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Chorea is a hallmark of Huntington disease (HD) along with cognitive decline and psychiatric impairment. It often develops early, gradually worsening and plateauing in late stages.1 Motor dysfunction, including chorea, decreases functional capacity, particularly in early HD.Chorea worsens weight loss5 and can compromise safety, including increasing fall risk. Treating chorea is an important part of HD management.

The pathophysiology and neurochemical bases of HD are complex and incompletely understood. Dopamine and glutamate transmission and interactions are affected, contributing to striatal and cortical vulnerability and to features such as chorea.8 Most agents investigated for HD chorea target these neurotransmitters and receptors. Neuroprotective trials often focus on agents that may prevent oxidative stress or glutamatergic changes related to excitotoxic stress.、

For this evidence-based guideline, we asked the following question: For adult patients with HD requiring symptomatic chorea therapy, what available pharmacologic agents effectively reduce chorea as measured by validated scales?