Chorea in Adults Medication

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

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


Antipsychotic agents

Class Summary

Block dopamine receptors and appear to have antispasmodic effects.

Haloperidol (Haldol)

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


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

Clozapine (Clozaril, FazaClo, Versacloz)

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.

Olanzapine (Zyprexa, Zyprexa Relprevv, Zyprexa Zydis )

A thienobenzodiazepine antipsychotic known to cause serotonergic, dopaminergic and adrenergic blockade

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.

Quetiapine (Seroquel)

May act by antagonizing dopamine and serotonin effects.


Monoamine depleting agents

Class Summary

Antichorea effect of central monamine-depleting agents is believed to be related to its effect on reversible depletion of monoamines (eg, dopamine, serotonin, norepinephrine) from nerve terminals.


First isolated from the root of Rauwolfia Serpentina (Indian Snakeroot), reserpine was FDA approved in 1955. Reserpine irreversibly blocks the Vesicular Monoamine Transporters- VMAT 1 and 2 reducing the stores of the monoamines (norepinephrine, dopamine, serotonin). It was primarily used as an antihypertensive agent and generally no longer used in the treatment of chorea due to its side effects. 

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.

Deutetrabenazine (Austedo)

Orally administered VMAT-2 inhibitor. It is indicated for chorea associated Huntington disease.



Class Summary

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

Clonazepam (Klonopin)

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.



Class Summary

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, Depakene)

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 the mechanism of action is not clearly 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 a 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 there is a concern that seizures are likely to occur with this reduction. Monitor patients closely for increased seizure frequency during this period.

As adjunctive therapy, divalproex sodium may be added to the patient's regimen at 10–15 mg/kg/d. Dosage may be increased by 5–10 mg/kg/d every week 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.

Carbamazepine (Carbatrol, Tegretol, Epitol, Equetro)

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 the thalamus, resulting in a reduction of polysynaptic responses and blocking post-tetanic potentiation. Reduces sustained high-frequency repetitive neuronal firing. Potent enzyme inducer that can induce own metabolism. Due to potentially serious blood dyscrasias, undertake benefit-to-risk evaluation before the drug is 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 the liver to an active metabolite (ie, epoxide derivative) with a half-life of 5-8 h. Metabolites are excreted through feces and urine.