Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Treatment of myoclonus

John N Caviness, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD


Myoclonus is a clinical sign characterized by sudden, brief, shock-like, involuntary movements caused by muscular contractions or inhibitions. Muscular contractions produce "positive myoclonus," whereas muscular inhibitions produce "negative myoclonus" or asterixis. Myoclonic movements have many possible etiologies, anatomic sources, and pathophysiologic features. Treatment is generally guided by the anatomic and physiologic classification of the myoclonus in question.

This topic will review the treatment of myoclonus. The treatment of myoclonic epilepsy is discussed separately. (See "Epilepsy syndromes in children", section on 'Juvenile myoclonic epilepsy' and "Epilepsy syndromes in children", section on 'Myoclonic epilepsy of infancy' and "Epilepsy syndromes in children", section on 'Absence epilepsies'.)

Other clinical aspects of myoclonus are reviewed elsewhere. (See "Classification and evaluation of myoclonus" and "Symptomatic (secondary) myoclonus".)


The classification of myoclonus is important because it is helpful to guide treatment choices for the many different types of myoclonus. The classification of myoclonus is reviewed here briefly, and is discussed in greater detail separately. (See "Classification and evaluation of myoclonus" and "Symptomatic (secondary) myoclonus".)

The most useful scheme for therapeutic decisions classifies myoclonus by the localization of the physiologic mechanism that generates it (table 1). These anatomic-physiologic categories (along with important examples) are as follows:

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: Nov 2017. | This topic last updated: Oct 03, 2016.
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 ©2017 UpToDate, Inc.
  1. Hinson VK, Weinstein S, Bernard B, et al. Single-blind clinical trial of psychotherapy for treatment of psychogenic movement disorders. Parkinsonism Relat Disord 2006; 12:177.
  2. Caviness JN. Treatment of myoclonus. Neurotherapeutics 2014; 11:188.
  3. Crest C, Dupont S, Leguern E, et al. Levetiracetam in progressive myoclonic epilepsy: an exploratory study in 9 patients. Neurology 2004; 62:640.
  4. Lim LL, Ahmed A. Limited efficacy of levetiracetam on myoclonus of different etiologies. Parkinsonism Relat Disord 2005; 11:135.
  5. Striano P, Manganelli F, Boccella P, et al. Levetiracetam in patients with cortical myoclonus: a clinical and electrophysiological study. Mov Disord 2005; 20:1610.
  6. Genton P, Gélisse P. Antimyoclonic effect of levetiracetam. Epileptic Disord 2000; 2:209.
  7. Krauss GL, Bergin A, Kramer RE, et al. Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam. Neurology 2001; 56:411.
  8. Frucht SJ, Louis ED, Chuang C, Fahn S. A pilot tolerability and efficacy study of levetiracetam in patients with chronic myoclonus. Neurology 2001; 57:1112.
  9. Magaudda A, Gelisse P, Genton P. Antimyoclonic effect of levetiracetam in 13 patients with Unverricht-Lundborg disease: clinical observations. Epilepsia 2004; 45:678.
  10. Gelisse P, Crespel A, Genton P, Baldy-Moulinier M. Dramatic effect of levetiracetam on epileptic negative myoclonus. Acta Neurol Scand 2003; 107:302.
  11. Imperiale D, Bortolotto S, Cucatto A, et al. Levetiracetam control of myoclonus in a patient with Creutzfeldt-Jakob disease. Eur Neurol 2003; 49:189.
  12. Mancuso M, Galli R, Pizzanelli C, et al. Antimyoclonic effect of levetiracetam in MERRF syndrome. J Neurol Sci 2006; 243:97.
  13. Kinrions P, Ibrahim N, Murphy K, et al. Efficacy of levetiracetam in a patient with Unverricht-Lundborg progressive myoclonic epilepsy. Neurology 2003; 60:1394.
  14. Cho JW, Lee JH. Suppression of myoclonus in corticobasal degeneration by levetiracetam. J Mov Disord 2014; 7:28.
  15. Keswani SC, Kossoff EH, Krauss GL, Hagerty C. Amelioration of spinal myoclonus with levetiracetam. J Neurol Neurosurg Psychiatry 2002; 73:457.
  16. Brown P, Steiger MJ, Thompson PD, et al. Effectiveness of piracetam in cortical myoclonus. Mov Disord 1993; 8:63.
  17. Ikeda A, Shibasaki H, Tashiro K, et al. Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. The Myoclonus/Piracetam Study Group. Mov Disord 1996; 11:691.
  18. Koskiniemi M, Van Vleymen B, Hakamies L, et al. Piracetam relieves symptoms in progressive myoclonus epilepsy: a multicentre, randomised, double blind, crossover study comparing the efficacy and safety of three dosages of oral piracetam with placebo. J Neurol Neurosurg Psychiatry 1998; 64:344.
  19. Fedi M, Reutens D, Dubeau F, et al. Long-term efficacy and safety of piracetam in the treatment of progressive myoclonus epilepsy. Arch Neurol 2001; 58:781.
  20. Obeso JA, Artieda J, Rothwell JC, et al. The treatment of severe action myoclonus. Brain 1989; 112 ( Pt 3):765.
  21. Obeso JA. Therapy of myoclonus. Clin Neurosci 1995-1996; 3:253.
  22. Bressman S, Fahn S. Essential myoclonus. Adv Neurol 1986; 43:287.
  23. Tijssen MA, Schoemaker HC, Edelbroek PJ, et al. The effects of clonazepam and vigabatrin in hyperekplexia. J Neurol Sci 1997; 149:63.
  24. Montagna P, Provini F, Plazzi G, et al. Propriospinal myoclonus upon relaxation and drowsiness: a cause of severe insomnia. Mov Disord 1997; 12:66.
  25. Fabiani G, Teive HA, Sá D, et al. Palatal myoclonus: report of two cases. Arq Neuropsiquiatr 2000; 58:901.
  26. Jankovic J, Pardo R. Segmental myoclonus. Clinical and pharmacologic study. Arch Neurol 1986; 43:1025.
  27. Fahn S. Posthypoxic action myoclonus: review of the literature and report of two new cases with response to valproate and estrogen. Adv Neurol 1979; 26:49.
  28. Borggreve F, Hageman G. A case of idiopathic palatal myoclonus: treatment with sodium valproate. Eur Neurol 1991; 31:403.
  29. Sumer M. Symptomatic palatal myoclonus: an unusual cause of respiratory difficulty. Acta Neurol Belg 2001; 101:113.
  30. Hainque E, Vidailhet M, Cozic N, et al. A randomized, controlled, double-blind, crossover trial of zonisamide in myoclonus-dystonia. Neurology 2016; 86:1729.
  31. Kyllerman M, Ben-Menachem E. Zonisamide for progressive myoclonus epilepsy: long-term observations in seven patients. Epilepsy Res 1998; 29:109.
  32. Yoshimura I, Kaneko S, Yoshimura N, Murakami T. Long-term observations of two siblings with Lafora disease treated with zonisamide. Epilepsy Res 2001; 46:283.
  33. Roze E, Bounolleau P, Ducreux D, et al. Propriospinal myoclonus revisited: Clinical, neurophysiologic, and neuroradiologic findings. Neurology 2009; 72:1301.
  34. Baizabal-Carvallo JF, Jankovic J. Movement disorders in autoimmune diseases. Mov Disord 2012; 27:935.
  35. Tan KM, Lennon VA, Klein CJ, et al. Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology 2008; 70:1883.
  36. Sinha S, Sarin YK. Rituximab for opsoclonus myoclonus ataxia syndrome associated with neuroblastoma. Indian J Pediatr 2014; 81:218.
  37. Krause E, Leunig A, Klopstock T, Gürkov R. Treatment of essential palatal myoclonus in a 10-year-old girl with botulinum neurotoxin. Otol Neurotol 2006; 27:672.
  38. Penney SE, Bruce IA, Saeed SR. Botulinum toxin is effective and safe for palatal tremor: a report of five cases and a review of the literature. J Neurol 2006; 253:857.
  39. Sinclair CF, Gurey LE, Blitzer A. Palatal myoclonus: algorithm for management with botulinum toxin based on clinical disease characteristics. Laryngoscope 2014; 124:1164.
  40. Lagueny A, Tison F, Burbaud P, et al. Stimulus-sensitive spinal segmental myoclonus improved with injections of botulinum toxin type A. Mov Disord 1999; 14:182.
  41. Campos CR, Limongi JC, Machado FC, Brotto MW. A case of primary spinal myoclonus: clinical presentation and possible mechanisms involved. Arq Neuropsiquiatr 2003; 61:112.
  42. Simpson DM, Blitzer A, Brashear A, et al. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2008; 70:1699.
  43. Yoshimura DM, Aminoff MJ, Tami TA, Scott AB. Treatment of hemifacial spasm with botulinum toxin. Muscle Nerve 1992; 15:1045.
  44. Park YC, Lim JK, Lee DK, Yi SD. Botulinum a toxin treatment of hemifacial spasm and blepharospasm. J Korean Med Sci 1993; 8:334.
  45. Defazio G, Abbruzzese G, Girlanda P, et al. Botulinum toxin A treatment for primary hemifacial spasm: a 10-year multicenter study. Arch Neurol 2002; 59:418.
  46. Kenney C, Jankovic J. Botulinum toxin in the treatment of blepharospasm and hemifacial spasm. J Neural Transm (Vienna) 2008; 115:585.
  47. Carnero-Pardo C, Sánchez-Alvarez JC, Gómez-Camello A, et al. Myoclonus associated with thoracodorsal neuropathy. Mov Disord 1998; 13:971.
  48. Birthi P, Walters C, Ortiz Vargas O, Karandikar N. The use of intrathecal baclofen therapy for myoclonus in a patient with Lance Adams syndrome. PM R 2011; 3:671.
  49. Chiodo AE, Saval A. Intrathecal baclofen for the treatment of spinal myoclonus: a case series. J Spinal Cord Med 2012; 35:64.
  50. Caviness JN, Brown P. Myoclonus: current concepts and recent advances. Lancet Neurol 2004; 3:598.
  51. Chokroverty S, Manocha MK, Duvoisin RC. A physiologic and pharmacologic study in anticholinergic-responsive essential myoclonus. Neurology 1987; 37:608.
  52. Uluduz D, Karaali-Savrun F, Gunduz A, Kiziltan ME. An unusual case of vascular loop syndrome. J Headache Pain 2007; 8:242.
  53. Nasr A, Brown N. Palatal myoclonus responding to lamotrigine. Seizure 2002; 11:136.
  54. Scott BL, Evans RW, Jankovic J. Treatment of palatal myoclonus with sumatriptan. Mov Disord 1996; 11:748.
  55. Siniscalchi A, Mancuso F, Russo E, et al. Spinal myoclonus responsive to topiramate. Mov Disord 2004; 19:1380.
  56. Duvoisin RC. Essential myoclonus: response to anticholinergic therapy. Clin Neuropharmacol 1984; 7:141.
  57. Eldridge R, Iivanainen M, Stern R, et al. "Baltic" myoclonus epilepsy: hereditary disorder of childhood made worse by phenytoin. Lancet 1983; 2:838.
  58. Frucht SJ, Houghton WC, Bordelon Y, et al. A single-blind, open-label trial of sodium oxybate for myoclonus and essential tremor. Neurology 2005; 65:1967.
  59. Franzini A, Ferroli P, Dones I, et al. Chronic motor cortex stimulation for movement disorders: a promising perspective. Neurol Res 2003; 25:123.
  60. Starr PA. Deep brain stimulation for other tremors, myoclonus, and chorea. Handb Clin Neurol 2013; 116:209.
  61. Rughani AI, Lozano AM. Surgical treatment of myoclonus dystonia syndrome. Mov Disord 2013; 28:282.
  62. Vidailhet M, Jutras MF, Grabli D, Roze E. Deep brain stimulation for dystonia. J Neurol Neurosurg Psychiatry 2013; 84:1029.
  63. Roze E, Vidailhet M, Hubsch C, et al. Pallidal stimulation for myoclonus-dystonia: Ten years' outcome in two patients. Mov Disord 2015; 30:871.
  64. Jang W, Kim JS, Ahn JY, Kim HT. Reversible propriospinal myoclonus due to thoracic disc herniation: long-term follow-up. J Neurol Sci 2012; 313:32.
  65. Ensink RJ, Vingerhoets HM, Schmidt CW, Cremers CW. Treatment for severe palatoclonus by occlusion of the eustachian tube. Otol Neurotol 2003; 24:714.
  66. Bento RF, Sanchez TG, Miniti A, Tedesco-Marchesi AJ. Continuous, high-frequency objective tinnitus caused by middle ear myoclonus. Ear Nose Throat J 1998; 77:814.
  67. Golz A, Fradis M, Netzer A, et al. Bilateral tinnitus due to middle-ear myoclonus. Int Tinnitus J 2003; 9:52.
  68. Golz A, Fradis M, Martzu D, et al. Stapedius muscle myoclonus. Ann Otol Rhinol Laryngol 2003; 112:522.
  69. Bhimrao SK, Masterson L, Baguley D. Systematic review of management strategies for middle ear myoclonus. Otolaryngol Head Neck Surg 2012; 146:698.
  70. Miller LE, Miller VM. Safety and effectiveness of microvascular decompression for treatment of hemifacial spasm: a systematic review. Br J Neurosurg 2012; 26:438.
  71. Caviness JN. Epileptic myoclonus. In: Atlas of Video-EEG Monitoring, Sirven JI, Stern JM (Eds), McGraw-Hill Medical, New York 2011. p.309.
  72. Caviness JN. Segmental myoclonus. In: Hyperkinetic Movement Disorders, Albanese A, Jankovic J (Eds), Wiley-Blackwell, Oxford 2012. p.221.