Huntington’s disease is a hereditary progressive neurodegenerative disorder. One hallmark of the condition is involuntary movements, which include akathisia (restlessness), dystonia (muscle spasms in the arms, head or trunk), and chorea.
Chorea is the most common involuntary movement problem in Huntington’s disease patients. It usually occurs during the early intermediate stage of the disease, several years after disease onset. Chorea diminishes at advanced stages of the disease, at which time dystonia emerges.
What is chorea?
The term “chorea” comes from the ancient Greek word “choreia,” which means dance. Chorea is characterized by brief and abrupt movements that are irregular and unpredictable. These movements often seem to flow from one body part to another, but they also can be confined to a single area in the body. They can interfere with swallowing, speech, posture, and walking. Chorea often leads to clumsiness, which causes the person to repeatedly drop objects. People who are not familiar with chorea also describe the movements as restlessness or fidgeting.
Chorea can occur with other abnormal movements, such as athetosis, a slower, writhing movement, referred to as choreoathetosis. It may occur simultaneously with dystonia.
Chorea symptoms in Huntington’s disease can improve through therapeutic intervention.
Dopamine is a signaling molecule in the brain that is involved in motor control. Increased levels of dopamine are thought to cause chorea in Huntington’s disease patients. So, the use of antidopaminergic agents is one approach to control chorea.
Two antidopaminergic agents Xenazine (tetrabenazine) and Austedo (deutetrabenazine) have been approved by the U.S. Food and Drug Ddministration (FDA) for the treatment of chorea in Huntington’s disease. Austedo is structurally related to Xenazine, but is more stable.
The antipsychotic medications Risperdal (risperidone) and Haldol (haloperidol) also are commonly used in Huntington’s disease patients. They do not only reduce chorea, but also help in managing psychiatric symptoms, such as irritability and psychosis.
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