Chorea

What is chorea?

Chorea is a symptom that causes involuntary, irregular or unpredictable muscle movements. It affects your arms, legs and facial muscles. Chorea comes from the Greek word that means “to dance.” It can make you look like you’re dancing, restless or fidgety.

Chorea itself isn’t life-threatening. It may occur on its own but it’s most often associated with conditions like Huntington’s disease.

What are the symptoms of chorea?

The most common symptoms of chorea are movements that your body makes that you can’t control. They include:

• Involuntary muscle movements: You may fidget, twist or jerk your arms, legs and/or the muscles in your face. They can affect the way you walk, swallow and talk.
• Grip changes: When you’re extending your hand or trying to shake someone’s hand, you may grip and release your fingers repeatedly. Healthcare providers describe this motion as milkmaid’s grip because it’s the same movement a person makes when milking a cow.
• Tongue movements: Your tongue may move in and out of your mouth. This is known as a jack-in-the-box tongue. Tongue movements may occur in other directions as well.

Children diagnosed with chorea may have the following symptoms in addition to the listed involuntary movements:

• Headaches.
• Seizures (associated with rheumatic fever, amongst other rare conditions).
• Slurred speech.

What makes chorea get worse?

Chorea symptoms may get worse if you’re:

• Stressed.
• Anxious.
• Sick from another medical illness or having a side effect from a medication.

What types of movements related to chorea happen with neurological conditions?

A neurological condition affects your brain and nerves. Specific movements related to chorea can indicate an underlying neurological condition:

• Athetosis is a continuous stream of slow, twisting or squirming-like motions usually involving your 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 your body (hemiballism).

A healthcare provider, like a neurologist, will examine your symptoms and diagnose the underlying cause. If you’re unsure what type of provider to see, first speak with a primary care physician and they can make a recommendation if you need to see a neurologist or a provider who specializes in movement disorders.

What causes chorea?

Overactivity of a hormone called dopamine in the part of your brain that affects your movement (basal ganglia) causes chorea.

The most common causes of chorea include:

• An underlying genetic condition like Huntington’s disease.
• A movement disorder like Parkinson’s disease or tardive dyskinesia.
• An autoimmune condition like lupus, multiple sclerosis or Sjogren syndrome.
• An endocrine or metabolic condition like hyperthyroidism or kernicterus.
• An infection or a complication from an infection (rheumatic fever leads to Sydenham chorea).
• A stroke.
• Pregnancy (chorea gravidarum).
• A brain tumor near the basal ganglia.

In addition, other causes of chorea include:

• Medications: Levodopa, tricyclic antidepressants, some antiseizure medications and antipsychotic medications.
• Drugs: Cocaine and amphetamines.
• Toxins: Carbon monoxide poisoning or mercury poisoning.

What are the risk factors for chorea?

Chorea can happen to anyone. You may be more at risk of developing chorea if you:

• Have a family history of Huntington’s disease.
• Had rheumatic fever, an infection or head trauma during childhood.
• Have an autoimmune condition.
• Are over 40 years old.

What are the complications of chorea?

Depending on the cause of chorea, complications can be severe and may include:

• Difficulty swallowing foods and beverages (malnutrition).
• Behavioral or emotional challenges like obsessive-compulsive disorder. This is common among children and patients with Huntington’s disease.
• Physical injury from involuntary movements.
• Social stigmatization.

Treatment for chorea could include:

• Taking medications to lessen involuntary movements.
• Stopping a medication that may cause your symptoms (don’t stop taking a medication unless your healthcare provider says you should).
• Treating any underlying health conditions.
• Managing your stress or anxiety by working with a mental health professional.
• Changing your nutritional intake (nutrition management) if symptoms affect your ability to swallow by adding easier-to-chew foods into your diet.
• Surgery to remove a brain tumor.
• Deep brain stimulation for some types of severe chorea or if other treatments don’t work.

Sometimes, very mild cases of chorea that don’t interfere with your daily activities don’t need treatment. A healthcare provider will discuss your treatment options and let you know what they recommend.

What medications treat chorea?

The type of medication your healthcare provider prescribes to decrease your chorea symptoms varies based on the cause:

• Huntington’s disease: Medications include tetrabenazine, deutetrabenazine (Austedo®) or tetrabenazine (Xenazine®).
• Tardive dyskinesia: Medications include tetrabenazine, valbenazine (Ingrezza®) and deutetrabenazine (Austedo®).
• Parkinson’s disease medication levodopa: Alternative medication includes amantadine.
• Rheumatic fever: Medications include corticosteroids or antibiotics.
• Dopamine management: Adjusting or reducing the doses, spreading the doses out and/or using extended-release formulations of medications like antipsychotics or antiseizure medications. As antipsychotics and antiseizure medications may cause symptoms of chorea, your healthcare provider will monitor your progress on these medications closely.

Are there side effects of the treatment?

The side effects are different for each type of treatment or medication for chorea.

For example, tetrabenazine, and to a lesser extent deutetrabenazine and valbenazine, can cause suicidal thoughts and actions in people with Huntington’s disease. Before taking these medications, it’s very important to tell your healthcare provider if you have depression. People who have a history of depression shouldn’t take them without approval of their provider. If you experience suicidal thoughts, contact your healthcare provider and dial 988 (call or text) to reach the Suicide and Crisis Lifeline. Someone is available to help you 24/7.

Your healthcare provider will let you know about the side effects of any medication before you start taking it so you can make an informed decision about your health.

What can I eat/drink with chorea?

If symptoms of chorea affect your ability to eat or drink, your healthcare provider may recommend a swallow study and possible changes to your diet, like adding liquid or soft foods that are easy to chew and swallow. Your healthcare provider may suggest you meet with a nutritionist to make sure you get enough nutrients during each meal. If you’re unable to eat, contact your healthcare provider.

How soon after treatment will I feel better?

Some cases of chorea resolve after treatment. Other people may have to continuously manage symptoms of chorea if the underlying condition is chronic. For children diagnosed with Sydenham chorea after rheumatic fever, they usually see their symptoms resolve within two years without treatment or sooner with treatment.

Can chorea be prevented?

You can’t prevent all causes of chorea. You can take steps to reduce your child’s risk of developing rheumatic fever by taking them to a healthcare provider to get tested for strep throat if they have a sore throat. If your child’s doctor prescribes antibiotics for strep throat, be sure your child takes them as directed and finishes all the medicine.

What’s the outlook for chorea?

Your chorea outlook depends on the cause. Your healthcare provider will discuss your outlook with you.

For example, Huntington’s disease is progressive. This means that the condition slowly gets worse over time. It makes it very difficult to do everyday activities like getting dressed, bathing and eating. Studies estimate that people diagnosed with Huntington’s disease can live 10 to 30 years after they develop symptoms.

Children who develop Sydenham chorea after rheumatic fever usually recover within two years. Some children may have lingering behavioral or emotional complications, such as obsessive-compulsive disorder (OCD).

When should I see a healthcare provider?

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

When should I go to the ER?

Visit the emergency room if an involuntary movement causes you to injure yourself. It’s common for people diagnosed with chorea to fall. Your care team can help you make adjustments around your home to prevent or reduce your risk of falls and injuries.

If your child has a seizure for the first time after rheumatic fever, contact 911 or your local emergency services number.

What questions should I ask my doctor?

• What caused my symptoms?
• If I treat the underlying cause of chorea, will my symptoms go away?
• What type of treatment do you recommend?
• Are there side effects of the treatment?
• If I have depression, what type of medication do you recommend?
• Can you recommend a support group or counseling?

Is chorea always Huntington’s disease?

No, not all cases of chorea are the result of Huntington’s disease, but Huntington’s disease is a common cause of chorea.

Is chorea associated with Parkinson’s disease?

There are many causes of chorea, and the movement condition called Parkinson’s disease is one of them.