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Practical recommendations for the treatment of chorea associated with Huntington's disease: An expert consensus
Erin Furr Stimming1, Jee Bang2, Amy Brown3
1Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX, USA.
Insights
Huntington's disease (HD) chorea treatment is underutilized despite its impact. This review offers practical strategies for pharmacologic management, emphasizing individualized care and multidisciplinary approaches to improve patient quality of life.
Area of Science:
- Neuroscience
- Genetics
- Pharmacology
Background:
- Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the huntingtin gene.
- Chorea, involuntary movements, are a hallmark symptom of HD, significantly impacting daily life.
- Pharmacologic treatment for chorea in HD is underutilized due to various clinical barriers.
Purpose of the Study:
- To discuss current practices and challenges in the pharmacologic treatment of chorea in Huntington's disease.
- To highlight practical, real-world strategies for evaluating and treating HD chorea based on expert consensus.
- To improve the optimization of pharmacologic therapy for chorea in individuals with HD.
Main Methods:
- A consensus meeting of 11 North American HD specialists from the Huntington Study Group (HSG) Motor Treatment Task Force.
- Supplementation of insights through responses to a long-form questionnaire distributed to experts.
- Review of collective clinical experience and practical approaches to chorea assessment and management.
Main Results:
- Experts described practical approaches to assessing chorea, engaging care partners, and setting goal-oriented treatment plans.
- The most common medication classes for chorea suppression are vesicular monoamine transporter 2 (VMAT2) inhibitors and antipsychotics.
- Individualized titration, regular feedback, and functional assessments are key to optimizing therapy.
Conclusions:
- Integrating pharmacologic therapy with multidisciplinary care and goal-oriented communication can improve outcomes for individuals with HD.
- Clinicians can enhance safety, independence, and quality of life for patients and families affected by HD chorea.
- Practical strategies for evaluation and treatment are essential for addressing underutilization of pharmacologic interventions.
Abstract:
Huntington's disease (HD) is a rare, autosomal dominant neurodegenerative disorder caused by a pathogenic CAG expansion in the huntingtin gene, classically characterized by a triad of cognitive, psychiatric, and motor symptoms. Involuntary movements known as chorea are the most notable feature of HD. Despite the negative impact chorea can have on many aspects of day-to-day life, pharmacologic treatment remains underutilized in clinical practice. Barriers include anosognosia, a complex treatment landscape, limited evidence-based guidelines, and variable access to care. In February 2025, 11 North American HD specialists and members of the Huntington Study Group (HSG) Motor Treatment Task Force, who collectively care for ∼1500 people with HD (PwHD) convened to discuss current practices and challenges in the pharmacologic treatment of chorea. Insights were supplemented by responses to a long-form questionnaire distributed via email. Attendees and respondents described practical approaches to assessing chorea, engaging care partners, and setting goal-oriented treatment plans. This review draws on collective experience to highlight practical, real-world strategies for the evaluation and treatment of HD chorea. The two most common medication classes used to suppress chorea are vesicular monoamine transporter 2 (VMAT2) inhibitors and antipsychotics. Individualized titration, regular patient and care partner feedback, and functional, rather than purely motor-based, assessments are key to optimizing therapy. By integrating pharmacologic therapy with multidisciplinary care and holistic, goal-oriented communication about the impact of chorea, clinicians can meaningfully improve safety, independence, and quality of life for individuals and families affected by HD.
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