available treatments.

When should I call my doctor about chorea?

You should call your doctor right away if you notice any unusual muscle movements or tics. Although you may not have chorea, it’s important to get it checked out to determine what’s causing the movements.