An Update on the Treatment of Chorea
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Purpose of review
There are many causes for chorea, including genetic, autoimmune, pharmacological, and structural lesions. Where appropriate, treatment is based on reversing the underlying cause of chorea; many cases are self-limited, resolving when the primary disorder is treated. This review focuses on the management of chorea due to untreatable causes.
There are a limited number of double-blind randomized control trials assessing the efficacy of specific chorea treatments. Most therapeutic recommendations are based on small open-label studies, case reports, and expert opinion. This is in part due to the heterogeneity of chorea and chorea-associated syndromes and the variety of neurodegenerative phenotypes with variable progression rates.
Chorea can be treated with a variety of medications ranging from antiepileptics to antipsychotics. The recent development of selective vesicular monoamine transporter blocking agents has allowed for targeted chorea management with minimal side effects. Neurosurgical interventions such as deep brain surgery (DBS) and pallidotomy are reserved for medication-refractory chorea. As a symptom of neurodegenerative disease, chorea is only one aspect of the basal ganglia syndromes, and often, a multidisciplinary approach tailored to individual patient needs provides the best management.
KeywordsChorea Deutetrabenazine Valbenazine Deep brain stimulation
Compliance with Ethical Standards
Conflict of Interest
Erin Feinstein declares no conflict of interest. Ruth Walker has received consulting fees from the manufacturers of valbenazine, Neurocrine Biosciences, Inc. She has also received honoraria from Advance Medical Opinion, the International Parkinson Disease and Movement Disorders Society, and GE Healthcare.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 1.Hermann A, Walker RH. Diagnosis and treatment of chorea syndromes. Curr Neurol Neurosci Rep. 2015;15 https://doi.org/10.1007/s11910-014-0514-0.
- 5.Waln O, Jankovic J. An Update on Tardive Dyskinesia: From Phenomenology to Treatment. Tremor and Other Hyperkinetic Movements. 2013;1–11.Google Scholar
- 13.Ong B, Devathasan G, Chong PN. Choreoacanthocytosis in a Chinese patient--a case report. Singap Med J. 1989;30:506–8.Google Scholar
- 14.Walker RH. Management of Neuroacanthocytosis Syndromes. Tremor Other Hyperkinet Mov (N Y). 2015;5:346.Google Scholar
- 17.•• Frank S, Testa CM, Stamler D, et al. Effect of Deutetrabenazine on chorea among patients with Huntington disease: a randomized clinical trial. JAMA. 2016;316:40–50. This is curently the largest double-blind, randomized, placebo-controlled study of the effects of deutetrabenazine on patients with Huntington's diseae.CrossRefGoogle Scholar
- 18.•• Fernandez HH, Factor SA, Hauser RA, Jimenez-Shahed J, Ondo WG, Jarskog LF, et al. Randomized controlled trial of deutetrabenazine for tardive dyskinesia: the ARM-TD study. Neurology. 2017;88:2003–10. This paper is the largest multicenter double-blind, randomized, placebo-controlled study of the effects of deutetrabenazine on tardive dyskinesia.CrossRefGoogle Scholar
- 19.•• Anderson KE, Stamler D, Davis MD, Factor SA, Hauser RA, Isojärvi J, et al. Deutetrabenazine for treatment of involuntary movements in patients with tardive dyskinesia (AIM-TD): a double-blind, randomised, placebo-controlled, phase 3 trial. The lancet Psychiatry. 2017;4:595–604. This paper is the only double-blind, randomized, placebo-controlled study using deutetrabenazine to specifically treat movements from tardive dyskinesia.CrossRefGoogle Scholar
- 21.•• Hauser RA, Factor SA, Marder SR, Knesevich MA, Ramirez PM, Jimenez R, et al. KINECT 3: a phase 3 randomized, double-blind, placebo-controlled trial of Valbenazine for tardive dyskinesia. Am J Psychiatry. 2017;174:476–84. This paper is the only double-blind, placebo-controlled study uding valbenazine for tardive dysinesia.CrossRefGoogle Scholar
- 28.Giménez-Roldán S, Mateo D. Huntington disease: tetrabenazine compared to haloperidol in the reduction of involuntary movements. Neurol (Barcelona, Spain). 1989;4:282–7.Google Scholar
- 33.Park K, Lee Y, Park H. Chorea in the both lower limbs associated with Nonketotic hyperglycemia. Magn Reson Imaging. 2009;2:98–100.Google Scholar
- 40.Ryu S, Yoo JH, Kim JH, Choi JS, Baek JH, Ha K, et al. Tardive dyskinesia and tardive dystonia with second-generation antipsychotics in non-elderly schizophrenic patients unexposed to first-generation antipsychotics: a cross-sectional and retrospective study. J Clin Psychopharmacol. 2015;35:13–21.CrossRefGoogle Scholar
- 45.Mason SL, Barker RA. Advancing pharmacotherapy for treating Huntington’s disease: a review of the existing literature. Expert Opin Pharmacother. 2015;6566:1–12.Google Scholar
- 88.• Vedam-Mai V, Martinez-Ramirez D, Hilliard JD, Carbunaru S, Yachnis AT, Bloom J, et al. Post-mortem findings in Huntington’s deep brain stimulation: a moving target due to atrophy. Tremor Other Hyperkinet Mov (N Y). 2016;6:372. This paper highlights some of the difficulties with using deep brain stimulation to treat neurodegenerative diseases, such as Huntington's disease.Google Scholar
- 98.• Pouclet-Courtemanche H, Rouaud T, Thobois S, Nguyen JM, Brefel-Courbon C, Chereau I, et al. Long-term efficacy and tolerability of bilateral pallidal stimulation to treat tardive dyskinesia. Neurology. 2016;86:651–9. This paper reports uding deep brain stimulation to treat tardive dyskinesia.CrossRefGoogle Scholar