Chorea in Adults Treatment & Management

Updated: Jul 01, 2019
  • Author: Pradeep C Bollu, MD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Medical Care

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 (eg, reserpine, tetrabenazine, deutetrabenazine), represent another option in the treatment of chorea. [13, 14, 97]

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]

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Surgical Care

Deep brain stimulation

Deep brain stimulation r(DBS) 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 DBS is not yet used routinely for chorea, as it is for PD, exciting progress has been made with this modality. Prior to DBS, surgeries like thalamotomy and pallidotomy were found to be effective in some cases. [106, 107]  However, due to the invasive nature of the surgeries and the complications due to lesional surgery, they are not used in routine clinical practice.

Cell transplantation

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]

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