Chorea

Chorea is a movement disorder that causes your body to make involuntary, dance-like movements you cannot control. These jerky, unpredictable movements can range from mild fidgeting to severe, disabling motions that interfere with everyday activities like eating, walking, and speaking.

Table of contents

• What is chorea?
• Signs and symptoms
• Related movement patterns
• Causes and risk factors
• How chorea is diagnosed
• Treatment approaches
• Outlook and prognosis
• Living with chorea

What is chorea?

The word chorea comes from the Greek word meaning “to dance.” Chorea is a movement disorder where your body makes excessive, spontaneous movements that are irregularly timed, non-repetitive, randomly distributed, and sudden in character.^[1] These movements are involuntary, meaning you cannot control them.

Chorea affects various parts of your body and can interfere with speech, swallowing, posture, and walking. The movements disappear when you are asleep.^[2] In milder cases, chorea may appear purposeful, making you look fidgety and clumsy. In severe cases, it can cause a continuous flow of disabling, violent movements.^[1]

Chorea results from problems in specific parts of your brain called the basal ganglia, which are clusters of nerve cells that help control movement. The condition involves a functional problem in the brain’s motor circuit, where signals that normally regulate movement become disrupted. This disruption can happen because of structural damage, selective death of nerve cells, blocked chemical messengers in the brain, metabolic problems, or autoimmune conditions.^[2]

Signs and symptoms

Chorea symptoms usually depend on the underlying condition causing them. The movements can be fast or slow, and a person may appear to be writhing and have no bodily control. These movements have also been described as dance-like or similar to piano playing.^[3]

A common symptom is called “milkmaid’s grip.” People with this condition don’t have coordinated hand muscles and will squeeze and release their hand repeatedly, as if milking a cow. Another symptom is involuntarily sticking out the tongue, sometimes called “jack-in-the-box tongue” or “harlequin’s tongue.”^[3][5]

People with chorea often show motor impersistence, which means they cannot maintain a sustained posture or action. When attempting to grip an object, they alternately squeeze and release. When trying to stick out their tongue, it often pops in and out. People often drop objects involuntarily.^[5]

The movements typically involve both the muscles close to the body’s center (proximal muscles) and those farther from the center (distal muscles), such as in the hands and feet. Chorea can affect various body parts and interfere with speech, swallowing, posture, and gait.^[1][5]

Some people with chorea may not be aware of their movements, a condition called anosognosia. This lack of awareness can make chorea less bothersome to the person experiencing it, even though it may be socially embarrassing or concerning to caregivers.^[6]

Related movement patterns

Chorea is often accompanied by related movement patterns that differ mainly in the speed and severity of movements.^[1]

Athetosis is a slower form of chorea. The slowed movements have a writhing, squirming, or twisting motion. Because of the slowness, the movements have a snake-like appearance. When movements combine features of both chorea and athetosis, the condition is called choreoathetosis.^[5]

Ballism (also called ballismus) is a very severe form of chorea where violent flinging of the arms or legs is observed. These movements are continuous, violent, and coordinated, causing the limbs to be flung about. Ballism usually affects only one side of the body (hemiballism), but rarely, it may affect both sides (biballism).^[1][5]

Causes and risk factors

The causes of chorea are extensive and include both hereditary and acquired conditions. Overactivity of a chemical messenger called dopamine in the part of your brain that controls movement causes chorea.^[9]

Hereditary causes

Hereditary forms of chorea tend to develop gradually and are generally symmetrical, meaning they affect both sides of the body equally. The most common hereditary cause is Huntington disease, an inherited condition caused by a genetic change in the HTT gene. People who have a parent with Huntington disease have a 50% chance of inheriting the condition.^[7]

Other hereditary causes include benign hereditary chorea, chorea-acanthocytosis, Wilson disease, and various other genetic conditions.^[2][6]

Acquired causes

Acquired forms of chorea are more likely to be acute or develop over weeks to months (subacute) and can be asymmetrical or affect only one side of the body (unilateral).^[2]

Common acquired causes include:^[2][9]

• Medications: Levodopa (used for Parkinson’s disease), antipsychotic drugs (neuroleptics), some anti-seizure medications, and oral contraceptives can cause chorea.
• Infections: Sydenham chorea is a complication of rheumatic fever following streptococcal infection. Viral infections can also cause chorea.
• Autoimmune conditions: Systemic lupus erythematosus, antiphospholipid antibody syndrome, and other autoimmune disorders can lead to chorea.
• Metabolic and endocrine disorders: Hyperthyroidism, hypo- or hyperparathyroidism, and hypo- or hyperglycemia can cause chorea.
• Stroke or brain injury: Focal damage to the basal ganglia from stroke or structural lesions can cause acute onset of chorea.
• Pregnancy: A condition called chorea gravidarum can occur during pregnancy.
• Drugs of abuse: Cocaine and amphetamines can cause chorea.
• Toxins: Carbon monoxide poisoning or mercury poisoning can lead to chorea.

Risk factors

Anyone can develop chorea. You may be more at risk if you:^[9]

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

How chorea is diagnosed

Diagnosis of chorea is challenging because the movements look the same regardless of the underlying cause. Differentiating chorea from other movement disorders requires careful observation of your entire body throughout the clinical encounter.^[6]

Medical history and examination

A complete medication and family history are required to investigate acquired or hereditary causes. However, a genetic cause cannot be dismissed based solely on a negative family history, as relatives may have been misdiagnosed or the condition may occur sporadically.^[6]

During a neurological examination, your doctor will test your motor symptoms (such as reflexes, muscle strength, and balance), sensory symptoms (including sense of touch, vision, and hearing), and psychiatric symptoms (such as mood and mental status).^[13]

The time course and onset of chorea can offer valuable clues for diagnosis. In general, genetic causes are associated with chronic chorea that develops slowly, whereas acquired causes commonly result in acute or subacute chorea that appears suddenly or over weeks to months.^[6]

Diagnostic tests

Several tests may be used to determine the cause of chorea:^[13]

• Laboratory tests: Blood tests to check for metabolic problems, thyroid function, blood sugar levels, immune markers, and other factors.
• Brain imaging: MRI or CT scans can show detailed images of the brain and may reveal changes in areas affected by various conditions, or rule out other problems like stroke.
• Genetic testing: If symptoms strongly suggest a hereditary cause like Huntington disease, genetic testing can confirm the diagnosis.
• Neuropsychological testing: Tests to check memory, reasoning, mental agility, language skills, and spatial reasoning.

Treatment approaches

Treatment of chorea focuses on addressing the underlying cause when possible and managing symptoms to improve quality of life.^[1]

When treatment is needed

Not all cases of chorea require treatment. Because some people with chorea may not be aware of their movements, treatment decisions should consider whether the movements interfere with daily activities, cause safety concerns, or result in physical harm.^[11]

Treatment is typically recommended when chorea:^[11]

• Interferes with activities of daily living such as dressing, eating, chewing, or personal hygiene
• Causes safety concerns or physical harm, such as injuries to the tongue or mouth, falls, or difficulty maintaining balance
• Affects the ability to sleep or causes falls from bed
• Puts caregivers or others nearby at risk of injury

Treating underlying causes

When possible, treating the root cause of chorea is the primary approach. For example, autoimmune chorea syndromes such as systemic lupus erythematosus may respond to treatment with glucocorticoids, plasma exchange, or intravenous immunoglobulin. In Wilson disease, copper-reducing therapies can slow or halt progression.^[2]

If chorea is medication-induced, adjusting or stopping the offending medication may resolve symptoms.^[9]

Symptomatic treatment

The most widely used medications for treating chorea symptoms are:^[8][12]

Dopamine-depleting agents reduce the amount of dopamine available in the brain. These include tetrabenazine, deutetrabenazine, and valbenazine, which work by blocking a protein called vesicular monoamine transporter type 2 (VMAT2) that packages dopamine into storage compartments in nerve cells.

Antipsychotic medications block dopamine receptors in the brain. These include typical antipsychotics like haloperidol and fluphenazine, and atypical antipsychotics like risperidone, olanzapine, clozapine, and quetiapine.

GABAergic drugs such as clonazepam, gabapentin, and valproate can be used as additional therapy alongside other treatments.^[8]

Other interventions

Deep brain stimulation is an emerging technique that may benefit some patients, though it remains investigational for chorea.^[8]

Physical therapy, occupational therapy, and speech therapy can help manage coordination, balance, swallowing, and communication problems.^[18]

Outlook and prognosis

The prognosis of chorea depends heavily on its underlying cause. Some cases resolve with treatment of the underlying condition, while others, particularly hereditary forms like Huntington disease, are progressive and worsen over time.^[1]

For Huntington disease, people typically live an average of 18 to 20 years after diagnosis. During that time, the disease progresses and symptoms gradually worsen. The thinking problems eventually interfere with the ability to work, keep track of appointments, or manage finances. Coordination and balance problems can interfere with independence in daily activities.^[18]

Acute causes of chorea, such as those related to infections or metabolic problems, may improve or resolve completely when the underlying condition is treated.^[2]

Living with chorea

While chorea can significantly impact daily life, several strategies can help maintain independence and quality of life for as long as possible.

Staying active

Gentle exercises like yoga, walking, and prescribed physical therapy exercises can help maintain strength and may delay worsening of balance problems. However, you may need assistive devices like a walker or wheelchair for safety as the condition progresses.^[18]

Creating a safe home environment

Movement and balance problems make falls more likely. Modify your home by:^[16][18]

• Removing clutter from floors and eliminating throw rugs
• Ensuring electrical cords and cables aren’t tripping hazards
• Using lights in hallways and bathrooms at night
• Installing grab bars near toilets and in bathtubs
• Placing nonskid mats in showers or bathtubs
• Using a chair or bench in the shower

Adapting mealtimes

Chorea can make eating challenging. Helpful adaptations include:^[16][18]

• Using durable plastic plates, bowls, and non-stemmed glassware
• Using utensils with large, weighted handles
• Using nonskid placemats to prevent dishes from moving
• Sitting down at a table during meals
• Reducing distractions during mealtimes
• Eating high-calorie, nutrient-rich foods, as involuntary movements burn considerable calories

Managing daily tasks

Adaptive equipment can make daily activities easier:^[16]

• Choose clothing with elastic waists and slip-on or Velcro shoes instead of those with buttons and laces
• Sit down while getting dressed to maintain balance
• Use electronic toothbrushes
• Consider using liquid soap instead of bar soap

Maintaining mental and emotional health

Scheduling regular time with a counselor or therapist can help navigate the emotional challenges of living with chorea. Support groups, both in-person and online, can connect you with others dealing with similar issues.^[15]

Challenging your brain with engaging activities like reading, playing games, and solving puzzles may help keep your mind active. Pursuing creative activities such as music, art, or writing can add value to your life.^[18]

Building a care team

A multidisciplinary team of healthcare professionals can provide comprehensive support:^[14]

• Physical therapist to improve strength, mobility, balance, and function
• Occupational therapist to suggest methods for improving everyday tasks
• Speech pathologist to address difficulties with speaking and communication
• Nutritionist or dietitian to assist with diet and weight management
• Psychiatrist or psychologist to treat associated mental health conditions
• Counselor to help manage stress and emotions

Early intervention and continuous support are key to enhancing quality of life for people with chorea.^[6]