Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Huntington disease: Management

INTRODUCTION

Huntington disease (HD) is an inherited progressive neurodegenerative disorder characterized by choreiform movements, psychiatric problems, and dementia. It is caused by a trinucleotide (CAG) expansion in the Huntington gene on chromosome 4p and inherited in an autosomal-dominant pattern. The pathophysiology of HD is not fully understood, although it is thought to be related to toxicity of the mutant huntingtin protein. As there is no known cure, treatment is symptomatic and remains supportive. Genetic mouse models are expanding our understanding of the disease and may lead to disease modifying therapy in the future.

This topic will review the management of HD. Other aspects of HD are discussed separately. (See "Huntington disease: Genetics and pathogenesis" and "Huntington disease: Clinical features and diagnosis".)

SUPPORTIVE CARE

No cure or disease-modifying treatment is currently available for HD. Therapy is focused on symptom management and supportive care in order to optimize quality of life. Despite these difficult circumstances, practitioners must be vigilant in caring for patients with HD and their families. The best care is provided by an interdisciplinary team that addresses the broad physical and psychological needs of patients and families, and manages new issues as they arise through long-term follow-up.

Use of physiotherapy, occupational therapy, and home care may allow for prolongation of community living, although there is a dearth of supporting literature.

Speech therapy and dietary services are beneficial in managing dysphagia and weight loss.

                         

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Aug 2014. | This topic last updated: Apr 22, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Rosenblatt A, Ranen NG, Nance MA, Paulsen JS. A physician's guide to the management of Huntington's disease, 2nd Ed, Huntington’s Disease Society of America, New York 1999. www.hdsa.org/images/content/1/1/11289.pdf (Accessed on January 24, 2011).
  2. Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev 2009; :CD006456.
  3. Armstrong MJ, Miyasaki JM, American Academy of Neurology. Evidence-based guideline: pharmacologic treatment of chorea in Huntington disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology 2012; 79:597.
  4. Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology 2006; 66:366.
  5. Frank S, Ondo W, Fahn S, et al. A study of chorea after tetrabenazine withdrawal in patients with Huntington disease. Clin Neuropharmacol 2008; 31:127.
  6. Ondo WG, Tintner R, Thomas M, Jankovic J. Tetrabenazine treatment for Huntington's disease-associated chorea. Clin Neuropharmacol 2002; 25:300.
  7. Guay DR. Tetrabenazine, a monoamine-depleting drug used in the treatment of hyperkinetic movement disorders. Am J Geriatr Pharmacother 2010; 8:331.
  8. Tetrabenazine (Xenazine) for Huntington's chorea. Med Lett Drugs Ther 2009; 51:7.
  9. Dallocchio C, Buffa C, Tinelli C, Mazzarello P. Effectiveness of risperidone in Huntington chorea patients. J Clin Psychopharmacol 1999; 19:101.
  10. Bonelli RM, Wenning GK. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des 2006; 12:2701.
  11. Bonelli RM, Mahnert FA, Niederwieser G. Olanzapine for Huntington's disease: an open label study. Clin Neuropharmacol 2002; 25:263.
  12. Madhusoodanan S, Brenner R. Use of risperidone in psychosis associated with Huntington's disease. Am J Geriatr Psychiatry 1998; 6:347.
  13. Meco G, Bonifati V, Alessandri A, Brusa L. Risperidone in Huntington's disease. Hum Psychopharmacol 1995; 10:353.
  14. van Vugt JP, Siesling S, Vergeer M, et al. Clozapine versus placebo in Huntington's disease: a double blind randomised comparative study. J Neurol Neurosurg Psychiatry 1997; 63:35.
  15. Ciammola A, Sassone J, Colciago C, et al. Aripiprazole in the treatment of Huntington's disease: a case series. Neuropsychiatr Dis Treat 2009; 5:1.
  16. Brusa L, Orlacchio A, Moschella V, et al. Treatment of the symptoms of Huntington's disease: preliminary results comparing aripiprazole and tetrabenazine. Mov Disord 2009; 24:126.
  17. Bonelli RM, Niederwieser G. Quetiapine in Huntington's disease: a first case report. J Neurol 2002; 249:1114.
  18. O'Suilleabhain P, Dewey RB Jr. A randomized trial of amantadine in Huntington disease. Arch Neurol 2003; 60:996.
  19. Heckmann JM, Legg P, Sklar D, et al. IV amantadine improves chorea in Huntington's disease: an acute randomized, controlled study. Neurology 2004; 63:597.
  20. Verhagen Metman L, Morris MJ, Farmer C, et al. Huntington's disease: a randomized, controlled trial using the NMDA-antagonist amantadine. Neurology 2002; 59:694.
  21. Lucetti C, Del Dotto P, Gambaccini G, et al. IV amantadine improves chorea in Huntington's disease: an acute randomized, controlled study. Neurology 2003; 60:1995.
  22. Landwehrmeyer GB, Dubois B, de Yébenes JG, et al. Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol 2007; 62:262.
  23. Huntington Study Group. Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 2003; 61:1551.
  24. Reilmann R. Pharmacological treatment of chorea in Huntington's disease-good clinical practice versus evidence-based guideline. Mov Disord 2013; 28:1030.
  25. Lacomblez L, Bensimon G, Leigh PN, et al. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet 1996; 347:1425.
  26. Consroe P, Laguna J, Allender J, et al. Controlled clinical trial of cannabidiol in Huntington's disease. Pharmacol Biochem Behav 1991; 40:701.
  27. Curtis A, Mitchell I, Patel S, et al. A pilot study using nabilone for symptomatic treatment in Huntington's disease. Mov Disord 2009; 24:2254.
  28. Zesiewicz TA, Sullivan KL, Hauser RA, Sanchez-Ramos J. Open-label pilot study of levetiracetam (Keppra) for the treatment of chorea in Huntington's disease. Mov Disord 2006; 21:1998.
  29. Gatto EM, Uribe Roca C, Raina G, et al. Vascular hemichorea/hemiballism and topiramate. Mov Disord 2004; 19:836.
  30. Racette BA, Perlmutter JS. Levodopa responsive parkinsonism in an adult with Huntington's disease. J Neurol Neurosurg Psychiatry 1998; 65:577.
  31. Bonelli RM, Niederwieser G, Diez J, et al. Pramipexole ameliorates neurologic and psychiatric symptoms in a Westphal variant of Huntington's disease. Clin Neuropharmacol 2002; 25:58.
  32. Seitz DP, Millson RC. Quetiapine in the management of psychosis secondary to huntington's disease: a case report. Can J Psychiatry 2004; 49:413.
  33. Alpay M, Koroshetz WJ. Quetiapine in the treatment of behavioral disturbances in patients with Huntington's disease. Psychosomatics 2006; 47:70.
  34. Madhusoodanan S, Brenner R, Moise D, et al. Psychiatric and neuropsychological abnormalities in Huntington's disease: a case study. Ann Clin Psychiatry 1998; 10:117.
  35. Erdemoglu AK, Boratav C. Risperidone in chorea and psychosis of Huntington's disease. Eur J Neurol 2002; 9:182.
  36. Paleacu D, Anca M, Giladi N. Olanzapine in Huntington's disease. Acta Neurol Scand 2002; 105:441.
  37. Fernandez HH, Friedman JH, Grace J, Beason-Hazen S. Donepezil for Huntington's disease. Mov Disord 2000; 15:173.
  38. Rot U, Kobal J, Sever A, et al. Rivastigmine in the treatment of Huntington's disease. Eur J Neurol 2002; 9:689.
  39. de Tommaso M, Specchio N, Sciruicchio V, et al. Effects of rivastigmine on motor and cognitive impairment in Huntington's disease. Mov Disord 2004; 19:1516.
  40. Petrikis P, Andreou C, Piachas A, et al. Treatment of Huntington's disease with galantamine. Int Clin Psychopharmacol 2004; 19:49.
  41. Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for disease progression in Huntington's disease. Cochrane Database Syst Rev 2009; :CD006455.
  42. Munoz-Sanjuan I, Bates GP. The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease. J Clin Invest 2011; 121:476.
  43. Sah DW, Aronin N. Oligonucleotide therapeutic approaches for Huntington disease. J Clin Invest 2011; 121:500.
  44. de Yebenes JG, Landwehrmeyer B, Squitieri F, et al. Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2011; 10:1049.
  45. Huntington Study Group HART Investigators. A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease. Mov Disord 2013; 28:1407.
  46. Verbessem P, Lemiere J, Eijnde BO, et al. Creatine supplementation in Huntington's disease: a placebo-controlled pilot trial. Neurology 2003; 61:925.
  47. Bender A, Auer DP, Merl T, et al. Creatine supplementation lowers brain glutamate levels in Huntington's disease. J Neurol 2005; 252:36.
  48. Tabrizi SJ, Blamire AM, Manners DN, et al. Creatine therapy for Huntington's disease: clinical and MRS findings in a 1-year pilot study. Neurology 2003; 61:141.
  49. Tabrizi SJ, Blamire AM, Manners DN, et al. High-dose creatine therapy for Huntington disease: a 2-year clinical and MRS study. Neurology 2005; 64:1655.
  50. Hersch SM, Gevorkian S, Marder K, et al. Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG. Neurology 2006; 66:250.
  51. Creatine safety, tolerability, and efficacy in Huntington's disease (CREST-E). http://clinicaltrials.gov/ct2/show/NCT00712426 (Accessed on January 26, 2012).
  52. Huntington Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001; 57:397.
  53. Coenzyme Q10 in Huntington's disease. http://clinicaltrials.gov/ct2/show/NCT00608881 (Accessed on January 26, 2012).
  54. Vaddadi KS, Soosai E, Chiu E, Dingjan P. A randomised, placebo-controlled, double blind study of treatment of Huntington's disease with unsaturated fatty acids. Neuroreport 2002; 13:29.
  55. Puri BK, Leavitt BR, Hayden MR, et al. Ethyl-EPA in Huntington disease: a double-blind, randomized, placebo-controlled trial. Neurology 2005; 65:286.
  56. Huntington Study Group TREND-HD Investigators. Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study. Arch Neurol 2008; 65:1582.
  57. Huntington Study Group DOMINO Investigators. A futility study of minocycline in Huntington's disease. Mov Disord 2010; 25:2219.
  58. Yamamoto A, Lucas JJ, Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000; 101:57.
  59. Martín-Aparicio E, Yamamoto A, Hernández F, et al. Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease. J Neurosci 2001; 21:8772.
  60. Harper SQ. Progress and challenges in RNA interference therapy for Huntington disease. Arch Neurol 2009; 66:933.
  61. Kordasiewicz HB, Stanek LM, Wancewicz EV, et al. Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron 2012; 74:1031.
  62. Lu XH, Yang XW. "Huntingtin holiday": progress toward an antisense therapy for Huntington's disease. Neuron 2012; 74:964.
  63. Miller TW, Shirley TL, Wolfgang WJ, et al. DNA vaccination against mutant huntingtin ameliorates the HDR6/2 diabetic phenotype. Mol Ther 2003; 7:572.
  64. Moro E, Lang AE, Strafella AP, et al. Bilateral globus pallidus stimulation for Huntington's disease. Ann Neurol 2004; 56:290.
  65. Cubo E, Shannon KM, Penn RD, Kroin JS. Internal globus pallidotomy in dystonia secondary to Huntington's disease. Mov Disord 2000; 15:1248.
  66. Hauser RA, Furtado S, Cimino CR, et al. Bilateral human fetal striatal transplantation in Huntington's disease. Neurology 2002; 58:687.
  67. Bachoud-Lévi AC, Hantraye P, Peschanski M. Fetal neural grafts for Huntington's disease: a prospective view. Mov Disord 2002; 17:439.
  68. Bachoud-Lévi AC, Gaura V, Brugières P, et al. Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery: a long-term follow-up study. Lancet Neurol 2006; 5:303.
  69. Wijeyekoon R, Barker RA. The Current Status of Neural Grafting in the Treatment of Huntington's Disease. A Review. Front Integr Neurosci 2011; 5:78.
  70. Alberch J, Pérez-Navarro E, Canals JM. Neurotrophic factors in Huntington's disease. Prog Brain Res 2004; 146:195.
  71. Emerich DF, Thanos CG. Intracompartmental delivery of CNTF as therapy for Huntington's disease and retinitis pigmentosa. Curr Gene Ther 2006; 6:147.
  72. Zuccato C, Cattaneo E. Role of brain-derived neurotrophic factor in Huntington's disease. Prog Neurobiol 2007; 81:294.
  73. Bloch J, Bachoud-Lévi AC, Déglon N, et al. Neuroprotective gene therapy for Huntington's disease, using polymer-encapsulated cells engineered to secrete human ciliary neurotrophic factor: results of a phase I study. Hum Gene Ther 2004; 15:968.