How to Manage Chorea in Huntington’s Disease

More than 90 percent of people with the genetic condition Huntington’s disease develop chorea — involuntary movements that can interfere with daily activities, communication, and independence.

Chorea is a movement disorder that causes brief, irregular, involuntary movements that may affect the face, trunk, arms, and legs. These movements can look like fidgeting or restlessness and may vary from subtle to more pronounced over time.

These “extra wiggly movements” come from overactivity of the hormone dopamine, in the part of the brain that regulates movement, says Emily Hill, MD, a movement disorder physician at UC Health in Cincinnati.

“In Huntington’s disease, those circuits are affected in a way that causes excessive involuntary movement (movements that your body makes that you can’t control), which shows up as chorea,” Dr. Hill says.

Not everyone with chorea needs medication: Treatment decisions are typically based on how much symptoms interfere with daily life and safety, says Hill.

“Is chorea getting in the way of getting dressed? Is it making walking more difficult?” she says. When treatment is needed, there are several types of medications that may help.

FDA-Approved Treatments

The main treatments for chorea block or reduce dopamine.

When dopamine is in the deep parts of the brain, it primarily affects movement — a different function from being a "happiness chemical,” which is what most people are familiar with, says Hill. “Too much movement is often related to too much dopamine,” she adds.

Three medications, known as vesicular monoamine transporter 2 (VMAT2) inhibitors, are approved by the U.S. Food and Drug Administration (FDA) to treat chorea in Huntington’s disease.

• Deutetrabenazine (Austedo) reduces dopamine signaling and is designed to last longer in the body than tetrabenazine, allowing less frequent dosing.
• Tetrabenazine (Xenazine) works in a similar way to deutetrabenazine by reducing the release of dopamine into the synapse, which helps calm excessive involuntary movements.
• Valbenazine (Ingrezza) is a newer VMAT2 inhibitor that also works similarly to control chorea.

Other Medications That May Help Control Chorea

If first-line VMAT2 inhibitors aren’t effective or aren’t well tolerated, doctors may consider other medications to suppress chorea, including the following:

• Amantadine (Gocovri ER, Osmolex ER) may help reduce involuntary movements in some people, though results vary.
• Clonazepam (Klonopin), a benzodiazepine, may help reduce excessive movements and can also ease anxiety that may worsen symptoms.
• Levetiracetam (Keppra, Elepsia XR, Spritam), an anticonvulsant, is sometimes used off-label to help control involuntary movements.

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Antipsychotics With Anti-Chorea Effects

Antipsychotic medications are also used off-label to suppress chorea, though the evidence is limited. They may also be prescribed to help with two things at once in people with agitation. Options include:

• Olanzapine (Zyprexa)
• Aripiprazole (Abilify, Aristada)
• Risperidone
• Haloperidol, typically reserved for severe or hard-to-treat cases

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Treatment also has limits over time. Anti-chorea therapy should be reevaluated regularly, because as Huntington’s progresses, the focus of care may shift.

“Typically, as symptoms progress, the thinking, memory, and behavior symptoms are the more disabling features,” says Hill.

Also, some medications that help with some symptoms may cause side effects that include worsening other symptoms. People with Huntington’s disease may also develop other movement problems like dystonia (abnormal muscle contractions) or even Parkinson-like symptoms, she says.

Lifestyle strategies can play an important role in managing chorea, particularly when symptoms are mild or fluctuate. They include:

• Reducing Stress and Triggers Chorea can worsen with stress and anxiety, so managing emotional triggers may help reduce symptoms.
• Creating Structured Routines Caregivers can help by setting schedules that allow extra time for dressing, hygiene, meals, and daily activities.
• Focusing During Eating Chorea can make chewing and swallowing difficult, which can increase the risk of choking. Removing distractions during meals and selecting foods that are easier to eat can help.

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• Chorea is common in Huntington’s disease, but it does not always need treatment right away.
• When chorea starts to affect safety or daily life, VMAT2 inhibitors are the main FDA-approved treatment option.
• Medication side effects and disease changes mean that the best treatment can change over time. Regular check-ins with a neurologist are essential.
• Lifestyle changes, like reducing stress, allowing extra time for tasks, and making meals easier can also help.