Practical recommendations for the treatment of chorea associated with Huntington's disease: An expert consensus

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.

Keywords

Huntington's disease chorea chorea treatment VMAT2 inhibitor antipsychotic

Introduction

Huntington's disease (HD) is a rare, dominantly inherited neurodegenerative disorder involving a pathogenic expansion of CAG repeats within exon 1 of the huntingtin gene.¹ A CAG expansion of 40 or more is associated with fully penetrant disease. HD is estimated to affect ∼41,000 people in the USA, with more than 200,000 at risk.^2–4 It typically presents during middle adulthood and progressively worsens over the course of 15–20 years until death.⁵ Around 10% of persons with HD (PwHD) develop symptoms before the age of 21, known as Juvenile onset Huntington's Disease (JoHD).⁶ HD is characterized by a triad of cognitive, behavioral, and motor symptoms. Subtle changes in executive function and mood may precede the development of chorea,^7,8 the involuntary movements that classically define HD and which affect up to 90% of individuals with adult-onset HD.

Although chorea is a defining characteristic of HD that can negatively impact quality of life,^9,10 based on health insurance records, only 20–30% of PwHD are prescribed medication for chorea within 7 years of motor diagnosis.¹¹ While no approved treatments currently exist that slow or halt the progression of HD, pharmacologic treatment of chorea can significantly improve functionality, lower fall risk, decrease weight loss, and improve quality of life.^12–18 There are a variety of medications available to treat chorea associated with HD, three of which are approved by the FDA.¹⁹

This manuscript aims to provide clinicians treating PwHD with practical, expert-informed guidance on when to initiate treatment and how best to select and use available medications. Rather than presenting evidence-based treatment guidelines,^20,21 the approaches described here are informed by the collective experience of North American HD specialists who routinely manage chorea in PwHD. The authors are all members of the Huntington Study Group (HSG) Motor Treatment Task Force who convened to share and synthesize real-world practice. We discuss our approaches to chorea assessment and intervention, including practical considerations for the use of VMAT inhibitors and antipsychotics, strategies for engagement with PwHD and their care partners, and identifying barriers to treatment.

Methods

In January 2025, 11 HD specialists who practice in North America, all members of the Huntington Study Group Motor Symptom Taskforce and collectively caring for more than 1500 PwHD, were invited to convene. The objective was to discuss strategies for the pharmacological treatment of chorea associated with HD, and to publish practical guidance for non-experts. In January of 2025, an informal, long-form questionnaire (Appendix 1) was distributed to all invited professionals. In February of 2025, 7 Movement Disorders neurologists and 1 Cognitive/Behavioral Neurologist convened in person, while the remaining 3 Movement Disorders neurologists provided written input via email. The questionnaire was employed as a discussion guide. Detailed meeting notes and email responses were compiled in order to draft the publication, which integrates a literature review with content derived from group discussion and was edited based on input from all attendees and email contributors.

Chorea

Historically, HD has been labeled as a hyperkinetic movement disorder, characterized most notably by abnormal movements known as chorea, described as irregular, unpredictable, “dance-like” movements that are involuntary and unsustained.²² Choreiform movements may involve the face, orobuccal lingual region, trunk, and extremities, and can vary in severity throughout the disease course.

Chorea can interfere with social interactions, mobility, and activities of daily living (ADLs). Troublesome chorea may lead to unwanted social scrutiny, impaired balance and coordination, difficulties with communication or swallowing, increased risk of falls, safety hazards when driving, cooking, and eating, and unintentional weight loss. Note that PwHD may present for clinical care only when chorea becomes prominent enough to interfere with their ADLs.^23–25

In later-stage disease, the chorea may decrease in severity, with bradykinesia and rigidity (parkinsonism) becoming more prominent. Importantly, antidopaminergic medications used to suppress chorea may exacerbate parkinsonian features. Together, these factors underscore the need for ongoing reassessment of treatment goals and dose adjustments over time.²⁶

Assessing chorea

Chorea has been assessed using a variety of scales, most commonly the Unified Huntington's Disease Rating Scale^® (UHDRS), developed in the 1990s to standardize cognitive, psychiatric, motor, and functional assessments of HD, and commonly used as a clinical trial outcome measure.^7,27–31 It has been widely studied for reliability.^12,14,16 It can also be performed remotely using a virtual version of the assessment (vUHDRS^®).³² In routine clinical care, a full UHDRS assessment is not always necessary; the motor examination, and particularly the Total Maximal Chorea (TMC) Score, provides a practical measure of severity. This score is often used to guide treatment decisions and may be required for prior authorization of pharmacologic therapies (see below). While the use of the UHDRS may be helpful to objectively assess HD, expert practice often places greater or equal weight on individual and care partner reports of day-to-day challenges and their impact on quality of life.

When do HD specialists treat chorea?

Pharmacologic therapy for mild to moderate chorea is not always necessary. HD specialists treat chorea when it meaningfully interferes with physical and social comfort, safety, or patient-defined goals, determined by a combination of reports from PwHD, their care partners, and clinical observation.

Although PwHD often report that cognitive and psychiatric features of HD are more burdensome than their involuntary movements, chorea can negatively impact many aspects of day-to-day living, employment, and independence.¹⁰ Higher chorea scores are also associated with poorer health related quality of life (HRQoL) measures including anxiety and stigma,⁹ as well as increased risk of falls^33,34 and earlier admission to nursing homes.^35,36 Notably, in recent VMAT-2 trials, both deutetrabenazine and valbenazine demonstrated a positive patient global impression of change,^12,16 reflecting participants’ perceptions of overall improvements in HD symptoms.

When chorea impacts ADLs or individual goals

Most specialists determine the need for pharmacologic treatment by presenting prompts about ADLs and focusing on individual goals. Rather than asking general questions about how their movements have changed, HD clinicians ask pointed questions about relevant activities. For example:

Do you find you drop or spill things more than previously?

Do people find it more difficult to understand you than before?

Can you perform a specific activity (for example, unloading a dishwasher) without difficulty, or have you modified the way you do it (for example, replacing plastic dishes with glass)?

Have you had any recent near falls or falls?

Many HD specialists find that using a clinic-based questionnaire during appointments helps to more efficiently assess symptom severity and standardize the decision-making process when initiating or modifying pharmacologic therapy. We note that discussions oriented to the person's goals – such as being able to safely cook, shave, or approach a round of job interviews – are the most effective approach to introducing the idea of medication for chorea control. Some clinicians ask PwHD to observe themselves in a mirror or to record a smartphone video to increase awareness of how their movements appear to others and how they may affect daily activities.

When the care partner's perspective differs

The role of a care partner cannot be understated in the determination of initiating or modifying treatment of HD chorea. Care partner perceptions of day-to-day function, progression, and behavior can differ significantly from those reported by PwHD themselves, particularly in the presence of anosognosia.^37–39 They may be more likely to share anecdotal observations like bruises, falls, new challenges, or changes in daily routine. Conversely, the care partner may deny the impact of chorea, yet state that they have adjusted how they handle household tasks (ex. converted all glassware to plasticware) and responsibilities, or report that they have replaced or relocated objects and furniture.

Chorea may be exacerbated by stress and can fluctuate throughout the day. Care partners may provide input on exacerbating factors, particularly when the individual has anosognosia. This information can help clinicians decide whether treatment is indicated. In some cases, addressing contributing factors like stress may be an appropriate first step.

For these reasons, HD clinical teams employ structured strategies to obtain input from care partners, including pre-visit calls, consent for separate meetings, or assessments designed to be completed by the companion. Another strategy is to deliberately seat the care partner behind the PwHD in the exam room, such that the clinician but not the PwHD can observe the care partner's reactions.

When clinical assessments indicate chorea-related impairment

HD-specific clinical assessments, such as the UHDRS total motor score (TMS), can be useful for measuring change at regular intervals and appraising treatment response. The TMS comprises multiple motor domains, including the Total Maximal Chorea score (TMC), a subscore of the UHDRS motor exam that measures chorea severity across seven body regions (face, orobuccal-lingual region, trunk, and each limb independently). These scores can be helpful to track motor symptoms and response to treatment, but experts do not rely on them alone to determine treatment necessity. Observation throughout the entire encounter can reveal the severity and location of the chorea and influence treatment decisions. That said, performing a TMC assessment prior to initiating therapy, if possible, is ideal and may actually be required in the US for medication prior authorization.

To assess the impact of chorea on mobility, eating, and communication, HD clinicians may review the postural and gait items on the motor section of the UHDRS or employ screening tools, such as the FALL-HD questionnaire⁴⁰ or the Eating Assessment Tool-10.⁴¹ A skin exam for superficial abrasions and bruises may also reveal chorea-induced injuries.⁴² Weight monitoring is critical; when compounded by chorea it may signal the need to control movements that contribute to unintentional weight loss, a poor prognostic factor.^43–45

Existing approaches to treat chorea

The most commonly used medications to treat chorea fall into two categories: VMAT2 inhibitors and antipsychotics. Additional medications prescribed as adjunct therapy may include benzodiazepines and amantadine.¹⁹

VMAT-2 inhibitors

There are three FDA-approved VMAT-2 inhibitors for HD-associated chorea: tetrabenazine, deutetrabenazine and valbenazine.⁴⁶ These agents block VMAT2 receptors responsible for the vesicular uptake of dopamine and other monoamine neurotransmitters.⁴⁷ Because alterations to synaptic dopamine levels are thought to play a role in HD, modulating the availability and release of dopamine can result in chorea control. The three drugs have similar mechanisms of action (MOA) but differ in their structure, metabolism, and dosing. These differences, recommended dosing, and real-world prescription practices are outlined in Table 1. Given the pharmacokinetics and side effect profile of tetrabenazine, the preferred VMAT-2 inhibitors are deutetrabenazine and valbenazine. These newer agents have comparable efficacy in regard to the pivotal studies that resulted in FDA approval, and both can be dosed once daily. All three VMAT-2 inhibitors have a boxed warning related to the potential for exacerbating depression and/or suicidality in HD, requiring careful monitoring for new or worsening mood after initiating treatment. Depression and suicidality are far more common in HD than the general population, making it imperative to regularly inquire about these and other psychiatric symptoms.^48,49 VMAT2 inhibitors can be initiated in PwHD with depression if adequately treated first, but should be avoided in patients with severe, refractory depression or multiple serious suicide attempts.

Table 1.

Comparison of medications indicated (in the USA) for the treatment of chorea associated with Huntington's disease.

Feature	Tetrabenazine	Deutetrabenazine		Valbenazine
Feature	Tetrabenazine	IR	XR	Capsule	Sprinkle
FDA-approved	2007	2017	2023	2023	2024
Half-life	5–7 h (short)	9–10 h (medium)		20 h (long)
Recommended Dosing	12.5–100 mg/day, divided TID	6–48 mg/day, divided BID	6–48 mg/day, once daily	40–80 mg/day, once daily
Formulation	Tablet, can be cut/crushed	Tablet, cannot be cut/crushed		Capsule, cannot be cut/crushed	Capsule, opened to release granules
Efficacy (drug minus placebo)	-3.5 points TMC	-2.5 points TMC		-3.2 points TMC
AEs (drug minus placebo)	Sedation 28%, Depression 20%, Parkinsonism 19%	Diarrhea 9%, Sedation 7%, Insomnia 4%		Sedation 13%, Urticartia 9%, Rash 8%

AEs: adverse effects; BID: twice daily; FDA: Federal Drug Administration; IR: Immediate Release; XR: Extended Release; TID: three times daily; TMC: total maximal chorea

For PwHD who have dysphagia, valbenazine is available in a sprinkle formulation for addition to soft food. Studies have also evaluated crushing the contents of valbenazine capsules for addition to soft food or administration via gastrostomy tube.⁵⁰ One case report discusses crushing of deutetrabenazine tablets for treatment of tardive dyskinesia,⁵¹ but no formal studies have been conducted in HD.

Antipsychotics and other medications

Antipsychotics, also referred to as neuroleptics or dopamine blocking drugs, are also commonly and effectively prescribed for chorea, especially in the presence of behavioral, psychiatric, or non-motor symptoms of HD.^{19–21,52,53} A discussion of the MOA and biological rationale for each is beyond the scope of this paper, but we note common and less commonly prescribed medications for adult and juvenile onset HD and elaborate on their applications and real-world dosing practices in in Table 2.

Table 2.

Medications commonly prescribed for the treatment of individuals presenting with HD chorea and psychiatric symptoms.

Class	Medication	Common dose range	Concurrent psychiatric symptoms	Common adverse effects
Atypical antipsychotics	risperidone	0.5 mg PO QHS – 6 mg daily in divided doses	Irritability, behavioral symptoms	Sedation, parkinsonism, weight gain, metabolic syndrome, akathisia, hyperprolactinemia
	olanzapine	2.5–20 mg QD	Irritability, sleep disruptions, weight loss	Sedation, metabolic syndrome, weight gain, parkinsonism, akathisia
	aripiprazole	2.5–20 mg QD	Depression/anxiety	Metabolic syndrome, weight gain, akathisia, parkinsonism
Typical antipsychotics	haloperidol	0.5 mg – 10 mg daily in divided doses	Behavioral symptoms/psychosis	Sedation, parkinsonism, akathisia, weight gain, metabolic syndrome
Typical antipsychotics	fluphenazine	1–5 mg daily	Behavioral symptoms/psychosis	Sedation, parkinsonism, akathisia, weight gain, metabolic syndrome
NMDA antagonist	amantadine	100–200 mg BID	Apathy	Livedo reticularis, lower extremity edema, constipation
Anxiolytic	clonazepam	0.5 mg BID mg – 1 mg TID	Extreme anxiety	Sedation, falls, dependence
Anxiolytic	diazepam	2–10 mg TID	Extreme anxiety	Sedation, falls, dependence

BID: twice daily; TID: three times daily; QD: Once daily

Antipsychotic medications most commonly prescribed by HD experts for chorea control are risperidone,^54,55 olanzapine,^56,57 and aripiprazole.⁵⁸ Less frequently used by those surveyed was haloperidol,^59,60 as well as the newer second-generation antipsychotics brexpiprazole and cariprazine,^61,62 for which anecdotal reports exist of chorea control, but further studies are needed. Although tiapride is frequently cited in the literature,^19,63 it does not have regulatory approval in the United States or Canada, and was therefore not mentioned during the discussion among North American HD clinicians.

The NMDA receptor antagonist amantadine^64–67 may be prescribed for control of mild chorea in the presence of significant apathy, and benzodiazepines as adjunct therapy may provide chorea control in the case of extreme anxiety or when nighttime movement suppression is needed.^68,69 The risk/benefit ratio should be considered for all medications.

Chorea is less common in individuals with JoHD, although it may be present, particularly with adolescent onset. Most individuals with JoHD experience a unique set of hypo and hyperkinetic motor symptoms including parkinsonism and dystonia, presenting a different set of challenges. Treatment of JoHD requires a sensitive, holistic and personalized approach and is beyond the scope of this review.

Other medications and non-pharmaceutical therapies

While this paper is focused on pharmacological treatments, specialists agree that multidisciplinary care including physical therapy (PT), occupational therapy (OT), and speech therapy (SLP) are imperative in conjunction with pharmacological therapy to minimize the risk of HD-related complications and optimize quality of life.⁷⁰

It is also important to mention that many PwHD report self-medicating with cannabis for anxiety, sleep, or even chorea control, but formal studies conducted in PwHD have lacked power. A broader discussion of psychoactive compounds, preclinical evidence, and clinical studies of cannabis or cannabinoid drugs for HD is beyond the scope of this review.^21,71–73 However, the clinicians surveyed noted that chronic use may exacerbate HD apathy or lead to emergent psychosis.

Common prescription practices among HD experts

The following reflects common real-world prescribing practices among HD specialists, rather than formal treatment algorithms. HD symptoms vary tremendously, and when psychiatric/behavioral symptoms predominate, they should be addressed concurrently. Mild chorea may not always warrant pharmacologic treatment but is dependent upon the needs of each individual.

When chorea is the primary symptom

In those presenting with chorea that needs treatment, most HD clinicians will select one of the three VMAT-2 inhibitors (with preference for the newer VMAT-2 medications with improved pharmacokinetics). Common practice is to start at the lowest available dose and titrate to a dose that sufficiently suppresses chorea without unwanted side effects. Comparisons of tetrabenazine, deutetrabenazine, and valbenazine including common dosing practices and AEs are outlined in Table 1. Dosing can be adjusted based on the severity of the chorea; with moderate to severe cases some specialists may titrate higher than the approved dose, though this practice has not been formally assessed and is currently limited to case reports and anecdotes.^74,75 Appendix 2 presents drug label information on interactions between deutetrabenazine and medications commonly co-prescribed for PwHD, and Appendix 3 provides parallel information for valbenazine.

When psychiatric/behavioral symptoms predominate

Many specialists initiate treatment with an antipsychotic when chorea and behavioral or psychiatric symptoms occur concurrently, with attention to the symptom constellation and cost considerations. Here we outline common HD symptoms and medications prescribed for concomitant chorea control.

Irritability and outbursts: D2 receptor antagonists such as risperidone and olanzapine.^19,52,55,56

Psychosis, including delusions and auditory or visual hallucinations: risperidone or olanzapine, and rarely, haloperidol or quetiapine.^59,60 HD specialists do not prefer haloperidol due to the high incidence of side effects. While quetiapine may be used to treat behavioral or psychiatric symptoms, due to its minimal D2 antagonism, it generally does not provide sufficient chorea suppression.

Sleep challenges, including insomnia, circadian rhythm disruptions, or chorea interfering with sleep initiation or quality: olanzapine,^56,57 and less frequently quetiapine. Benzodiazepines may also be used for adjunctive nighttime chorea control.^69,76,77

Depression/anxiety: Aripiprazole is a D2 and serotonin 5-HT1A partial agonist and 5-HT2A antagonist.⁵⁸ The unique D2 partial agonism of aripiprazole has modulatory effects on dopamine transmission and may be less effective for chorea but helpful with mood, and is frequently used as an adjunctive treatment for major depression. Benzodiazepines (e.g., diazepam or clonazepam) help with extreme anxiety, though they can cause increased risk of sedation, brain fog, falls and balance impairment.

Unintentional or extreme weight loss due to an increased metabolic rate and/or chorea: Olanzapine. Some clinicians note that deutetrabenazine can also promote weight gain, as observed in the FIRST-HD trial.⁷⁸

Apathy: amantadine (a dopamine modulator and NMDA antagonist). Note that evidence of its efficacy for chorea control is mixed in open-label and randomized controlled studies, and only anecdotal for apathy.^64–66

Combination treatments

Some clinicians may use dual therapy if monotherapy is insufficient for chorea control, or if other psychiatric symptoms are present. Dual therapy with VMAT2 inhibitors and antipsychotics is not routinely recommended by HD experts due to increased burden of side effects, such as cognitive blunting, apathy, parkinsonism, akathisia, or QT prolongation, but is often necessary if chorea is refractory or if behavioral issues are severe.^76,79,80 If chorea worsens situationally, for example due to heightened anxiety, adding an SSRI or another anxiolytic therapy may be sufficient.

Adjusting medications

PwHD and families are the best source of information to assess response to a drug aimed at chorea control. Establishing realistic goals of therapy prior to initiation of medication is key; for example, a chef experiencing mild chorea may connect with the goal of optimizing his ability to slice vegetables. If there is significant resistance to therapy due to anosognosia, it can be helpful to focus on daily consequences and outcomes, such as fall frequency, damage to their cell phone due to dropping it frequently, or broken household objects.

Safety considerations

As mentioned above, all three FDA-approved VMAT-2 inhibitors carry a boxed warning for risk of exacerbating depression and suicidality, which is already higher in HD patients than the general population.^48,49 Therefore, it is particularly important to discuss this with the PwHD and their care partner and regularly monitor for worsening mood and/or onset of new psychiatric symptoms. In addition, VMAT2 inhibitors may cause adverse events such as somnolence, akathisia, and diarrhea. Amantadine can cause livedo reticularis, lower extremity edema and constipation, among other effects. The commonly used antipsychotics (risperidone, olanzapine, and aripiprazole) are associated with a risk of QT prolongation that may warrant baseline and subsequent electrocardiogram (ECG) monitoring, especially with high doses or combination therapy. HD specialists concurred that our use of routine ECG monitoring is minimal, emphasizing that decisions are guided by clinical judgment, with many prioritizing symptom control while weighing individual cardiac risk. Risperidone, olanzapine, and to a lesser extent aripiprazole, are associated with risks of weight gain and metabolic syndrome, warranting the consideration of hemoglobin A1C and lipid profile monitoring, either by the prescriber or in collaboration with primary care. Risperidone, and to a lesser extent olanzapine, is associated with a risk of hyperprolactinemia, which may cause gynecomastia, galactorrhea, and sexual dysfunction. While parkinsonism (primarily akinesia and rigidity) often develops intrinsically with disease progression and eventually supervenes, VMAT2 inhibitors and antipsychotics themselves may cause or exacerbate existing parkinsonism. Therefore, regular evaluation of antichorea medication dosages and necessity are important, especially in more advanced patients where polypharmacy may be more common.

Role of clinic staff

Many experts recommend a return visit shortly after medication initiation or dose change if possible. In many cases, clinic staff and hospital pharmacists play a role in checking in on the patient and family's experience via direct outreach. A nurse or clinic coordinator may contact the patient or family before or in between appointments to discuss titration or any medication issues, which they can then communicate to the treating physician. If this relationship is feasible, it can be extremely informative for understanding tolerability and effectiveness and making adjustments.

Barriers to the treatment of chorea

Specialists participating in the discussion identified several barriers to the treatment of chorea in HD, including limited or retired treatment guidelines,^20,21 variable access to specialty care,⁸¹ and diagnostic, administrative, and financial complexity. VMAT2 inhibitors in particular can be cost prohibitive, and they are not globally available. Symptomatic and experiential factors such as anosognosia, apathy, cognitive decline, and prior family experiences can further complicate recognition of functionally significant chorea and willingness to initiate treatment.^37–39 To overcome these challenges, HD providers develop clinic-specific strategies to gather accurate information, approach families with sensitive counseling and education, and emphasize functional goals over clinical scores.

Conclusions

Huntington's disease is a devastating, progressive neurodegenerative disease that has been historically defined by its canonical motor symptom, chorea. Although non-motor symptoms often have the greatest impact on QoL, chorea can significantly affect function, independence, and daily activities. In the absence of a disease-modifying therapy (DMT), expert HD clinicians emphasize available symptomatic treatment to improve quality of life.¹⁰

This consensus highlights key considerations for the use of VMAT-2 inhibitors and antipsychotics for the treatment of chorea, with an emphasis on patient- and care partner-centered goal setting and feedback. Given that many PwHD are treated by clinicians without regular exposure to HD, improving communication and clarifying treatment strategies may help reduce barriers to care and increase confidence in chorea management.

It is important to acknowledge that we offer a North American perspective and do not address all clinical scenarios, including juvenile-onset HD, advanced-stage disease, polypharmacy, medication switching or discontinuation, and management of side effects when treatment remains clinically necessary. Furthermore, many common treatments discussed here are unavailable to those outside of the US, due to differing regulatory approvals, economic burden, or limited access to healthcare. Many of us operate with multidisciplinary teams that include specialists familiar with the complexities of HD, and we are aware that these supports are unavailable within many practices. Importantly, there are several nonprofit, HD specific organizations that provide educational online resources directed at clinicians. These include the Huntington's Disease Society of America, the HSG, Help4HD, HDYO, and the European Huntington's Disease Network, among others.

The field of HD research is advancing rapidly, with new medications for chorea in development, including bevantolol, a non-selective β-adrenergic antagonist, which has shown promising Phase 2 results (NCT05475483).⁸² A variety of DMTs are also under clinical investigation and future approvals could profoundly influence care-seeking behaviors and care practices.

The members of the HSG HD Motor Treatment Task Force agree that chorea remains an important modifiable symptom of HD. We have observed firsthand that appropriate symptomatic interventions can be life-changing and that sensitively guiding individuals through their HD journeys is realizable and rewarding for providers and most importantly, PwHD.

Footnotes

Acknowledgments

The authors would like to acknowledge the following individuals for their input: Dr Arik Johnson, Chief Mission Officer, and Dr Tamara Maiuri, Associate Director, Research and Mission Programs, Huntington's Disease Society of America. Medical writing and editorial support were provided by Dr Leora Fox (Fox Scientific Consulting), in accordance with Good Publication Practice (GPP) guidelines. A grant from Neurocrine Biosciences made this assistance possible; authors have authorized the submission of their manuscript via third party and approved any statements or declarations. Finally, the authors extend their heartfelt thanks to the many Huntington's disease families who have entrusted us with their care.

ORCID iDs

Jee Bang

Amy Brown

Samuel Frank

Victor Sung

Ethical considerations

There are no human participants in this article and informed consent is not required.

Consent to participate

Not applicable

Consent for publication

Not applicable

Author contributions

EFS and DC proposed ideas and outlined the manuscript. JB, AB, BB, SF, DH, NM, TM, SA, and VA participated in the questionnaire, live discussion, and email follow-up. VS contributed Table 1, DC , and SA updated Tables and provided detailed edits. EFS and DC generated the discussion questions in Appendix 1, and SA Appendix 2 and 3. All authors reviewed the text.

Funding

Support for the preparation of this manuscript was provided by Neurocrine Biosciences, Inc.

Declaration of conflicting interests

AB receives compensation from HSG as Chair-Elect of the Site Development Advisory Council. She has received consult fees from Novartis for a research advisory role, and contract funding for conducting HD-related research from CHDI Foundation, Novartis, Neurocrine Biosciences, Prilenia Therapeutics, PTC Therapeutics, Novartis, Teva pharmaceuticals, and UniQure. BB has received compensation for speaking engagements from Teva, Neurocrine, and Acadia. DH receives institutional grant/research funding from Abbvie, Amylyx, Neurocrine, UCB, the Huntington's Disease Society of America, and the Parkinson Foundation. EFS receives grant and research funding from the Huntington Study Group, Roche/Genentech, uniQure, CHDI Foundation, Prilenia Therapeutics, and the NIH/University of Iowa. She receives personal compensation for consulting from Medscape, PTC Therapeutics, Alnylam, Novartis, Latus Bio, and Mitre Consulting, and honoraria from the International Parkinson and Movement Disorder Society. She has received travel support from the Huntington Study Group/CHDI Foundation and Help4HD International. She serves on a data safety monitoring board for Skyhawk Therapeutics and an advisory board for Wave Life Sciences. She serves as Chair of the Huntington Study Group Executive Committee and as an unpaid member of the Huntington's Disease Society of America Board of Trustees. She receives royalties from Oxford University Press. DC Daniel O. Claassen, MD, MS has served as a consultant and/or advisory board member for Novartis, Sarepta Therapeutics, Alterity Therapeutics, uniQure, AbbVie, Advera, SOM Biotech, and Upstream Vision. Dr. Claassen serves on a Data Safety Monitoring Board (DSMB) for the University of Michigan and Amylyx. He declares no other financial or non-financial competing interests relevant to this work. JB has received personal compensation for consulting/advising for Roche/Genetech, Wave Life Sciences, and UniQure. She has or will receive compensation for her professional role from the Huntington Study Group (Chair of the Education Advisory Committee). NRM has received support from the NIH, Michael J. Fox Foundation, and Parkinson Foundation. He has served as a consultant for AbbVie, Inc. and on advisory boards for AbbVie, Novartis, ONO Pharmaceuticals, Farrer, and received travel/speaker fees for various Parkinson and movement disorders educational/CME events. SF receives grant funding from Roche/Genentech, CHDI Foundation, Huntington's Disease Society of America, Huntington Study Group (Prilenia), Cerevel Therapeutics, Bial Therapeutics, UCB Pharma, Koneska. He receives personal compensation for consulting to Novartis, Amylyx, Reata, AskBio, Teva Pharmaceuticals, Life Edit Therapeutics, WKD SMRT, Roche, Alnylam, Sarepta. He has received compensation for professional role from these organizations (role): Huntington Study Group (Neurocrine, Chair of DSMB, virtual UHDRS and co-Chair of HSG Executive Membership Committee), uniQure (Steering Committee), HCD Economics (Chair of Steering Committee). TAM has received consulting fees and speaker honoraria from AbbVie, the International Parkinson and Movement Disorder Society, CHDI Foundation/Management, and the Canadian Movement Disorders Society. He has served on advisory boards for AbbVie, Knight Therapeutics, UCB/IQVIA, Roche, ALN Therapeutics, and Wave Life Sciences. He has received research funding from the University of Ottawa Brain and Mind Research Institute (uOBMRI), the Canadian Institutes of Health Research (CIHR), the Michael J. Fox Foundation, Parkinson Canada, the Parkinson Research Consortium, Brain Canada, BAMO, the Tallman Foundation, and INOVAIT. He receives salary support from the University of Ottawa Medical Associates. VS serves on a Steering Committee for UniQure and has served as a consultant for Teva and Neurocrine. EFS is an Editorial Board Member of this journal, and DC, SF, and TAM are Associate Editors of this journal, but they were not involved in the peer-review process of this article nor had access to any information regarding its peer-review.

Data availability

No new data were created or analyzed in this study. Data sharing is not applicable to this article.

Appendix 1: Questions Posed to HD Experts for Round Table Discussion

Appendix 2: Quick reference for drug-drug interactions between valbenazine and other commonly prescribed medications in individuals with HD

Appendix 3: Quick reference for drug-drug interactions between deutetrabenazine and other commonly prescribed medications in individuals with HD

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