Huntington’s disease: treatment for chorea

Chorea is a movement disorder where a person experiences involuntary, irregular, uncontrolled movements. These movements can vary in severity. Mild chorea symptoms can seem like restlessness or fidgeting and may occur infrequently. Severe symptoms can be violent, frequent, and disabling. While chorea can have numerous causes, it is most often associated with Huntington’s disease.

What causes Huntington’s disease?

Huntington’s disease is a neurodegenerative disorder that causes a loss of nerve cells and neural functioning in the brain. This breakdown and loss of nerve cells is caused by a genetic mutation of the huntingtin (HTT) gene, which a person with the disorder would have inherited from their biological parents. This mutation impairs the body’s ability to make HTT protein, which plays an important—but not fully understood—role in the health of neurons in the brain.

Symptoms of Huntington’s disease

In addition to chorea, Huntington’s disease causes cognitive and psychiatric problems. People with the condition can experience difficulty with balance, coordination, thinking, organization, and memory. They may also experience changes in behavior, irritability, insomnia, obsessive-compulsive disorder, and depression. As the disease progresses, symptoms become more severe, leading to problems with swallowing, speech, and communication. People with Huntington’s disease will require caregiving and will be fully dependent on caregivers in the advanced stages of the disease.

Treatment for chorea and other symptoms

There is no cure for Huntington’s disease, and no treatments that can alter the course of the disease or slow its progression. However, new treatments are continually being explored by scientists. There are also treatments that can help a person ease and manage specific symptoms, including medications that can help manage and control chorea. Examples include the following.

VMAT2 inhibitors

Vesicular monoamine transporter 2 inhibitors (VMAT2 inhibitors) work by regulating chemical signaling in the brain. VMAT2 inhibitors are used to treat chorea caused by Huntington’s disease as well as involuntary movements associated with other disorders.

Neuroleptic drugs

Neuroleptic medications are used to treat psychosis and are a mainstay of treatment for serious mental illnesses. Neuroleptics can also help control involuntary movements. This may be a treatment option for people with Huntington’s disease who are experiencing chorea as well as psychiatric symptoms. In some cases, people with Huntington’s disease experience psychosis.

Benzodiazepines

These are depressant medications that act on receptors on nerve cells and help slow down activity in the nervous system, including the brain. Benzodiazepines are used to treat a number of conditions, including seizure disorders. Several are indicated to help manage chorea symptoms caused by Huntington’s disease.

Antidepressants, antianxiety medications, and mood stabilizers

Mental health conditions like depression, anxiety, and bipolar disorder affect many people who have Huntington’s disease. Medications are often part of treatment for these conditions and may be part of a treatment plan for Huntington’s disease.

Treatment will vary from person to person. Treatment will also evolve with time, to keep up with changing symptoms and the changing needs of a person living with Huntington’s disease.

Questions when starting a medication

Medications that act on the nervous system can cause side effects. Managing the symptoms of Huntington’s disease often involves balancing the benefit of a medication against the potential side effects of that medication. Again, treatment is individualized, and a healthcare provider (HCP) will be your best source of information.

Some questions to consider when starting a medication:

• What is the medication and what symptoms does it treat?
• Why is this medication being recommended?
• What side effects can the medication cause?
• Can this medication interact with other drugs? (Keep a list of all medications you take and share this with your HCP.)
• What do I do if I experience a side effect or drug interaction?
• How will we know if the medication is working?
• What is the next step if this medication doesn’t work?
• What does the medication cost?

Always know the name of the medication, the dose of the medication, and instructions for taking a medication. Before you or a loved one begins taking a medication, talk to your HCP about any concerns you have.