What Is Chorea?

Chorea is a type of involuntary movement characterized by brief, random, recurrent writhing or twisting of any part of the body that gives the impression of restlessness to the observer. It can occur due to certain neurological conditions, systemic illnesses that affect the brain, or a side effect of medication.

Chorea is considered a symptom of disease, and not an independent condition. It is generally one of several symptoms, and it rarely occurs on its own. If you have chorea, you will need a thorough medical evaluation to identify the underlying cause.

Chorea can often be managed with medication, and the underlying cause usually needs to be treated as well.

This article describes the symptoms and causes chorea, including associated symptoms and underlying conditions. It also explains how chorea is diagnosed and treated.

Chorea Symptoms

You can have a number of movement patterns with chorea. While chorea can manifest with a variety of physical motions, you may notice that you specifically have only a few types of movements and that it typically affects only certain parts of your body.

As the underlying condition progresses, you can start to experience the movements more frequently, you can have movement patterns of chorea that you didn’t experience before, and they can affect additional areas of your body.

New-onset chorea due to a medical condition might occur unexpectedly, infrequently, and for a few seconds or minutes at a time. As the condition worsens, you can experience chorea almost all the time.

Chorea that occurs as a drug side effect can be intense right when it begins and may occur several times a day and/or for prolonged periods of time.

Symptoms that are considered to be chorea include:

• Fidgeting involuntarily
• Inability to maintain an ongoing motor activity, such as maintaining a constant grip or holding the tongue out
• Abrupt, interrupted speech
• Involuntary vocalization

A key characteristic of chorea is that it is erratic and irregular, and it is not rhythmic or pulsing. It can appear to “flow” from one muscle to another or from one part of the body to another. It is not associated with changes in consciousness.

Associated Symptoms

The conditions that cause chorea often also cause other movements or neurological symptoms along with chorea. The presence of absence of these associated symptoms can help guide your diagnosis.

Associated symptoms that commonly occur with chorea include:

• Athetosis (slow, involuntary writhing movements)
• Ballismus or hemiballismus (spontaneous involuntary movements)
• Cognitive decline (difficulty thinking) or dementia
• Tremors
• Spasms
• Muscle twitches
• Weakness
• Sensory changes

Many of these associated symptoms appear similar to chorea and to each other. They might each feel different from each other, however.

Complications of Chorea

Aside from being distressing, the involuntary movements of chorea can cause health problems or injuries.

Adverse complications that can be associated with chorea include:

• Swallowing problems, choking
• Speech problems
• Muscle spasms and stiffness
• Pain or discomfort
• Injury

Getting treatment for your chorea—even if the treatment doesn’t cure the underlying cause—can help prevent some of these issues.

Causes of Chorea

Chorea can develop due to conditions that affect the brain. Genetic abnormalities are what usually cause chorea that starts in adults. Huntington’s disease is the genetic disease most commonly associated with chorea. In children, the most common form of chorea is Sydenham chorea.

Conditions that can cause chorea include:

• Huntington’s disease: A hereditary disease that causes gradually worsening dementia, loss of motor control, and chorea.
• Sydenham chorea: This illness can develop in children after a group A streptococcal infection. It is associated with rheumatic fever and can last for several years after the infection has resolved. Sydenham chorea may resolve on its own.
• Medication side effect: Chorea can develop as a side effect of medications, especially medications used to treat Parkinson’s disease.
• Parathyroid hormone abnormality: Decrease in parathyroid hormone can result in low levels of calcium, which can produce generalized or focal chorea.
• Thyroid disease: Sometimes thyroid disease is associated with chorea. This is believed to be related to the direct effect of the high levels of thyroid hormones in the brain.
• Paraneoplastic: Rarely, cancerous tumors can produce antibodies that can attack the brain causing chorea.
• Systemic lupus erythematosus (SLE): This autoimmune condition causes a number of symptoms, and in rare instances, it can cause chorea.
• Chorea gravidarum: Chorea due to pregnancy is not common, and it generally resolves after delivery.
• Brain damage: A stroke, brain tumor, injury, or infection can damage parts of the brain associated with movement, causing chorea.

Brain Involvement

Chorea can occur when there is a structural or functional problem with the basal ganglia and the thalamus, which are areas of the brain that control movement. The cause can be related to dysfunction of the neurotransmitters in these regions of the brain, especially dopamine, or to a structural issue.

The basal ganglia includes several structures in the brain—the caudate, globus pallidus, putamen, subthalamic nucleus, and the substantia nigra. These small structures work together to regulate the movement of the body. Damage can affect the structures of one or both sides of the brain.

However, damage or dysfunction of these structures does not always result in chorea and can cause other neurological or movement problems.

Diagnosing Chorea

Generally, diagnosis of chorea is based on your symptom history and your healthcare provider’s observation during your physical examination. Your healthcare provider will do a thorough neurological and cognitive examination, which will help in identifying conditions that cause chorea.

A major component in the evaluation of chorea is the diagnosis of your underlying medical issue. This can include diagnostic testing. Your diagnostic tests would be directed by your medical history, your family history, and your physical examination.

Some of the diagnostic tests you may need in the evaluation of the cause of chorea include:

• Imaging if there is concern about a problem in the brain or cancer in any location of the body
• A genetic test if there is a possibility that you could have Huntington’s disease
• Hormone levels if you could have thyroid or parathyroid disease
• Immune markers if you could have SLE or another autoimmune condition

Treatment for Chorea

There are several medications used to treat chorea. If you have a treatable underlying cause, such as thyroid disease or lupus, the treatment for the underlying condition may stop you from continuing to experience chorea. If your chorea is a side effect of medication, your healthcare provider may stop or adjust the medication to manage your chorea.

Some medications can reduce the movements. You may need to use medication for the long term if you have an irreversible cause of chorea, such as Huntington’s disease or a stroke.

Medications used to treat chorea include:

• Vesicular monoamine transporter type 2 (VMAT2) inhibitors: Tetrabenazine and deutetrabenazine are approved to treat chorea in Huntington’s disease, and valbenazine is used to treat certain movement disorders.
• Medications that interact with dopamine receptors, including antipsychotics are often used to manage chorea.
• Anti-epileptics and anti-glutamatergics are used off-label to manage this condition.

Deep brain stimulation is a procedure in which a stimulation device is implanted in the brain. In some situations, this method can be used to manage chorea when medication is not effective.

Summary

Chorea is a very noticeable symptom that can occur as a result of several different conditions, including Huntington's disease or side effects of medication. Sometimes chorea resolves on its own. When it doesn't, you may need treatment for the underlying condition as well as treatment to reduce the symptom of chorea.

Talk to a healthcare provider if you experience any type of involuntary movements, including the uncontrollable twisting, jerking, and writhing movements seen in chorea. You may undergo diagnostic tests as part of your evaluation. Some cases of chorea are reversible, while others may require lifelong treatment.