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Chorea in Adults Treatment & Management

  • Author: Stephanie M Vertrees, MD; Chief Editor: Selim R Benbadis, MD  more...
Updated: Oct 24, 2014

Medical Care

See the list below:

  • Only symptomatic treatment is available for patients with chorea. Chorea may be a disabling symptom, leading to bruises, fractures, and falls, and may impair the ability of patients to feed themselves. In addition, patients sometimes express a desire for antichorea treatment for cosmetic reasons.
  • The most widely used agents in the treatment of chorea are the neuroleptics. The basis of their mechanism of action is thought to be related to blocking of dopamine receptors. Neuroleptics can be classified as typical and atypical. Typical neuroleptics include haloperidol and fluphenazine. Atypical neuroleptics include risperidone, olanzapine, clozapine, and quetiapine.
  • Dopamine-depleting agents, such as reserpine and tetrabenazine, represent another option in the treatment of chorea.[13, 14]
  • GABAergic drugs, such as clonazepam, gabapentin, and valproate[89] , can be used as adjunctive therapy.
  • Coenzyme Q10 alone and in combination with minocycline have been proposed as potential therapies and have shown promise in HD rodent models. Coenzyme Q10 is thought to target mitochondrial dysfunction, which has been implicated as one of the pathologic mechanisms of mutant huntingtin. Minocycline, one of the tetracyclines, is known to have anti-apoptosis effects.[10, 11]
  • Intravenous immunoglobulin and plasmapheresis may shorten the course of the illness and decrease symptom severity in patients with Sydenham chorea.
  • Chorea following cardiac transplantation has been reported to be responsive to steroid treatment.[64]
  • Reports of drug treatment for hemiballism must take into account the high spontaneous remission rate for the disorder. Anecdotal reports must be viewed with caution, unless they can demonstrate that the response is due to the agent (by recurrence of the movements with drug withdrawal). The rarity of this disorder and the severity of its manifestations have precluded placebo-controlled drug trials. Pharmacologic treatment is the same as that prescribed for other choreatic disorders.[22, 24, 90, 8]

Surgical Care

See the list below:

  • Deep brain stimulation is an emerging technique that may benefit patients, at least in certain cases.
    • In 2000, Thompson et al reported a reduction in choreiform movements in 2 pediatric cases of chorea. One patient had cerebral palsy from birth secondary to brain hemorrhage. The other, an 11-year-old child, developed chorea subsequent to a thalamic hemorrhage 4 years before. Both children improved after the procedure.[91]
    • Reported in 2003, Krauss et al tested globus pallidus stimulation on 2 patients with dystonia (one adult and one child) and 4 adult patients with essentially static (ie, nonchanging) chorea secondary to cerebral palsy. The dystonia patients markedly improved. Two of the 4 chorea patients showed no improvement, but 2 showed mild improvement.[92]
    • In 2004, Moro et al reported on bilateral globus pallidus internus stimulation on a patient with Huntington disease (HD). Stimulation at 130 and 40 Hz improved the chorea, but the stimulation at 130 Hz worsened the bradykinesia. Stimulation of 40 Hz had little effect on the bradykinesia and appeared to increase blood flow (assessed by positron emission tomography scanning) in areas associated with executive functions and judgment.[93]
  • Although deep brain stimulation is not yet used routinely for chorea, as it is for PD, exciting progress has been made with this modality.
  • Cell transplantation is controversial and in early stages of research. It has shown variable results for HD patient participants.
    • In 2006, Bachoud-L é vi et al reported that fetal neural cell transplantation into host striatum resulted in stabilization or improvement in chorea, oculomotor dysfunction, gait, tapping, and cognition, but dystonia progressed at the same rate as nongrafted patients. However, these results persisted for up to 6 years only, and then patients' disease continued to progress at pretransplantation rates.[94]
    • In 2008, Keene et al demonstrated on autopsy that fetal neural cell grafts in 2 patients had shown neuronal differentiation and survival, but they had poor integration with host striatum, likely explaining the lack of clinical improvement in these patients.[95]
Contributor Information and Disclosures

Stephanie M Vertrees, MD Fellow in Public Health, Weill Cornell Medical College-Hospital for Special Surgery Fellowship in Medical Ethics; Fellow in Neuromuscular Medicine, Hospital for Special Surgery

Stephanie M Vertrees, MD is a member of the following medical societies: American Academy of Neurology, American Medical Womens Association

Disclosure: Nothing to disclose.


Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Additional Contributors

Stephen T Gancher, MD Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University

Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Eric Dinnerstein, MD, Maria Alejandra Herrera, MD, and Nestor Galvez-Jimenez, MD, MSc, MHA, to the development and writing of this article.

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