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Huntington Disease Treatment & Management

  • Author: Fredy J Revilla, MD; Chief Editor: Selim R Benbadis, MD  more...
 
Updated: Jul 08, 2016
 

Surgical Care

Ablative surgical procedures and fetal cell transplantation have been attempted in patients with HD. Currently, enough data to support this type of treatment are not available. It is still experimental.

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Approach Consideration

Consider general safety measures and nonpharmacologic interventions first in the management of Huntington disease (HD).

If chorea is severe enough to interfere with function, consider treatment with benzodiazepines, such as clonazepam or diazepam; valproic acid; dopamine-depleting agents, such as reserpine or tetrabenazine (approved by the US Food and Drug Administration [FDA] in August 2008); and finally, neuroleptics.

The drug tetrabenazine has shown some positive effects in the treatment of chorea, for patients with HD. It selectively depletes central monoamines by reversibly binding to the type-2 vesicular monoamine transporter.

Results from a phase III clinical study showed that this investigational drug is an effective treatment for chorea associated with HD. The dosing range that proved effective was 12.5-100 mg/d.[16] Its manufacturer has been granted fast track and orphan drug status by the FDA. It is the first treatment for chorea in patients with HD in the United States. Always weigh potential adverse effects against the benefits of each drug.

Patients who have HD and predominant features of bradykinesia and rigidity may benefit from treatment with levodopa or dopamine agonists.[17]

Depression in patients with HD is treatable and should be recognized promptly. Selective serotonin reuptake inhibitors (SSRIs) should be considered as first-line therapy. Other antidepressants, including bupropion, venlafaxine, nefazodone, and tricyclic antidepressants, also can be used. Electroconvulsive therapy (ECT) can be used in patients with refractory depression.

Antipsychotic medications may be necessary in patients with hallucinations, delusions, or schizophrenia-like syndromes. Newer agents, such as quetiapine, clozapine, olanzapine, and risperidone, are preferred to older agents because of the lower incidence of extrapyramidal side effects and the decreased risk for tardive syndromes.

Irritability may be treated with antidepressants, particularly the SSRIs; mood stabilizers, such as valproic acid or carbamazepine; and, if needed, atypical neuroleptics.

Other less frequent aspects of HD that may require pharmacologic treatment are mania, obsessive-compulsive disorder, anxiety, sexual disorders, myoclonus, tics, dystonia, and epilepsy.

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Contributor Information and Disclosures
Author

Fredy J Revilla, MD Associate Professor of Neurology, Director, Movement Disorders Center, James J and Joan A Gardner Family Center Endowed Chair for Parkinson's Disease and Other Movement Disorders, University of Cincinnati College of Medicine; Staff Physician/Neurologist, Cincinnati Veterans Affairs Medical Center; Staff Physician/Neurologist, Huntington's Disease Clinic, Cincinnati Children's Hospital Medical Center

Fredy J Revilla, MD is a member of the following medical societies: American Academy of Neurology, International Parkinson and Movement Disorder Society, Society for Neuroscience

Disclosure: Nothing to disclose.

Coauthor(s)

Jaime Grutzendler, MD Assistant Professor, Department of Neurology and Physiology, Northwestern University School of Medicine

Jaime Grutzendler, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience

Disclosure: Nothing to disclose.

Travis R Larsh Department of Neurology, University of Cincinnati College 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 College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Additional Contributors

Robert A Hauser, MD, MBA Professor of Neurology, Molecular Pharmacology and Physiology, Director, USF Parkinson's Disease and Movement Disorders Center, National Parkinson Foundation Center of Excellence, Byrd Institute, Clinical Chair, Signature Interdisciplinary Program in Neuroscience, University of South Florida College of Medicine

Robert A Hauser, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Neuroimaging, International Parkinson and Movement Disorder Society

Disclosure: Received consulting fee from Cerecor for consulting; Received consulting fee from L&M Healthcare for consulting; Received consulting fee from Cleveland Clinic for consulting; Received consulting fee from Heptares for consulting; Received consulting fee from Gerrson Lehrman Group for consulting; Received consulting fee from Indus for consulting; Received consulting fee from University of Houston for consulting; Received consulting fee from AbbVie for consulting; Received consulting fee from Adama.

References
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  12. R. Avila-Giron. Medical and Social Aspects of Huntington's Chorea in the State of Zulia, Venezuela. A. Barbeau, T.N. Chase and G.W. Paulson. Advances in Neurology. New York: Raven Press; 1973. Volume 1: 261-266.

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  14. Pringsheim T, Wiltshire K, Day L, Dykeman J, Steeves T, Jette N. The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis. Mov Disord. 2012 Aug. 27 (9):1083-91. [Medline].

  15. Quaid KA. Presymptomatic testing for Huntington disease in the United States. Am J Hum Genet. Sep 1993. 53(3):785-7. [Medline].

  16. Ondo WG, Tintner R, Thomas M, Jankovic J. Tetrabenazine treatment for Huntington's disease-associated chorea. Clin Neuropharmacol. Nov-Dec 2002. 25(6):300-2. [Medline].

  17. Racette BA, Perlmutter JS. Levodopa responsive parkinsonism in an adult with Huntington's disease. J Neurol Neurosurg Psychiatry. Oct 1998. 65(4):577-9. [Medline].

 
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