UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Huntington disease: Management

Author
Oksana Suchowersky, MD, FRCPC, FCCMG
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

Huntington disease (HD) is an inherited progressive neurodegenerative disorder characterized by choreiform movements, psychiatric problems, and dementia. It is caused by a trinucleotide (cytosine-adenine-guanine [CAG]) expansion in the Huntington gene on chromosome 4p and inherited in an autosomal dominant pattern. As there is no known cure, treatment is symptomatic and remains supportive.

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 [1]. Despite these difficult circumstances, practitioners must be vigilant in caring for patients with HD and their families.

Multidisciplinary care — The best care for patients with HD is provided by a multidisciplinary team of health professionals and supportive caregivers that addresses the broad physical and psychological needs of patients and families, and manages new issues as they arise through long-term follow-up [2]. Ideally, the health care team includes a variety of professionals and covers various areas of expertise that may be needed over time to support the patient with HD, including neurology, psychiatry, genetics, social work, and nutrition (table 1) [2-4]. Use of physical therapy, occupational therapy, and home care may allow for prolongation of community living, although there is a dearth of supporting literature.

Palliative care and advance care planning — Palliative care focuses on preventing and relieving suffering and on supporting the best possible quality of life for patients and their families facing serious illness. Although HD is a chronic disease with a relatively slow course, the principles of palliative care are appropriate throughout the disease progression.

                          
To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Dec 21, 2017.
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 ©2018 UpToDate, Inc.
References
Top
  1. Rosenblatt A. Overview and principles of treatment. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, 2011. p.5.
  2. Nance M. Team care for Huntington’s disease. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, p.27.
  3. Klimek ML, Rohs G, Young L, et al. Multidisciplinary approach to management of a hereditary neurodegenerative disorder: Huntington disease. Axone 1997; 19:34.
  4. Tarolli CG, Chesire AM, Biglan KM. Palliative Care in Huntington Disease: Personal Reflections and a Review of the Literature. Tremor Other Hyperkinet Mov (N Y) 2017; 7:454.
  5. Nance M. Management of late stage HD. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, 2011. p.99.
  6. Miyasaki JM. Palliative care in Parkinson's disease. Curr Neurol Neurosci Rep 2013; 13:367.
  7. Jankovic J, Roos RA. Chorea associated with Huntington's disease: to treat or not to treat? Mov Disord 2014; 29:1414.
  8. Wheelock V. The motor disorder. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, 2011. p.39.
  9. 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.
  10. Dallocchio C, Buffa C, Tinelli C, Mazzarello P. Effectiveness of risperidone in Huntington chorea patients. J Clin Psychopharmacol 1999; 19:101.
  11. Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev 2009; :CD006456.
  12. Huntington Study Group. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology 2006; 66:366.
  13. 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.
  14. Tetrabenazine (Xenazine) for Huntington's chorea. Med Lett Drugs Ther 2009; 51:7.
  15. Guay DR. Tetrabenazine, a monoamine-depleting drug used in the treatment of hyperkinetic movement disorders. Am J Geriatr Pharmacother 2010; 8:331.
  16. Huntington Study Group, Frank S, Testa CM, et al. Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial. JAMA 2016; 316:40.
  17. Geschwind MD, Paras N. Deutetrabenazine for Treatment of Chorea in Huntington Disease. JAMA 2016; 316:33.
  18. Frank S, Stamler D, Kayson E, et al. Safety of Converting From Tetrabenazine to Deutetrabenazine for the Treatment of Chorea. JAMA Neurol 2017; 74:977.
  19. 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.
  20. Bonelli RM, Wenning GK. Pharmacological management of Huntington's disease: an evidence-based review. Curr Pharm Des 2006; 12:2701.
  21. Bonelli RM, Mahnert FA, Niederwieser G. Olanzapine for Huntington's disease: an open label study. Clin Neuropharmacol 2002; 25:263.
  22. Madhusoodanan S, Brenner R. Use of risperidone in psychosis associated with Huntington's disease. Am J Geriatr Psychiatry 1998; 6:347.
  23. Erdemoglu AK, Boratav C. Risperidone in chorea and psychosis of Huntington's disease. Eur J Neurol 2002; 9:182.
  24. Cankurtaran ES, Ozalp E, Soygur H, Cakir A. Clinical experience with risperidone and memantine in the treatment of Huntington's disease. J Natl Med Assoc 2006; 98:1353.
  25. Reveley MA, Dursun SM, Andrews H. A comparative trial use of sulpiride and risperidone in Huntington's disease: a pilot study. J Psychopharmacol 1996; 10:162.
  26. Duff K, Beglinger LJ, O'Rourke ME, et al. Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Ann Clin Psychiatry 2008; 20:1.
  27. 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.
  28. 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.
  29. Bonelli RM, Niederwieser G. Quetiapine in Huntington's disease: a first case report. J Neurol 2002; 249:1114.
  30. O'Suilleabhain P, Dewey RB Jr. A randomized trial of amantadine in Huntington disease. Arch Neurol 2003; 60:996.
  31. 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.
  32. 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.
  33. 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.
  34. 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.
  35. Huntington Study Group. Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 2003; 61:1551.
  36. Reilmann R. Pharmacological treatment of chorea in Huntington's disease-good clinical practice versus evidence-based guideline. Mov Disord 2013; 28:1030.
  37. 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.
  38. Consroe P, Laguna J, Allender J, et al. Controlled clinical trial of cannabidiol in Huntington's disease. Pharmacol Biochem Behav 1991; 40:701.
  39. 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.
  40. 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.
  41. Gatto EM, Uribe Roca C, Raina G, et al. Vascular hemichorea/hemiballism and topiramate. Mov Disord 2004; 19:836.
  42. Racette BA, Perlmutter JS. Levodopa responsive parkinsonism in an adult with Huntington's disease. J Neurol Neurosurg Psychiatry 1998; 65:577.
  43. 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.
  44. van Duijn E. Medical treatment of behavioral manifestations of Huntington disease. Handb Clin Neurol 2017; 144:129.
  45. Chou KL, Borek LL, Friedman JH. The management of psychosis in movement disorder patients. Expert Opin Pharmacother 2007; 8:935.
  46. Seitz DP, Millson RC. Quetiapine in the management of psychosis secondary to huntington's disease: a case report. Can J Psychiatry 2004; 49:413.
  47. Alpay M, Koroshetz WJ. Quetiapine in the treatment of behavioral disturbances in patients with Huntington's disease. Psychosomatics 2006; 47:70.
  48. Paleacu D, Anca M, Giladi N. Olanzapine in Huntington's disease. Acta Neurol Scand 2002; 105:441.
  49. Laks J, Rocha M, Capitão C, et al. Functional and motor response to low dose olanzapine in Huntington's disease: case report. Arq Neuropsiquiatr 2004; 62:1092.
  50. 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.
  51. Lin WC, Chou YH. Aripiprazole effects on psychosis and chorea in a patient with Huntington's disease. Am J Psychiatry 2008; 165:1207.
  52. Oulis P, Mourikis I, Konstantakopoulos G, et al. Aripiprazole in the treatment of olanzapine-resistant psychotic and motor symptoms of Huntington's disease. J Neuropsychiatry Clin Neurosci 2010; 22:352c.e4.
  53. Rosenblatt A. The psychiatric disorder. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, 2011. p.63.
  54. Fernandez HH, Friedman JH, Grace J, Beason-Hazen S. Donepezil for Huntington's disease. Mov Disord 2000; 15:173.
  55. Rot U, Kobal J, Sever A, et al. Rivastigmine in the treatment of Huntington's disease. Eur J Neurol 2002; 9:689.
  56. 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.
  57. Petrikis P, Andreou C, Piachas A, et al. Treatment of Huntington's disease with galantamine. Int Clin Psychopharmacol 2004; 19:49.
  58. Saft C, Andrich JE, Müller T, et al. Oral and dental health in Huntington's disease - an observational study. BMC Neurol 2013; 13:114.
  59. Paulsen JS. The cognitive disorder. In: A Physician's Guide to the Management of Huntington's Disease, 3rd edition, Nance M, Paulsen JS, Rosenblatt A, Wheelock V (Eds), Huntington's Disease Society of America, 2011. p.51.
  60. Mestre T, Ferreira J, Coelho MM, et al. Therapeutic interventions for disease progression in Huntington's disease. Cochrane Database Syst Rev 2009; :CD006455.
  61. Wild EJ, Tabrizi SJ. Targets for future clinical trials in Huntington's disease: what's in the pipeline? Mov Disord 2014; 29:1434.
  62. Shannon KM, Fraint A. Therapeutic advances in Huntington's Disease. Mov Disord 2015; 30:1539.
  63. Wild EJ, Tabrizi SJ. Therapies targeting DNA and RNA in Huntington's disease. Lancet Neurol 2017; 16:837.
  64. Harper SQ. Progress and challenges in RNA interference therapy for Huntington disease. Arch Neurol 2009; 66:933.
  65. 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.
  66. Lu XH, Yang XW. "Huntingtin holiday": progress toward an antisense therapy for Huntington's disease. Neuron 2012; 74:964.