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Medication

Medication Summary

Medication for chorea and dyskinesia is usually inadequate but is worth trying.

Haloperidol, tetrabenazine, and diazepam have been used in high doses in selected patients with NA. Only modest temporary benefit can be expected. Levetiracetam and topiramate have shown some benefit in secondary choreas and may be considered in treating NA. Levetiracetam has also been shown to help a patient with truncal tic.

Rhabdomyolysis and the neuroleptic malignant syndrome are always concerns in patients with NA, particularly because compliance and swallowing problems may lead to undesired daily variations of the dopamine blocking agent blood levels.

Even when no dramatic haloperidol dosing changes could be documented, Robinson noted rhabdomyolysis in a patient with NA.^[35]After treatment for the life-threatening condition, the patient was switched from haloperidol to a combination of molindone and divalproex to effectively reduce involuntary movements.

Epilepsy management is very individualized. Phenytoin has been used successfully. Carbamazepine and lamotrigine have been reported to worsen the involuntary movements.

Digitalis and diuretics have been used successfully for congestive heart failure in patients with NA and cardiomyopathy.

Anticonvulsant therapy with phenytoin has been successful in patients with generalized tonic-clonic seizures.

In view of potential anticholinergic problems in patients with cardiomyopathy, selective serotonin reuptake inhibitors (SSRIs) are probably a better choice than tricyclic antidepressants.

Citalopram was found to help a patient with NA and obsessive-compulsive disorder.^[36]

Generally, if hemolytic anemia is noted owing to acanthocyte accumulation/degradation, it is mild and does not require treatment.

In a case report, electroconvulsive therapy helped only speech but not chorea and other progressive neurologic problems.^[37]

New protocols similar to those for Huntington disease are worth discussing with the medical treatment team. Agents such as the atypical antipsychotics for chorea and anti-Parkinson disease drugs for patients with NA and Parkinson disease features may be of value. Newer antidepressant drugs may also have a role, particularly SSRIs.

Neuroprotective agents and gene therapy may also have key future roles.

Class Summary

Chorea, tics, and other adventitious movements such as oral dyskinesia may respond to drugs that block dopamine receptors and facilitate GABA transmission.

Haloperidol (Haldol)

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In general, can decrease chorea, tics, and dyskinesia through blockade of dopamine receptors of CNS.

Class Summary

An agent that can decrease anxiety, such as a benzodiazepine, can also decrease movement disorders often made worse by associated stress.

Diazepam (Valium)

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Long-acting benzodiazepine that can decrease anxiety in patients with NA throughout the day.

Follow-up

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Patient with choreoacanthocytosis. A: Note self-mutilation of the lips owing to orofacial dyskinesia. B: Peripheral blood smear exhibits acanthocytes (Wright-Giemsa, original magnification, >100). C: Coronal view of T1-weighted MRI shows atrophy of the caudate nuclei. Archives of Neurology 64(11):1661-1664 2007 Copyright © 2010 American Medical Association.

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Author

Kenneth B V Gross, MD Founder, Owner, Fusion Clinical Multimedia, Inc, Division of Neurology, Fusion Clinical Multimedia Educational Center

Kenneth B V Gross, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Coauthor(s)

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and CEO/CMO, PHLT Consultants; Former Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, CPE, MHCM, FAAPL is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for Physician Leadership

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.

Nestor Galvez-Jimenez, MD, MSc, MHA The Pauline M Braathen Endowed Chair in Neurology, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

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, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

Additional Contributors

Roberta J Seidman, MD Associate Professor of Pathology, Director of Neuropathology, Department of Pathology, Renaissance School of Medicine at Stony Brook University, Stony Brook Medicine

Roberta J Seidman, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, New York Association of Neuropathologists (The Neuroplex)

Disclosure: Nothing to disclose.

Neuroacanthocytosis Syndromes Medication

Medication Summary

Antipsychotic Agents

Class Summary

Haloperidol (Haldol)

Haloperidol (Haldol)

Anxiolytic

Class Summary

Diazepam (Valium)

Diazepam (Valium)

Neuroacanthocytosis Syndromes Medication

Medication Summary

Antipsychotic Agents

Class Summary

Haloperidol (Haldol)

Haloperidol (Haldol)

Anxiolytic

Class Summary

Diazepam (Valium)

Diazepam (Valium)

References

Tables

Contributor Information and Disclosures

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