Update on Treatments for Dystonia

Movement Disorders (M Okun, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Movement Disorders

Abstract

Oral medication, botulinum toxin injections, and deep brain stimulation are the current mainstays of treatment for dystonia. In addition, physical and other supportive therapies may help prevent further complications (eg, contractures) and improve function. This review discusses evidence-based medical treatment of dystonia with an emphasis on recent advances in treatment. We will also review the current treatment approaches and suggest ways in which these therapies can be applied to individuals with dystonia.

Keywords

Dystonia Treatment Medication Deep brain stimulation 

Notes

Compliance with Ethics Guidelines

Conflict of Interest

D. Cristopher Bragg declares that he has no conflict of interest. Nutan Sharma has received consultancy fees from Merz Pharmaceuticals and Allergan; a grant from NIH (NIH/NINDS P50NS037409); honorarium payment from The American Academy of Neurology; and payment for development of an educational presentation from Oakstone Publishing.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.••
    Albanese A, Bhatia K, Bressman SB, DeLong MR, Fahn S, Fung VSC, et al. Phenomenology and classification of dystonia: a consensus update. Mov Disord. 2013;28:863–72. The most recent international consensus on the classification of dystonia.PubMedCentralPubMedCrossRefGoogle Scholar
  2. 2.
    Segawa M, Hosaka A, Miyagawa F, Nomura Y, Imai H. Hereditary progressive dystonia with marked diurnal fluctuation. Adv Neurol. 1976;14:215–33.PubMedGoogle Scholar
  3. 3.
    Swoboda KJ, Furukawa Y. Tyrosine Hydroxylase Deficiency. 2008 Feb 8. In: Pagon RA, Adam MP, Bird TD, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2014. Available from: http://www.ncbi.nlm.nih.gov.ezp-prod1.hul.harvard.edu/books/NBK1437/. Accessed 8 Mar 2014.
  4. 4.
    Jankovic J. Medical treatment of dystonia. Mov Disord. 2013;28:1001–12.PubMedCrossRefGoogle Scholar
  5. 5.
    Mathur SN, Chu SK, McCormick Z, Chien GCC, Marciniak CM. Long-term intrathecal baclofen: outcomes after more than 10 years of treatment. PM&R 2013; doi: 10.1016/j.pmrj.2013.12.005.
  6. 6.
    Albright AL, Barry MJ, Shafron DH, Ferson SS. Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol. 2001;43:652–7.PubMedCrossRefGoogle Scholar
  7. 7.•
    Miyazaki Y, Sako W, Asanuma K, Izumi Y, Miki T, Kaji R. Efficacy of zolpidem for dystonia: a study among different subtypes. FNEURO. 2012;3:1–5. First evidence that zolpidem may be of benefit in dystonia.Google Scholar
  8. 8.
    Burke RE, Fahn S, Marsden CD. Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology. 1986;36:160–4.PubMedCrossRefGoogle Scholar
  9. 9.
    Kenney C, Hunter C, Jankovic J. Long-term tolerability of tetrabenazine in the treatment of hyperkinetic movement disorders. Mov Disord. 2007;22:193–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Zadikoff C, Wadia PM, Miyasaki J, Chen R, Lang AE, So J, et al. Cannabinoid, CB1 agonists in cervical dystonia: failure in a phase IIa randomized controlled trial. Basal Ganglia. 2011;1:91–5.CrossRefGoogle Scholar
  11. 11.
    Dickerson JW, Conn PJ. Therapeutic potential of targeting metabotropic glutamate receptors for Parkinson’s disease. Neurodegener Dis Manag. 2012;2:221–32.PubMedCentralPubMedCrossRefGoogle Scholar
  12. 12.
    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;70:1699–706.PubMedCrossRefGoogle Scholar
  13. 13.
    Colosimo C, Tiple D, Berardelli A. Efficacy and safety of long-term botulinum toxin treatment in craniocervical dystonia: a systematic review. Neurotox Res. 2012;22:265–73.PubMedCrossRefGoogle Scholar
  14. 14.••
    Hallett M, Albanese A, Dressler D, Segal KR, Simpson DM, Truong D, et al. Evidence-based review and assessment of botulinum neurotoxin for the treatment of movement disorders. Toxicon. 2013;67:94–114. Provides most comprehensive review regarding the use of botulinum toxin in focal dystonias.PubMedCrossRefGoogle Scholar
  15. 15.•
    Ramirez-Castaneda J, Jankovic J. Long-term efficacy and safety of botulinum toxin injections in dystonia. Toxins (Basel). 2013;5:249–66. Comprehensive review of the long-term use of botulinum toxin.CrossRefGoogle Scholar
  16. 16.
    Lim ECH, Seet RCS. Use of botulinum toxin in the neurology clinic. Nat Rev Neurol. 2010;6:624–36.PubMedCrossRefGoogle Scholar
  17. 17.
    Van Gerpen JA, Matsumoto JY, Ahlskog JE, Maraganore DM, McManis PG. Utility of an EMG mapping study in treating cervical dystonia. Muscle Nerve. 2000;23:1752–6.PubMedCrossRefGoogle Scholar
  18. 18.
    Deuschl G, Heinen F, Kleedorfer B, Wagner M, Lucking CH, Poewe W. Clinical and polymyographic investigation of spasmodic torticollis. J Neurol. 1992;239:9–15.PubMedCrossRefGoogle Scholar
  19. 19.
    Dressler D. Electromyographic evaluation of cervical dystonia for planning of botulinum toxin therapy. Eur J Neurol. 2000;7:713–8.PubMedCrossRefGoogle Scholar
  20. 20.
    Brans JW, Aramideh M, Koelman JH, Lindeboom R, Speelman JD, Ongerboer de Visser BW. Electromyography in cervical dystonia: changes after botulinum and trihexyphenidyl. Neurology. 1998;51:815–9.PubMedCrossRefGoogle Scholar
  21. 21.
    Miocinovic S, Somayajula S, Chitnis S, Vitek JL. History, applications and mechanisms of deep brain stimulation. JAMA Neurol. 2013;70:163–71.PubMedCrossRefGoogle Scholar
  22. 22.
    Petrossian MT, Paul LR, Multhaupt-Buell TJ, Eckhardt C, Hayes MT, Duhaime AC, et al. Pallidal deep brain stimulation for dystonia: a case series. J Neurosurg Pediatr. 2013;12:582–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Kupsch A, Benecke R, Muller J, Trottenberg T, Schneider GH, Poewe W, et al. Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med. 2006;355:1978–90.PubMedCrossRefGoogle Scholar
  24. 24.
    Kiss ZH, Doig-Beyaert K, Eliasziw M, Tsui J, Haffenden A, Suchowersky O, et al. The Canadian multicentre study of deep brain stimulation for cervical dystonia. Brain. 2007;130:2879–86.PubMedCrossRefGoogle Scholar
  25. 25.
    Reese R, Gruber D, Schoenecker T, Bazner H, Blahak C, Capelle HH, et al. Long-term clinical outcome in Meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord. 2011;26:691–8.PubMedCrossRefGoogle Scholar
  26. 26.
    Borggraefe I, Mehrkens JH, Telegravciska M, Berweck S, Botzel K, Heinen F. Bilateral pallidal stimulation in children and adolescents with primary generalized dystonia report of six patients and literature-based analysis of predictive outcomes variables. Brain Dev. 2010;32:223–8.PubMedCrossRefGoogle Scholar
  27. 27.
    Markun LC, Starr PA, Air EL, Marks Jr WJ, Volz MM, Ostrem JL. Shorter disease duration correlates with improved long-term deep brain stimulation outcomes in young-onset DYT1 dystonia. Neurosurgery. 2012;71:325–30.PubMedCrossRefGoogle Scholar
  28. 28.
    Okun MS, Foote KD. Setting realistic expectations for DBS in dystonia. Lancet Neurol. 2012;11:1014–5.PubMedCrossRefGoogle Scholar
  29. 29.
    Gruber D, Trottenberg T, Kivi A, Schoenecker T, Kopp UA, Hoffmann KT, et al. Long-term effects of pallidal deep brain stimulation in tardive dystonia. Neurology. 2009;73:53–8.PubMedCrossRefGoogle Scholar
  30. 30.
    Capelle HH, Blahak C, Schrader C, Baezner H, Kinfe TM, Herzog J, et al. Chronic deep brain stimulation in patients with tardive dystonia without a history of major psychosis. Mov Disord. 2010;25:1477–81.PubMedCrossRefGoogle Scholar
  31. 31.
    Chang EF, Schrock LE, Starr PA, Ostrem JL. Long-term benefit sustained after bilateral pallidal deep brain stimulation in patients with refractory tardive dystonia. Stereotact Funct Neurosurg. 2010;88:304–10.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of NeurologyMassachusetts General HospitalBostonUSA
  2. 2.CharlestownUSA

Personalised recommendations