UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Treatment of dystonia

Author
Cynthia Comella, MD
Section Editor
Howard I Hurtig, MD
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

The treatment of dystonia is symptomatic. No curative therapies are available. Management options include oral medications, botulinum toxin injection, and deep brain stimulation.

This topic will review the treatment of dystonia. Other clinical aspects of dystonia are discussed separately. (See "Classification and evaluation of dystonia".)

DRUG TREATMENTS

Many oral agents have been used to treat dystonia, but they have not been extensively studied in rigorous controlled trials. The available evidence suggests that several drugs are beneficial in a proportion of patients with dystonia (table 1). However, the therapeutic window for most of the oral agents is narrow, and side effects frequently limit clinical benefit [1].

Levodopa — Levodopa is dramatically beneficial in dopa-responsive dystonia (DRD). Often, small doses of levodopa one to three times daily can virtually eliminate the dystonic symptoms of DRD for an indefinite period of time without causing motor complications [2-4]. A sustained and complete response occurs with variable doses ranging from 100 to 750 mg daily [2,5]. One case series of 20 patients reported that the mean daily levodopa doses for clinical benefit in patients with and without associated dyskinesia were 343 mg and 189 mg [4].

In other forms of dystonia, the response rate with levodopa is approximately 15 percent [6].

                    

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: Nov 2016. | This topic last updated: Wed Nov 09 00:00:00 GMT+00:00 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 ©2016 UpToDate, Inc.
References
Top
  1. Jankovic J. Treatment of dystonia. Lancet Neurol 2006; 5:864.
  2. Rajput AH, Gibb WR, Zhong XH, et al. Dopa-responsive dystonia: pathological and biochemical observations in a case. Ann Neurol 1994; 35:396.
  3. Nutt JG, Nygaard TG. Response to levodopa treatment in dopa-responsive dystonia. Arch Neurol 2001; 58:905.
  4. Hwang WJ, Calne DB, Tsui JK, de la Fuente-Fernández R. The long-term response to levodopa in dopa-responsive dystonia. Parkinsonism Relat Disord 2001; 8:1.
  5. Albanese A, Barnes MP, Bhatia KP, et al. A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force. Eur J Neurol 2006; 13:433.
  6. Greene P, Shale H, Fahn S. Experience with high dosages of anticholinergic and other drugs in the treatment of torsion dystonia. Adv Neurol 1988; 50:547.
  7. Burke RE, Fahn S. Double-blind evaluation of trihexyphenidyl in dystonia. Adv Neurol 1983; 37:189.
  8. Fahn S. High dosage anticholinergic therapy in dystonia. Neurology 1983; 33:1255.
  9. Burke RE, Fahn S, Marsden CD. Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology 1986; 36:160.
  10. Nutt JG, Hammerstad JP, deGarmo P, Carter J. Cranial dystonia: double-blind crossover study of anticholinergics. Neurology 1984; 34:215.
  11. Albanese A, Asmus F, Bhatia KP, et al. EFNS guidelines on diagnosis and treatment of primary dystonias. Eur J Neurol 2011; 18:5.
  12. Jankovic J. Treatment of hyperkinetic movement disorders with tetrabenazine: a double-blind crossover study. Ann Neurol 1982; 11:41.
  13. Kenney C, Hunter C, Jankovic J. Long-term tolerability of tetrabenazine in the treatment of hyperkinetic movement disorders. Mov Disord 2007; 22:193.
  14. Jankovic J, Beach J. Long-term effects of tetrabenazine in hyperkinetic movement disorders. Neurology 1997; 48:358.
  15. Greene P. Baclofen in the treatment of dystonia. Clin Neuropharmacol 1992; 15:276.
  16. Ford B, Greene PE, Louis ED, et al. Intrathecal baclofen in the treatment of dystonia. Adv Neurol 1998; 78:199.
  17. An JY, Kim JS, Kim YI, Lee KS. Successful treatment of the Meige syndrome with oral zolpidem monotherapy. Mov Disord 2008; 23:1619.
  18. Park IS, Kim JS, An JY, et al. Excellent response to oral zolpidem in a sporadic case of the myoclonus dystonia syndrome. Mov Disord 2009; 24:2172.
  19. Garretto NS, Bueri JA, Rey RD, et al. Improvement of blepharospasm with Zolpidem. Mov Disord 2004; 19:967.
  20. Evidente VG. Zolpidem improves dystonia in "Lubag" or X-linked dystonia-parkinsonism syndrome. Neurology 2002; 58:662.
  21. Lang AE. Dopamine agonists and antagonists in the treatment of idiopathic dystonia. Adv Neurol 1988; 50:561.
  22. Karp BI, Goldstein SR, Chen R, et al. An open trial of clozapine for dystonia. Mov Disord 1999; 14:652.
  23. Simpson DM, Hallett M, Ashman EJ, et al. Practice guideline update summary: Botulinum neurotoxin for the treatment of blepharospasm, cervical dystonia, adult spasticity, and headache: Report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology 2016; 86:1818.
  24. Jinnah HA, Factor SA. Diagnosis and treatment of dystonia. Neurol Clin 2015; 33:77.
  25. Marques RE, Duarte GS, Rodrigues FB, et al. Botulinum toxin type B for cervical dystonia. Cochrane Database Syst Rev 2016; :CD004315.
  26. Duarte GS, Castelão M, Rodrigues FB, et al. Botulinum toxin type A versus botulinum toxin type B for cervical dystonia. Cochrane Database Syst Rev 2016; 10:CD004314.
  27. Costa J, Espírito-Santo C, Borges A, et al. Botulinum toxin type A therapy for cervical dystonia. Cochrane Database Syst Rev 2005; :CD003633.
  28. Mejia NI, Vuong KD, Jankovic J. Long-term botulinum toxin efficacy, safety, and immunogenicity. Mov Disord 2005; 20:592.
  29. Brans JW, Lindeboom R, Snoek JW, et al. Botulinum toxin versus trihexyphenidyl in cervical dystonia: a prospective, randomized, double-blind controlled trial. Neurology 1996; 46:1066.
  30. Costa J, Espírito-Santo C, Borges A, et al. Botulinum toxin type A versus anticholinergics for cervical dystonia. Cochrane Database Syst Rev 2005; :CD004312.
  31. 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.
  32. Jankovic J, Comella C, Hanschmann A, Grafe S. Efficacy and safety of incobotulinumtoxinA (NT 201, Xeomin) in the treatment of blepharospasm-a randomized trial. Mov Disord 2011; 26:1521.
  33. Kruisdijk JJ, Koelman JH, Ongerboer de Visser BW, et al. Botulinum toxin for writer's cramp: a randomised, placebo-controlled trial and 1-year follow-up. J Neurol Neurosurg Psychiatry 2007; 78:264.
  34. Troung DD, Rontal M, Rolnick M, et al. Double-blind controlled study of botulinum toxin in adductor spasmodic dysphonia. Laryngoscope 1991; 101:630.
  35. Bielamowicz S, Squire S, Bidus K, Ludlow CL. Assessment of posterior cricoarytenoid botulinum toxin injections in patients with abductor spasmodic dysphonia. Ann Otol Rhinol Laryngol 2001; 110:406.
  36. Albanese A. Terminology for preparations of botulinum neurotoxins: what a difference a name makes. JAMA 2011; 305:89.
  37. Chapman MA, Barron R, Tanis DC, et al. Comparison of botulinum neurotoxin preparations for the treatment of cervical dystonia. Clin Ther 2007; 29:1325.
  38. Pagan FL, Harrison A. A guide to dosing in the treatment of cervical dystonia and blepharospasm with Xeomin®: a new botulinum neurotoxin A. Parkinsonism Relat Disord 2012; 18:441.
  39. Colosimo C, Tiple D, Berardelli A. Efficacy and safety of long-term botulinum toxin treatment in craniocervical dystonia: a systematic review. Neurotox Res 2012; 22:265.
  40. Brin MF, Comella CL, Jankovic J, et al. Long-term treatment with botulinum toxin type A in cervical dystonia has low immunogenicity by mouse protection assay. Mov Disord 2008; 23:1353.
  41. Lange O, Bigalke H, Dengler R, et al. Neutralizing antibodies and secondary therapy failure after treatment with botulinum toxin type A: much ado about nothing? Clin Neuropharmacol 2009; 32:213.
  42. Naumann M, Carruthers A, Carruthers J, et al. Meta-analysis of neutralizing antibody conversion with onabotulinumtoxinA (BOTOX®) across multiple indications. Mov Disord 2010; 25:2211.
  43. Benecke R. Xeomin in the treatment of cervical dystonia. Eur J Neurol 2009; 16 Suppl 2:6.
  44. Kaňovský P, Slawek J, Denes Z, et al. Efficacy and safety of treatment with incobotulinum toxin A (botulinum neurotoxin type A free from complexing proteins; NT 201) in post-stroke upper limb spasticity. J Rehabil Med 2011; 43:486.
  45. Chinnapongse RB, Lew MF, Ferreira JJ, et al. Immunogenicity and long-term efficacy of botulinum toxin type B in the treatment of cervical dystonia: report of 4 prospective, multicenter trials. Clin Neuropharmacol 2012; 35:215.
  46. Vidailhet M, Vercueil L, Houeto JL, et al. Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 2005; 352:459.
  47. Zorzi G, Marras C, Nardocci N, et al. Stimulation of the globus pallidus internus for childhood-onset dystonia. Mov Disord 2005; 20:1194.
  48. Kupsch A, Benecke R, Müller J, et al. Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 2006; 355:1978.
  49. Parr JR, Green AL, Joint C, et al. Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia. Arch Dis Child 2007; 92:708.
  50. Alterman RL, Miravite J, Weisz D, et al. Sixty hertz pallidal deep brain stimulation for primary torsion dystonia. Neurology 2007; 69:681.
  51. Kiss ZH, Doig-Beyaert K, Eliasziw M, et al. The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain 2007; 130:2879.
  52. Alterman RL, Snyder BJ. Deep brain stimulation for torsion dystonia. Acta Neurochir Suppl 2007; 97:191.
  53. Vidailhet M, Vercueil L, Houeto JL, et al. Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 2007; 6:223.
  54. Fox MD, Alterman RL. Brain Stimulation for Torsion Dystonia. JAMA Neurol 2015; 72:713.
  55. Volkmann J, Wolters A, Kupsch A, et al. Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 2012; 11:1029.
  56. Isaias IU, Alterman RL, Tagliati M. Deep brain stimulation for primary generalized dystonia: long-term outcomes. Arch Neurol 2009; 66:465.
  57. Cif L, Vasques X, Gonzalez V, et al. Long-term follow-up of DYT1 dystonia patients treated by deep brain stimulation: an open-label study. Mov Disord 2010; 25:289.
  58. Reese R, Gruber D, Schoenecker T, et al. Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 2011; 26:691.
  59. Walsh RA, Sidiropoulos C, Lozano AM, et al. Bilateral pallidal stimulation in cervical dystonia: blinded evidence of benefit beyond 5 years. Brain 2013; 136:761.
  60. Starr PA, Turner RS, Rau G, et al. Microelectrode-guided implantation of deep brain stimulators into the globus pallidus internus for dystonia: techniques, electrode locations, and outcomes. J Neurosurg 2006; 104:488.
  61. Vayssiere N, van der Gaag N, Cif L, et al. Deep brain stimulation for dystonia confirming a somatotopic organization in the globus pallidus internus. J Neurosurg 2004; 101:181.
  62. Berman BD, Starr PA, Marks WJ Jr, Ostrem JL. Induction of bradykinesia with pallidal deep brain stimulation in patients with cranial-cervical dystonia. Stereotact Funct Neurosurg 2009; 87:37.
  63. Zauber SE, Watson N, Comella CL, et al. Stimulation-induced parkinsonism after posteroventral deep brain stimulation of the globus pallidus internus for craniocervical dystonia. J Neurosurg 2009; 110:229.
  64. Blahak C, Capelle HH, Baezner H, et al. Micrographia induced by pallidal DBS for segmental dystonia: a subtle sign of hypokinesia? J Neural Transm (Vienna) 2011; 118:549.
  65. Schrader C, Capelle HH, Kinfe TM, et al. GPi-DBS may induce a hypokinetic gait disorder with freezing of gait in patients with dystonia. Neurology 2011; 77:483.
  66. Sun B, Chen S, Zhan S, et al. Subthalamic nucleus stimulation for primary dystonia and tardive dystonia. Acta Neurochir Suppl 2007; 97:207.
  67. Kleiner-Fisman G, Liang GS, Moberg PJ, et al. Subthalamic nucleus deep brain stimulation for severe idiopathic dystonia: impact on severity, neuropsychological status, and quality of life. J Neurosurg 2007; 107:29.
  68. Ostrem JL, Racine CA, Glass GA, et al. Subthalamic nucleus deep brain stimulation in primary cervical dystonia. Neurology 2011; 76:870.
  69. Schjerling L, Hjermind LE, Jespersen B, et al. A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia. J Neurosurg 2013; 119:1537.
  70. Pretto TE, Dalvi A, Kang UJ, Penn RD. A prospective blinded evaluation of deep brain stimulation for the treatment of secondary dystonia and primary torticollis syndromes. J Neurosurg 2008; 109:405.
  71. Eltahawy HA, Saint-Cyr J, Giladi N, et al. Primary dystonia is more responsive than secondary dystonia to pallidal interventions: outcome after pallidotomy or pallidal deep brain stimulation. Neurosurgery 2004; 54:613.
  72. Deutschländer A, Asmus F, Gasser T, et al. Sporadic rapid-onset dystonia-parkinsonism syndrome: failure of bilateral pallidal stimulation. Mov Disord 2005; 20:254.
  73. Castelnau P, Cif L, Valente EM, et al. Pallidal stimulation improves pantothenate kinase-associated neurodegeneration. Ann Neurol 2005; 57:738.
  74. Zhang JG, Zhang K, Wang ZC, et al. Deep brain stimulation in the treatment of secondary dystonia. Chin Med J (Engl) 2006; 119:2069.
  75. Koy A, Hellmich M, Pauls KA, et al. Effects of deep brain stimulation in dyskinetic cerebral palsy: a meta-analysis. Mov Disord 2013; 28:647.
  76. Gonzalez-Alegre P, Bode N, Davidson BL, Paulson HL. Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia. J Neurosci 2005; 25:10502.
  77. Kimberley TJ, Borich MR, Arora S, Siebner HR. Multiple sessions of low-frequency repetitive transcranial magnetic stimulation in focal hand dystonia: clinical and physiological effects. Restor Neurol Neurosci 2013; 31:533.
  78. Pirio Richardson S, Tinaz S, Chen R. Repetitive transcranial magnetic stimulation in cervical dystonia: effect of site and repetition in a randomized pilot trial. PLoS One 2015; 10:e0124937.
  79. Tarsy D, Simon DK. Dystonia. N Engl J Med 2006; 355:818.