Sections

Chorea in Adults

Sections Chorea in Adults
Overview
Presentation
- History
- Physical
- Causes
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Workup
Treatment
Medication
Follow-up
References

Medication

Medication Summary

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

Class Summary

Block dopamine receptors and appear to have antispasmodic effects.

Haloperidol (Haldol)

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

Fluphenazine

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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)

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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 )

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

Risperidone (Risperdal)

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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)

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May act by antagonizing dopamine and serotonin effects.

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.

Reserpine

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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)

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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)

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

Valbenazine (Ingrezza)

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VMAT2 inhibitor indicated for chorea associated with 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)

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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)

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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)

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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.

Follow-up

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Author

Pradeep C Bollu, MD Associate Professor of Neurology, Vice Chair of Quality, Department of Neurology, Prisma Health, University of South Carolina School of Medicine, Columbia

Pradeep C Bollu, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, Boone County Medical Society, Indian Medical Association, Indian Red Cross Society, International Parkinson and Movement Disorder Society, Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Tejas R Mehta, MBBS Resident Physician, Department of Neurology, University of Missouri-Columbia School of Medicine

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 Morsani College of Medicine

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, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Catalyst, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK Life Science Science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Catalyst, Jazz, LivaNova, Neurelis, SK Life Science, Stratus, UCB<br/>Received research grant from: Cerevel Therapeutics; Ovid Therapeutics; Neuropace; Jazz; SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Longboard.

Additional Contributors

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.

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.

Stephanie M Vertrees, MD Fellow in Public Health, Weill Cornell Medical College; Fellow 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 Women's Association

Disclosure: Nothing to disclose.

Acknowledgements

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.

Sections Chorea in Adults
Overview
Presentation
- History
- Physical
- Causes
- Show All
Workup
Treatment
Medication
Follow-up
References

Chorea in Adults Medication

Medication Summary

Antipsychotic agents

Class Summary

Haloperidol (Haldol)

Haloperidol (Haldol)

Fluphenazine

Fluphenazine

Clozapine (Clozaril, FazaClo, Versacloz)

Clozapine (Clozaril, FazaClo, Versacloz)

Olanzapine (Zyprexa, Zyprexa Relprevv, Zyprexa Zydis )

Olanzapine (Zyprexa, Zyprexa Relprevv, Zyprexa Zydis )

Risperidone (Risperdal)

Risperidone (Risperdal)

Quetiapine (Seroquel)

Quetiapine (Seroquel)

Monoamine depleting agents

Class Summary

Reserpine

Reserpine

Tetrabenazine (Xenazine)

Tetrabenazine (Xenazine)

Deutetrabenazine (Austedo)

Deutetrabenazine (Austedo)

Valbenazine (Ingrezza)

Valbenazine (Ingrezza)

Benzodiazepines

Class Summary

Clonazepam (Klonopin)

Clonazepam (Klonopin)

Anticonvulsants

Class Summary

Valproic acid (Depacon, Depakote, Depakote ER, Depakene)

Valproic acid (Depacon, Depakote, Depakote ER, Depakene)

Carbamazepine (Carbatrol, Tegretol, Epitol, Equetro)

Carbamazepine (Carbatrol, Tegretol, Epitol, Equetro)

Chorea in Adults Medication

Medication Summary

Antipsychotic agents

Class Summary

Haloperidol (Haldol)

Fluphenazine

Clozapine (Clozaril, FazaClo, Versacloz)

Olanzapine (Zyprexa, Zyprexa Relprevv, Zyprexa Zydis )

Risperidone (Risperdal)

Quetiapine (Seroquel)

Monoamine depleting agents

Class Summary

Reserpine

Tetrabenazine (Xenazine)

Deutetrabenazine (Austedo)

Valbenazine (Ingrezza)

Benzodiazepines

Class Summary

Clonazepam (Klonopin)

Anticonvulsants

Class Summary

Valproic acid (Depacon, Depakote, Depakote ER, Depakene)

Carbamazepine (Carbatrol, Tegretol, Epitol, Equetro)

References

Tables

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