eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Chorea in Adults: Treatment & Medication

Author: Stephanie M Vertrees, MD, Staff Physician, Section of Internal Medicine, Department of Neurology, Dartmouth-Hitchcock Medical Center
Coauthor(s): Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Contributor Information and Disclosures

Updated: May 7, 2009

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, such as reserpine and tetrabenazine, represent another option in the treatment of chorea.13,14
  • GABAergic drugs, such as clonazepam, gabapentin, and valproate85 , 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.60
  • 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.22,24,86,8

Surgical Care

  • 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.87
    • 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.88
    • 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.89
  • 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.90
    • 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.91

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Antipsychotic agents

Block dopamine receptors and appear to have antispasmodic effects.


Haloperidol (Haldol)

Useful in treatment of irregular spasmodic movements of limbs or facial muscles.

Adult

Initial doses should be low: 0.5-1 mg/d PO; doses >10 mg/d have yielded little or no increased benefit over lower doses

Pediatric

Not established

May increase serum concentrations of TCAs and hypotensive action of antihypertensive agents; phenobarbital or carbamazepine may decrease effects; anticholinergics may increase intraocular pressure; lithium may cause encephalopathylike syndrome

Documented hypersensitivity; narrow-angle glaucoma; bone marrow suppression; severe cardiac or liver disease; severe hypotension; subcortical brain damage

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Patients may experience extrapyramidal symptoms, such as rigidity, akinesia, acute dystonic reactions, tardive dyskinesia, and neuroleptic malignant syndrome; less likely than other antipsychotic agents to cause sedation and hypotension


Fluphenazine (Prolixin)

Blocks postsynaptic mesolimbic dopaminergic D1 and D2 receptors in brain. Exhibits strong alpha-adrenergic and anticholinergic effects. May depress reticular activating system.

Adult

0.5-1 mg/d PO initially

Pediatric

Not established

May potentiate effects of narcotics, including respiratory depression; lithium increases CNS effects; barbiturates may decrease effects

Documented hypersensitivity; narrow-angle glaucoma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Besides extrapyramidal symptoms as described for haloperidol, mild leukocytosis, leukopenia, and eosinophilia occasionally occur; dermatological reactions are common; watch for urinary retention, blurred vision, dry mouth, and constipation as result of anticholinergic effects


Clozapine (Clozaril)

New atypical neuroleptic medication available in 25- and 100-mg tab. Blocks norepinephrine, serotonergic, cholinergic, histamine, and dopaminergic receptors. Mechanism of action still unclear. Affinity for mesolimbic D4 dopamine receptor accounts for striking effects in control of behavioral and psychiatric symptoms with low incidence of extrapyramidal symptoms. Histamine receptor blockade accounts for increased incidence of sleep disturbances.

Adult

Chorea: 12.5 mg PO qd; increase dose weekly to 50-75 mg PO qd
Dystonia: Doses of up to 700 mg/d may be needed
PD: 25-50 mg PO qd required to control hallucinations
Schizophrenia: Higher doses required

Pediatric

Not established

Epinephrine and phenytoin may decrease effects; other dopamine-depleting agents, TCAs, neuroleptics, CNS depressants, guanabenz, and anticholinergics may increase effects

Documented hypersensitivity; history of agranulocytosis; history of pulmonary embolism, diabetes mellitus, hepatitis, narrow-angle glaucoma, bladder retention, prostate enlargement

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Monitor for agranulocytosis and orthostatic hypotension; caution in patients who take other drugs that can cause agranulocytosis, such as carbamazepine and ticlopidine; all patients should undergo weekly WBC counts with differential; if WBC falls to <3000/µL or if absolute neutrophil count falls to <1500/µL, interrupt or discontinue therapy; anticholinergic reactions can be quite severe; may cause pulmonary embolism or hepatitis; may elevate LFT results


Olanzapine (Zyprexa)

May inhibit serotonin, muscarinic, and dopamine effects.

Adult

5-10 mg PO qd initially; increase to 10 mg PO qd within 5-7 d; not to exceed 20 mg/d

Pediatric

Not established

Fluvoxamine may increase effects; antihypertensives may increase risk of hypotension and orthostatic hypotension; levodopa, pergolide, bromocriptine, charcoal, carbamazepine, omeprazole, rifampin, and cigarette smoking may decrease effects

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Agranulocytosis has not been reported to date; watch for orthostatic hypotension and constipation; less risk of extrapyramidal effects than traditional neuroleptics; serum half-life increases by approximately 50% in patients >65 y and can be expected to increase in patients with liver dysfunction; both groups may require smaller-than-average dosages


Risperidone (Risperdal)

Binds to dopamine D2-receptor with 20 times lower affinity than for 5-HT2 receptor. Improves negative symptoms of psychoses and reduces incidence of extrapyramidal adverse effects.

Adult

1 mg PO bid initially; increase slowly to 4-6 mg/d

Pediatric

Not established

Carbamazepine may decrease effects; may inhibit effects of levodopa; clozapine may increase levels

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Low risk of extrapyramidal adverse effects; may cause extrapyramidal reactions, hypotension, tachycardia, and arrhythmias


Quetiapine (Seroquel)

May act by antagonizing dopamine and serotonin effects.

Adult

25 mg PO bid initially; titrate slowly to effect in 2-3 divided doses; not to exceed 800 mg/d

Pediatric

Not established

May antagonize levodopa and dopamine agonists; phenytoin, thioridazine, and other liver enzyme inducers may reduce levels

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May induce orthostatic hypotension associated with dizziness, tachycardia, and syncope; neuroleptic malignant syndrome has been associated with this treatment

Dopamine depleting agents

Deplete CNS of dopamine, thereby reducing chorea.


Reserpine

Depletes norepinephrine and epinephrine, which, in turn, depress sympathetic nerve functions.

Adult

0.5 mg PO qd; titrate to 1 mg PO qd

Pediatric

Not recommended

TCAs may decrease antihypertensive effects; either digitalis or quinidine may increase risk of cardiac arrhythmia

Documented hypersensitivity; diagnosed mental depression

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Sedation and inability to concentrate or perform complex tasks are most common adverse effects; occasional psychotic depression may occur that can lead to suicide (usually appears insidiously over many weeks or months and may not be attributed to drug because of delayed and gradual onset of symptoms); must be discontinued at first sign of depression; do not give to patients with history of depression; other adverse effects include nasal stuffiness and exacerbation of peptic ulcer disease; orthostatic hypotension may occur but does not usually cause symptoms; parkinsonism may manifest as adverse effect


Tetrabenazine (Xenazine)

Depletes neurotransmitter stores of dopamine, serotonin, and noradrenaline within nerve cells in the brain, thereby altering transmission of electric signals from the brain that control movement by reversibly inhibiting vesicular monoamine transporter 2 (VMAT2).
Efficacy and safety established in a randomized, double-blind, placebo-controlled, multicenter study. Patients treated with tetrabenazine had significant improvement in chorea compared with those treated with placebo. Additional studies support this effect. Indicated for chorea associated with Huntington disease.

Adult

12.5 mg PO qam initially; after 1 wk, increase to 12.5 mg bid; titrate slowly at weekly intervals in 12.5-mg increments to identify dose that reduces chorea and is well tolerated; if 37.5-50 mg/d required, administer as tid regimen; not to exceed 100 mg/d
CYP2D6 poor metabolizers: Titrate as described; not to exceed single dose of 25 mg or daily dose of 50 mg
Patients requiring >50 mg/d should be genotyped for CYP2D6

Pediatric

Not established

Active metabolites (alpha and beta dihydrotetrabenazine [HTBZ]) are principally metabolized by CYP2D6; poor metabolizers of CYP2D6 or strong CYP2D6 inhibitors (eg, paroxetine) increase exposure to these metabolites; caution if coadministered with weak CYP2D6 inhibitors (eg, duloxetine, sertraline, amiodarone)

Documented hypersensitivity; patients who are actively suicidal or are untreated or inadequately treated for depression; coadministration with MAOIs or reserpine (at least 20 d should elapse after stopping reserpine before starting tetrabenazine); hepatic impairment

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Serious adverse effects include depression and suicidal ideation; common adverse effects include insomnia, depression, drowsiness, restlessness, and nausea; may worsen mood, cognition, rigidity, and functional capacity; may cause QTc prolongation

Benzodiazepines

Demonstrated to reduce GABA concentrations in the caudate, putamen, substantia nigra, and globus pallidus. By analogy, increased GABA activity might ameliorate chorea.


Clonazepam (Klonopin, Rivotril)

Developed as antiepileptic, hypnotic, and anxiolytic used as adjunct for treatment of chorea. Belongs to benzodiazepine group, increasing GABAergic transmission in CNS. Reaches peak plasma concentration at 2-4 h after oral or rectal administration.

Adult

0.5 mg PO qd; increase dose weekly according to need and response

Pediatric

Not established

Phenytoin and barbiturates may reduce effects; CNS depressants increase toxicity

Documented hypersensitivity; severe liver disease; acute narrow-angle glaucoma

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in chronic respiratory disease or impaired renal function; withdrawal symptoms can result from abrupt discontinuation; main adverse effects include sedation, tolerance, ataxia, depression, and confusion

Anticonvulsants

May help by various neuropharmacological mechanisms. Valproate is a GABAergic agent and thus it may help in the same way as benzodiazepines. Main mechanism of action of carbamazepine appears to be stabilization of inactivated state of voltage-gated sodium channels. This may reduce neuronal firing in many systems and therefore may nonspecifically reduce abnormal movements in some patients.


Valproic acid (Depacon, Depakote, Depakote ER)

Off-label therapy sometimes helpful in reducing choreiform movements and ameliorating disruptive behavior (eg, behavior induced by anger) in patients with HD. Dosages and other information mentioned is taken from dosages used for epilepsy because dosages for HD are not clearly established. Chemically unrelated to other drugs used to treat seizure disorders. Although mechanism of action not established, its activity may be related to increased brain levels of GABA or enhanced GABA action. Also may potentiate postsynaptic GABA responses, affect potassium channel, or have direct membrane-stabilizing effect.
For conversion to monotherapy, concomitant AED dosage ordinarily can be reduced by approximately 25% q2wk. This reduction may be started at initiation of therapy or delayed by 1-2 wk if concern that seizures are likely to occur with reduction. Monitor patients closely for increased seizure frequency during this period.
As adjunctive therapy, divalproex sodium may be added to patient's regimen at 10-15 mg/kg/d. Dosage may be increased by 5-10 mg/kg/d qwk to achieve optimal clinical response. Ordinarily, optimal clinical response achieved at daily doses of <60 mg/kg/d.
Depakote Sprinkle Capsules (daily doses >250 mg should be divided bid/tid) and Depakote ER (once-daily formulation) are convenient dosage forms used in adults and children >10 y.

Adult

Monotherapy: 10-15 mg/kg/d PO in 1-3 divided doses and increase by 5-10 mg/kg/wk until seizures controlled or adverse effects prevent further increases; not to exceed 60 mg/kg/d; if total daily dose >250 mg, give in divided doses

Pediatric

Administer as in adults

Coadministration with cimetidine, salicylates, felbamate, and erythromycin may increase toxicity; rifampin may significantly reduce levels; in pediatric patients, protein binding and metabolism of valproate decrease when taken concomitantly with salicylates; coadministration with carbamazepine may result in variable changes of carbamazepine concentrations with possible loss of seizure control; may increase diazepam and ethosuximide toxicity (monitor closely); may increase phenobarbital and phenytoin levels while either one may decrease valproate levels; may displace warfarin from protein binding sites (monitor coagulation); may increase zidovudine levels in HIV-seropositive patients

Documented hypersensitivity; hepatic disease/dysfunction; hyperammonemic encephalopathy and urea cycle disorders

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Thrombocytopenia and abnormal coagulation parameters have occurred; risk of thrombocytopenia increases significantly at total trough valproate plasma concentrations >110 mcg/mL in females and 135 mcg/mL in males; at periodic intervals and prior to surgery, determine platelet count and bleeding time before initiating therapy; reduce dose or discontinue therapy if hemorrhage, bruising, or a hemostasis/coagulation disorder occur; hyperammonemia may occur, resulting in hepatotoxicity; monitor patients closely for appearance of malaise, weakness, facial edema, anorexia, jaundice, and vomiting; may cause drowsiness


Carbamazepine (Carbatrol, Tegretol, Epitol)

Has been of symptomatic help in chorea, particularly in Sydenham chorea and chorea gravidarum, but also in other types. Dosage recommendations and cautions are essentially the same in this off-label use as for the more common indication of seizures.
When used as an anticonvulsant, mechanism of action may involve depressing activity in nucleus ventralis anterior of thalamus, resulting in reduction of polysynaptic responses and blocking posttetanic potentiation. Reduces sustained high-frequency repetitive neural firing. Potent enzyme inducer that can induce own metabolism. Due to potentially serious blood dyscrasias, undertake benefit-to-risk evaluation before drug instituted. Therapeutic plasma levels are 4-12 mcg/mL for analgesic and antiseizure response. Peak serum levels in 4-5 h. Half-life (serum) in 12-17 h with repeated doses. Metabolized in liver to active metabolite (ie, epoxide derivative) with half-life of 5-8 h. Metabolites excreted through feces and urine.

Adult

200 mg PO bid on day 1 (100 mg qid susp) initially; increase by 200 mg/d or less qwk until best response attained; divide total dose and administer q6-8h; ER tab may be used for bid dosing instead of dosing tid/qid; not to exceed 1200 mg/d
Maintenance: Decrease dose gradually to minimum effective level, usually 800-1200 mg/d

Pediatric

<6 years: 10-20 mg/kg/d PO bid/tid (qid with susp); increase qwk to achieve optimal clinical response tid/qid; not to exceed 100 mg/d
6-12 years: 100 mg PO bid (50 mg qid of susp) increase gradually qwk by adding 100 mg/d PO divided tid/qid (bid with ER tab) until best response obtained; not to exceed 1000 mg/d
>12 years: Administer as in adults; not to exceed 1000 mg/d in children 12-15 y or 1200 mg/d in >15 y

Serum levels may increase significantly within 30 d of danazol coadministration (avoid whenever possible); do not coadminister with MAOIs; cimetidine may increase toxicity, especially if taken in first 4 wk of therapy; may decrease primidone and phenobarbital levels (their coadministration may increase carbamazepine levels)

Documented hypersensitivity; history of bone marrow depression; administration of MAOIs within last 14 d

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Do not use to relieve minor aches or pains; caution with increased intraocular pressure; obtain CBC count and serum iron baseline prior to treatment, during first 2 mo, and yearly or every other year thereafter; can cause drowsiness, dizziness, and blurred vision; caution while driving or performing other tasks requiring alertness

More on Chorea in Adults

Overview: Chorea in Adults
Differential Diagnoses & Workup: Chorea in Adults
Treatment & Medication: Chorea in Adults
Follow-up: Chorea in Adults
References
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Further Reading

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Keywords

adult chorea, ballism, hemiballism, biballism, paraballism, ballismus, hemiballismus, biballismus, paraballismus, choreoathetosis, athetosis, benign hereditary chorea, Sydenham chorea, Sydenham's chorea, Huntington's disease, Huntington disease, HD, senile chorea, neuroacanthocytosis, Wilson disease, Wilson's disease, WD

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Contributor Information and Disclosures

Author

Stephanie M Vertrees, MD, Staff Physician, Section of Internal Medicine, Department of Neurology, Dartmouth-Hitchcock Medical Center
Stephanie M Vertrees, MD is a member of the following medical societies: American Academy of Neurology and American Medical Women's Association
Disclosure: Nothing to disclose.

Coauthor(s)

Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Stephen A Berman, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

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, and Movement Disorders Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Richard J Caselli, MD, Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale
Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
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